question,question_id,question_type,answer,focus,id,source,url,cui,semanticType,semanticGroup What is (are) Adult Acute Lymphoblastic Leukemia ?,0000001_1-1,information,"Key Points - Adult acute lymphoblastic leukemia (ALL) is a type of cancer in which the bone marrow makes too many lymphocytes (a type of white blood cell). - Leukemia may affect red blood cells, white blood cells, and platelets. - Previous chemotherapy and exposure to radiation may increase the risk of developing ALL. - Signs and symptoms of adult ALL include fever, feeling tired, and easy bruising or bleeding. - Tests that examine the blood and bone marrow are used to detect (find) and diagnose adult ALL. - Certain factors affect prognosis (chance of recovery) and treatment options. Adult acute lymphoblastic leukemia (ALL) is a type of cancer in which the bone marrow makes too many lymphocytes (a type of white blood cell). Adult acute lymphoblastic leukemia (ALL; also called acute lymphocytic leukemia) is a cancer of the blood and bone marrow. This type of cancer usually gets worse quickly if it is not treated. Leukemia may affect red blood cells, white blood cells, and platelets. Normally, the bone marrow makes blood stem cells (immature cells) that become mature blood cells over time. A blood stem cell may become a myeloid stem cell or a lymphoid stem cell. A myeloid stem cell becomes one of three types of mature blood cells: - Red blood cells that carry oxygen and other substances to all tissues of the body. - Platelets that form blood clots to stop bleeding. - Granulocytes (white blood cells) that fight infection and disease. A lymphoid stem cell becomes a lymphoblast cell and then one of three types of lymphocytes (white blood cells): - B lymphocytes that make antibodies to help fight infection. - T lymphocytes that help B lymphocytes make the antibodies that help fight infection. - Natural killer cells that attack cancer cells and viruses. In ALL, too many stem cells become lymphoblasts, B lymphocytes, or T lymphocytes. These cells are also called leukemia cells. These leukemia cells are not able to fight infection very well. Also, as the number of leukemia cells increases in the blood and bone marrow, there is less room for healthy white blood cells, red blood cells, and platelets. This may cause infection, anemia, and easy bleeding. The cancer can also spread to the central nervous system (brain and spinal cord). This summary is about adult acute lymphoblastic leukemia. See the following PDQ summaries for information about other types of leukemia: - Childhood Acute Lymphoblastic Leukemia Treatment. - Adult Acute Myeloid Leukemia Treatment. - Childhood Acute Myeloid Leukemia/Other Myeloid Malignancies Treatment. - Chronic Lymphocytic Leukemia Treatment. - Chronic Myelogenous Leukemia Treatment. - Hairy Cell Leukemia Treatment.",Adult Acute Lymphoblastic Leukemia,0000001_1,CancerGov,https://www.cancer.gov/types/leukemia/patient/adult-all-treatment-pdq,C0751606,T191,Disorders What are the symptoms of Adult Acute Lymphoblastic Leukemia ?,0000001_1-2,symptoms,"Signs and symptoms of adult ALL include fever, feeling tired, and easy bruising or bleeding. The early signs and symptoms of ALL may be like the flu or other common diseases. Check with your doctor if you have any of the following: - Weakness or feeling tired. - Fever or night sweats. - Easy bruising or bleeding. - Petechiae (flat, pinpoint spots under the skin, caused by bleeding). - Shortness of breath. - Weight loss or loss of appetite. - Pain in the bones or stomach. - Pain or feeling of fullness below the ribs. - Painless lumps in the neck, underarm, stomach, or groin. - Having many infections. These and other signs and symptoms may be caused by adult acute lymphoblastic leukemia or by other conditions.",Adult Acute Lymphoblastic Leukemia,0000001_1,CancerGov,https://www.cancer.gov/types/leukemia/patient/adult-all-treatment-pdq,C0751606,T191,Disorders How to diagnose Adult Acute Lymphoblastic Leukemia ?,0000001_1-3,exams and tests,"Tests that examine the blood and bone marrow are used to detect (find) and diagnose adult ALL. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as infection or anything else that seems unusual. A history of the patient's health habits and past illnesses and treatments will also be taken. - Complete blood count (CBC) with differential : A procedure in which a sample of blood is drawn and checked for the following: - The number of red blood cells and platelets. - The number and type of white blood cells. - The amount of hemoglobin (the protein that carries oxygen) in the red blood cells. - The portion of the blood sample made up of red blood cells. - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. - Peripheral blood smear : A procedure in which a sample of blood is checked for blast cells, the number and kinds of white blood cells, the number of platelets, and changes in the shape of blood cells. - Bone marrow aspiration and biopsy : The removal of bone marrow, blood, and a small piece of bone by inserting a hollow needle into the hipbone or breastbone. A pathologist views the bone marrow, blood, and bone under a microscope to look for abnormal cells. The following tests may be done on the samples of blood or bone marrow tissue that are removed: - Cytogenetic analysis: A laboratory test in which the cells in a sample of blood or bone marrow are looked at under a microscope to find out if there are certain changes in the chromosomes of lymphocytes. For example, in Philadelphia chromosome positive ALL, part of one chromosome switches places with part of another chromosome. This is called the Philadelphia chromosome. - Immunophenotyping : A process used to identify cells, based on the types of antigens or markers on the surface of the cell. This process is used to diagnose the subtype of ALL by comparing the cancer cells to normal cells of the immune system. For example, a cytochemistry study may test the cells in a sample of tissue using chemicals (dyes) to look for certain changes in the sample. A chemical may cause a color change in one type of leukemia cell but not in another type of leukemia cell.",Adult Acute Lymphoblastic Leukemia,0000001_1,CancerGov,https://www.cancer.gov/types/leukemia/patient/adult-all-treatment-pdq,C0751606,T191,Disorders What is the outlook for Adult Acute Lymphoblastic Leukemia ?,0000001_1-4,outlook,"Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - The age of the patient. - Whether the cancer has spread to the brain or spinal cord. - Whether there are certain changes in the genes, including the Philadelphia chromosome. - Whether the cancer has been treated before or has recurred (come back).",Adult Acute Lymphoblastic Leukemia,0000001_1,CancerGov,https://www.cancer.gov/types/leukemia/patient/adult-all-treatment-pdq,C0751606,T191,Disorders Who is at risk for Adult Acute Lymphoblastic Leukemia? ?,0000001_1-5,susceptibility,"Previous chemotherapy and exposure to radiation may increase the risk of developing ALL. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesnt mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Possible risk factors for ALL include the following: - Being male. - Being white. - Being older than 70. - Past treatment with chemotherapy or radiation therapy. - Being exposed to high levels of radiation in the environment (such as nuclear radiation). - Having certain genetic disorders, such as Down syndrome.",Adult Acute Lymphoblastic Leukemia,0000001_1,CancerGov,https://www.cancer.gov/types/leukemia/patient/adult-all-treatment-pdq,C0751606,T191,Disorders What are the stages of Adult Acute Lymphoblastic Leukemia ?,0000001_1-6,stages,"Key Points - Once adult ALL has been diagnosed, tests are done to find out if the cancer has spread to the central nervous system (brain and spinal cord) or to other parts of the body. - There is no standard staging system for adult ALL. Once adult ALL has been diagnosed, tests are done to find out if the cancer has spread to the central nervous system (brain and spinal cord) or to other parts of the body. The extent or spread of cancer is usually described as stages. It is important to know whether the leukemia has spread outside the blood and bone marrow in order to plan treatment. The following tests and procedures may be used to determine if the leukemia has spread: - Chest x-ray : An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. - Lumbar puncture : A procedure used to collect a sample of cerebrospinal fluid (CSF) from the spinal column. This is done by placing a needle between two bones in the spine and into the CSF around the spinal cord and removing a sample of the fluid. The sample of CSF is checked under a microscope for signs that leukemia cells have spread to the brain and spinal cord. This procedure is also called an LP or spinal tap. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of the abdomen, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). There is no standard staging system for adult ALL. The disease is described as untreated, in remission, or recurrent. Untreated adult ALL The ALL is newly diagnosed and has not been treated except to relieve signs and symptoms such as fever, bleeding, or pain. - The complete blood count is abnormal. - More than 5% of the cells in the bone marrow are blasts (leukemia cells). - There are signs and symptoms of leukemia. Adult ALL in remission The ALL has been treated. - The complete blood count is normal. - 5% or fewer of the cells in the bone marrow are blasts (leukemia cells). - There are no signs or symptoms of leukemia other than in the bone marrow.",Adult Acute Lymphoblastic Leukemia,0000001_1,CancerGov,https://www.cancer.gov/types/leukemia/patient/adult-all-treatment-pdq,C0751606,T191,Disorders What are the treatments for Adult Acute Lymphoblastic Leukemia ?,0000001_1-7,treatment,"Key Points - There are different types of treatment for patients with adult ALL. - The treatment of adult ALL usually has two phases. - Four types of standard treatment are used: - Chemotherapy - Radiation therapy - Chemotherapy with stem cell transplant - Targeted therapy - New types of treatment are being tested in clinical trials. - Biologic therapy - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Patients with ALL may have late effects after treatment. - Follow-up tests may be needed. There are different types of treatment for patients with adult ALL. Different types of treatment are available for patients with adult acute lymphoblastic leukemia (ALL). Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. The treatment of adult ALL usually has two phases. The treatment of adult ALL is done in phases: - Remission induction therapy: This is the first phase of treatment. The goal is to kill the leukemia cells in the blood and bone marrow. This puts the leukemia into remission. - Post-remission therapy: This is the second phase of treatment. It begins once the leukemia is in remission. The goal of post-remission therapy is to kill any remaining leukemia cells that may not be active but could begin to regrow and cause a relapse. This phase is also called remission continuation therapy. Treatment called central nervous system (CNS) sanctuary therapy is usually given during each phase of therapy. Because standard doses of chemotherapy may not reach leukemia cells in the CNS (brain and spinal cord), the cells are able to ""find sanctuary"" (hide) in the CNS. Systemic chemotherapy given in high doses, intrathecal chemotherapy, and radiation therapy to the brain are able to reach leukemia cells in the CNS. They are given to kill the leukemia cells and lessen the chance the leukemia will recur (come back). CNS sanctuary therapy is also called CNS prophylaxis. Four types of standard treatment are used: Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid (intrathecal chemotherapy), an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is treatment using more than one anticancer drug. The way the chemotherapy is given depends on the type and stage of the cancer being treated. Intrathecal chemotherapy may be used to treat adult ALL that has spread, or may spread, to the brain and spinal cord. When used to lessen the chance leukemia cells will spread to the brain and spinal cord, it is called central nervous system (CNS) sanctuary therapy or CNS prophylaxis. See Drugs Approved for Acute Lymphoblastic Leukemia for more information. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type of cancer. External radiation therapy may be used to treat adult ALL that has spread, or may spread, to the brain and spinal cord. When used this way, it is called central nervous system (CNS) sanctuary therapy or CNS prophylaxis. External radiation therapy may also be used as palliative therapy to relieve symptoms and improve quality of life. Chemotherapy with stem cell transplant Stem cell transplant is a method of giving chemotherapy and replacing blood-forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. See Drugs Approved for Acute Lymphoblastic Leukemia for more information. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Targeted therapy drugs called tyrosine kinase inhibitors are used to treat some types of adult ALL. These drugs block the enzyme, tyrosine kinase, that causes stem cells to develop into more white blood cells (blasts) than the body needs. Three of the drugs used are imatinib mesylate (Gleevec), dasatinib, and nilotinib. See Drugs Approved for Acute Lymphoblastic Leukemia for more information. New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Biologic therapy Biologic therapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Patients with ALL may have late effects after treatment. Side effects from cancer treatment that begin during or after treatment and continue for months or years are called late effects. Late effects of treatment for ALL may include the risk of second cancers (new types of cancer). Regular follow-up exams are very important for long-term survivors. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Adult Acute Lymphoblastic Leukemia Untreated Adult Acute Lymphoblastic Leukemia Standard treatment of adult acute lymphoblastic leukemia (ALL) during the remission induction phase includes the following: - Combination chemotherapy. - Tyrosine kinase inhibitor therapy with imatinib mesylate, in certain patients. Some of these patients will also have combination chemotherapy. - Supportive care including antibiotics and red blood cell and platelet transfusions. - CNS prophylaxis therapy including chemotherapy (intrathecal and/or systemic) with or without radiation therapy to the brain. Check the list of NCI-supported cancer clinical trials that are now accepting patients with untreated adult acute lymphoblastic leukemia. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Adult Acute Lymphoblastic Leukemia in Remission Standard treatment of adult ALL during the post-remission phase includes the following: - Chemotherapy. - Tyrosine kinase inhibitor therapy. - Chemotherapy with stem cell transplant. - CNS prophylaxis therapy including chemotherapy (intrathecal and/or systemic) with or without radiation therapy to the brain. Check the list of NCI-supported cancer clinical trials that are now accepting patients with adult acute lymphoblastic leukemia in remission. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Recurrent Adult Acute Lymphoblastic Leukemia Standard treatment of recurrent adult ALL may include the following: - Combination chemotherapy followed by stem cell transplant. - Low-dose radiation therapy as palliative care to relieve symptoms and improve the quality of life. - Tyrosine kinase inhibitor therapy with dasatinib for certain patients. Some of the treatments being studied in clinical trials for recurrent adult ALL include the following: - A clinical trial of stem cell transplant using the patient's stem cells. - A clinical trial of biologic therapy. - A clinical trial of new anticancer drugs. Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent adult acute lymphoblastic leukemia. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Adult Acute Lymphoblastic Leukemia,0000001_1,CancerGov,https://www.cancer.gov/types/leukemia/patient/adult-all-treatment-pdq,C0751606,T191,Disorders What is (are) Adult Acute Myeloid Leukemia ?,0000001_2-1,information,"Key Points - Adult acute myeloid leukemia (AML) is a type of cancer in which the bone marrow makes abnormal myeloblasts (a type of white blood cell), red blood cells, or platelets. - Leukemia may affect red blood cells, white blood cells, and platelets. - There are different subtypes of AML. - Smoking, previous chemotherapy treatment, and exposure to radiation may affect the risk of adult AML. - Signs and symptoms of adult AML include fever, feeling tired, and easy bruising or bleeding. - Tests that examine the blood and bone marrow are used to detect (find) and diagnose adult AML. - Certain factors affect prognosis (chance of recovery) and treatment options. Adult acute myeloid leukemia (AML) is a type of cancer in which the bone marrow makes abnormal myeloblasts (a type of white blood cell), red blood cells, or platelets. Adult acute myeloid leukemia (AML) is a cancer of the blood and bone marrow. This type of cancer usually gets worse quickly if it is not treated. It is the most common type of acute leukemia in adults. AML is also called acute myelogenous leukemia, acute myeloblastic leukemia, acute granulocytic leukemia, and acute nonlymphocytic leukemia. Leukemia may affect red blood cells, white blood cells, and platelets. Normally, the bone marrow makes blood stem cells (immature cells) that become mature blood cells over time. A blood stem cell may become a myeloid stem cell or a lymphoid stem cell. A lymphoid stem cell becomes a white blood cell. A myeloid stem cell becomes one of three types of mature blood cells: - Red blood cells that carry oxygen and other substances to all tissues of the body. - White blood cells that fight infection and disease. - Platelets that form blood clots to stop bleeding. In AML, the myeloid stem cells usually become a type of immature white blood cell called myeloblasts (or myeloid blasts). The myeloblasts in AML are abnormal and do not become healthy white blood cells. Sometimes in AML, too many stem cells become abnormal red blood cells or platelets. These abnormal white blood cells, red blood cells, or platelets are also called leukemia cells or blasts. Leukemia cells can build up in the bone marrow and blood so there is less room for healthy white blood cells, red blood cells, and platelets. When this happens, infection, anemia, or easy bleeding may occur. The leukemia cells can spread outside the blood to other parts of the body, including the central nervous system (brain and spinal cord), skin, and gums. This summary is about adult AML. See the following PDQ summaries for information about other types of leukemia: - Childhood Acute Myeloid Leukemia/Other Myeloid Malignancies Treatment - Chronic Myelogenous Leukemia Treatment - Adult Acute Lymphoblastic Leukemia Treatment - Childhood Acute Lymphoblastic Leukemia Treatment - Chronic Lymphocytic Leukemia Treatment - Hairy Cell Leukemia Treatment There are different subtypes of AML. Most AML subtypes are based on how mature (developed) the cancer cells are at the time of diagnosis and how different they are from normal cells. Acute promyelocytic leukemia (APL) is a subtype of AML that occurs when parts of two genes stick together. APL usually occurs in middle-aged adults. Signs of APL may include both bleeding and forming blood clots.",Adult Acute Myeloid Leukemia,0000001_2,CancerGov,https://www.cancer.gov/types/leukemia/patient/adult-aml-treatment-pdq,C0220615,T191,Disorders Who is at risk for Adult Acute Myeloid Leukemia? ?,0000001_2-2,susceptibility,"Smoking, previous chemotherapy treatment, and exposure to radiation may affect the risk of adult AML. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesnt mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Possible risk factors for AML include the following: - Being male. - Smoking, especially after age 60. - Having had treatment with chemotherapy or radiation therapy in the past. - Having had treatment for childhood acute lymphoblastic leukemia (ALL) in the past. - Being exposed to radiation from an atomic bomb or to the chemical benzene. - Having a history of a blood disorder such as myelodysplastic syndrome.",Adult Acute Myeloid Leukemia,0000001_2,CancerGov,https://www.cancer.gov/types/leukemia/patient/adult-aml-treatment-pdq,C0220615,T191,Disorders What are the symptoms of Adult Acute Myeloid Leukemia ?,0000001_2-3,symptoms,"Signs and symptoms of adult AML include fever, feeling tired, and easy bruising or bleeding. The early signs and symptoms of AML may be like those caused by the flu or other common diseases. Check with your doctor if you have any of the following: - Fever. - Shortness of breath. - Easy bruising or bleeding. - Petechiae (flat, pinpoint spots under the skin caused by bleeding). - Weakness or feeling tired. - Weight loss or loss of appetite.",Adult Acute Myeloid Leukemia,0000001_2,CancerGov,https://www.cancer.gov/types/leukemia/patient/adult-aml-treatment-pdq,C0220615,T191,Disorders How to diagnose Adult Acute Myeloid Leukemia ?,0000001_2-4,exams and tests,"Tests that examine the blood and bone marrow are used to detect (find) and diagnose adult AML. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following: - The number of red blood cells, white blood cells, and platelets. - The amount of hemoglobin (the protein that carries oxygen) in the red blood cells. - The portion of the sample made up of red blood cells. - Peripheral blood smear : A procedure in which a sample of blood is checked for blast cells, the number and kinds of white blood cells, the number of platelets, and changes in the shape of blood cells. - Bone marrow aspiration and biopsy : The removal of bone marrow, blood, and a small piece of bone by inserting a hollow needle into the hipbone or breastbone. A pathologist views the bone marrow, blood, and bone under a microscope to look for signs of cancer. - Cytogenetic analysis : A laboratory test in which the cells in a sample of blood or bone marrow are viewed under a microscope to look for certain changes in the chromosomes. Other tests, such as fluorescence in situ hybridization (FISH), may also be done to look for certain changes in the chromosomes. - Immunophenotyping : A process used to identify cells, based on the types of antigens or markers on the surface of the cell. This process is used to diagnose the subtype of AML by comparing the cancer cells to normal cells of the immune system. For example, a cytochemistry study may test the cells in a sample of tissue using chemicals (dyes) to look for certain changes in the sample. A chemical may cause a color change in one type of leukemia cell but not in another type of leukemia cell. - Reverse transcriptionpolymerase chain reaction test (RTPCR): A laboratory test in which cells in a sample of tissue are studied using chemicals to look for certain changes in the structure or function of genes. This test is used to diagnose certain types of AML including acute promyelocytic leukemia (APL).",Adult Acute Myeloid Leukemia,0000001_2,CancerGov,https://www.cancer.gov/types/leukemia/patient/adult-aml-treatment-pdq,C0220615,T191,Disorders What is the outlook for Adult Acute Myeloid Leukemia ?,0000001_2-5,outlook,Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on: - The age of the patient. - The subtype of AML. - Whether the patient received chemotherapy in the past to treat a different cancer. - Whether there is a history of a blood disorder such as myelodysplastic syndrome. - Whether the cancer has spread to the central nervous system. - Whether the cancer has been treated before or recurred (come back). It is important that acute leukemia be treated right away.,Adult Acute Myeloid Leukemia,0000001_2,CancerGov,https://www.cancer.gov/types/leukemia/patient/adult-aml-treatment-pdq,C0220615,T191,Disorders What are the stages of Adult Acute Myeloid Leukemia ?,0000001_2-6,stages,"Key Points - Once adult acute myeloid leukemia (AML) has been diagnosed, tests are done to find out if the cancer has spread to other parts of the body. - There is no standard staging system for adult AML. Once adult acute myeloid leukemia (AML) has been diagnosed, tests are done to find out if the cancer has spread to other parts of the body. The extent or spread of cancer is usually described as stages. In adult acute myeloid leukemia (AML), the subtype of AML and whether the leukemia has spread outside the blood and bone marrow are used instead of the stage to plan treatment. The following tests and procedures may be used to determine if the leukemia has spread: - Lumbar puncture : A procedure used to collect a sample of cerebrospinal fluid (CSF) from the spinal column. This is done by placing a needle between two bones in the spine and into the CSF around the spinal cord and removing a sample of the fluid. The sample of CSF is checked under a microscope for signs that leukemia cells have spread to the brain and spinal cord. This procedure is also called an LP or spinal tap. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of the abdomen, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. There is no standard staging system for adult AML. The disease is described as untreated, in remission, or recurrent. Untreated adult AML In untreated adult AML, the disease is newly diagnosed. It has not been treated except to relieve signs and symptoms such as fever, bleeding, or pain, and the following are true: - The complete blood count is abnormal. - At least 20% of the cells in the bone marrow are blasts (leukemia cells). - There are signs or symptoms of leukemia. Adult AML in remission In adult AML in remission, the disease has been treated and the following are true: - The complete blood count is normal. - Less than 5% of the cells in the bone marrow are blasts (leukemia cells). - There are no signs or symptoms of leukemia in the brain and spinal cord or elsewhere in the body. Recurrent Adult AML Recurrent AML is cancer that has recurred (come back) after it has been treated. The AML may come back in the blood or bone marrow.",Adult Acute Myeloid Leukemia,0000001_2,CancerGov,https://www.cancer.gov/types/leukemia/patient/adult-aml-treatment-pdq,C0220615,T191,Disorders What are the treatments for Adult Acute Myeloid Leukemia ?,0000001_2-7,treatment,"Key Points - There are different types of treatment for patients with adult acute myeloid leukemia. - The treatment of adult AML usually has 2 phases. - Four types of standard treatment are used: - Chemotherapy - Radiation therapy - Stem cell transplant - Other drug therapy - New types of treatment are being tested in clinical trials. - Targeted therapy - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with adult acute myeloid leukemia. Different types of treatment are available for patients with adult acute myeloid leukemia (AML). Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. The treatment of adult AML usually has 2 phases. The 2 treatment phases of adult AML are: - Remission induction therapy: This is the first phase of treatment. The goal is to kill the leukemia cells in the blood and bone marrow. This puts the leukemia into remission. - Post-remission therapy: This is the second phase of treatment. It begins after the leukemia is in remission. The goal of post-remission therapy is to kill any remaining leukemia cells that may not be active but could begin to regrow and cause a relapse. This phase is also called remission continuation therapy. Four types of standard treatment are used: Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid (intrathecal chemotherapy), an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Intrathecal chemotherapy may be used to treat adult AML that has spread to the brain and spinal cord. Combination chemotherapy is treatment using more than one anticancer drug. The way the chemotherapy is given depends on the subtype of AML being treated and whether leukemia cells have spread to the brain and spinal cord. See Drugs Approved for Acute Myeloid Leukemia for more information. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type of cancer being treated and whether leukemia cells have spread to the brain and spinal cord. External radiation therapy is used to treat adult AML. Stem cell transplant Stem cell transplant is a method of giving chemotherapy and replacing blood -forming cells that are abnormal or destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. Other drug therapy Arsenic trioxide and all-trans retinoic acid (ATRA) are anticancer drugs that kill leukemia cells, stop the leukemia cells from dividing, or help the leukemia cells mature into white blood cells. These drugs are used in the treatment of a subtype of AML called acute promyelocytic leukemia. See Drugs Approved for Acute Myeloid Leukemia for more information. New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Monoclonal antibody therapy is one type of targeted therapy being studied in the treatment of adult AML. Monoclonal antibody therapy is a cancer treatment that uses antibodies made in the laboratory from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Adult Acute Myeloid Leukemia Untreated Adult Acute Myeloid Leukemia Standard treatment of untreated adult acute myeloid leukemia (AML) during the remission induction phase depends on the subtype of AML and may include the following: - Combination chemotherapy. - High-dose combination chemotherapy. - Low-dose chemotherapy. - Intrathecal chemotherapy. - All-trans retinoic acid (ATRA) plus arsenic trioxide for the treatment of acute promyelocytic leukemia (APL). - ATRA plus combination chemotherapy followed by arsenic trioxide for the treatment of APL. Check the list of NCI-supported cancer clinical trials that are now accepting patients with untreated adult acute myeloid leukemia. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Adult Acute Myeloid Leukemia in Remission Treatment of adult AML during the remission phase depends on the subtype of AML and may include the following: - Combination chemotherapy. - High-dose chemotherapy, with or without radiation therapy, and stem cell transplant using the patient's stem cells. - High-dose chemotherapy and stem cell transplant using donor stem cells. - A clinical trial of arsenic trioxide. Check the list of NCI-supported cancer clinical trials that are now accepting patients with adult acute myeloid leukemia in remission. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Recurrent Adult Acute Myeloid Leukemia There is no standard treatment for recurrent adult AML. Treatment depends on the subtype of AML and may include the following: - Combination chemotherapy. - Targeted therapy with monoclonal antibodies. - Stem cell transplant. - Arsenic trioxide therapy. - A clinical trial of arsenic trioxide therapy followed by stem cell transplant. Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent adult acute myeloid leukemia. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Adult Acute Myeloid Leukemia,0000001_2,CancerGov,https://www.cancer.gov/types/leukemia/patient/adult-aml-treatment-pdq,C0220615,T191,Disorders what research (or clinical trials) is being done for Adult Acute Myeloid Leukemia ?,0000001_2-8,research,"New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Monoclonal antibody therapy is one type of targeted therapy being studied in the treatment of adult AML. Monoclonal antibody therapy is a cancer treatment that uses antibodies made in the laboratory from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Adult Acute Myeloid Leukemia,0000001_2,CancerGov,https://www.cancer.gov/types/leukemia/patient/adult-aml-treatment-pdq,C0220615,T191,Disorders What is (are) Chronic Lymphocytic Leukemia ?,0000001_3-1,information,"Key Points - Chronic lymphocytic leukemia is a type of cancer in which the bone marrow makes too many lymphocytes (a type of white blood cell). - Leukemia may affect red blood cells, white blood cells, and platelets. - Older age can affect the risk of developing chronic lymphocytic leukemia. - Signs and symptoms of chronic lymphocytic leukemia include swollen lymph nodes and tiredness. - Tests that examine the blood, bone marrow, and lymph nodes are used to detect (find) and diagnose chronic lymphocytic leukemia. - Certain factors affect treatment options and prognosis (chance of recovery). Chronic lymphocytic leukemia is a type of cancer in which the bone marrow makes too many lymphocytes (a type of white blood cell). Chronic lymphocytic leukemia (also called CLL) is a blood and bone marrow disease that usually gets worse slowly. CLL is one of the most common types of leukemia in adults. It often occurs during or after middle age; it rarely occurs in children. Leukemia may affect red blood cells, white blood cells, and platelets. Normally, the body makes blood stem cells (immature cells) that become mature blood cells over time. A blood stem cell may become a myeloid stem cell or a lymphoid stem cell. A myeloid stem cell becomes one of three types of mature blood cells: - Red blood cells that carry oxygen and other substances to all tissues of the body. - White blood cells that fight infection and disease. - Platelets that form blood clots to stop bleeding. A lymphoid stem cell becomes a lymphoblast cell and then one of three types of lymphocytes (white blood cells): - B lymphocytes that make antibodies to help fight infection. - T lymphocytes that help B lymphocytes make antibodies to fight infection. - Natural killer cells that attack cancer cells and viruses. In CLL, too many blood stem cells become abnormal lymphocytes and do not become healthy white blood cells. The abnormal lymphocytes may also be called leukemia cells. The lymphocytes are not able to fight infection very well. Also, as the number of lymphocytes increases in the blood and bone marrow, there is less room for healthy white blood cells, red blood cells, and platelets. This may cause infection, anemia, and easy bleeding. This summary is about chronic lymphocytic leukemia. See the following PDQ summaries for more information about leukemia: - Adult Acute Lymphoblastic Leukemia Treatment. - Childhood Acute Lymphoblastic Leukemia Treatment. - Adult Acute Myeloid Leukemia Treatment. - Childhood Acute Myeloid Leukemia/Other Myeloid Malignancies Treatment. - Chronic Myelogenous Leukemia Treatment. - Hairy Cell Leukemia Treatment.",Chronic Lymphocytic Leukemia,0000001_3,CancerGov,https://www.cancer.gov/types/leukemia/patient/cll-treatment-pdq,C0023434,T191,Disorders Who is at risk for Chronic Lymphocytic Leukemia? ?,0000001_3-2,susceptibility,"Older age can affect the risk of developing chronic lymphocytic leukemia. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesnt mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for CLL include the following: - Being middle-aged or older, male, or white. - A family history of CLL or cancer of the lymph system. - Having relatives who are Russian Jews or Eastern European Jews.",Chronic Lymphocytic Leukemia,0000001_3,CancerGov,https://www.cancer.gov/types/leukemia/patient/cll-treatment-pdq,C0023434,T191,Disorders What are the symptoms of Chronic Lymphocytic Leukemia ?,0000001_3-3,symptoms,"Signs and symptoms of chronic lymphocytic leukemia include swollen lymph nodes and tiredness. Usually CLL does not cause any signs or symptoms and is found during a routine blood test. Signs and symptoms may be caused by CLL or by other conditions. Check with your doctor if you have any of the following: - Painless swelling of the lymph nodes in the neck, underarm, stomach, or groin. - Feeling very tired. - Pain or fullness below the ribs. - Fever and infection. - Weight loss for no known reason.",Chronic Lymphocytic Leukemia,0000001_3,CancerGov,https://www.cancer.gov/types/leukemia/patient/cll-treatment-pdq,C0023434,T191,Disorders How to diagnose Chronic Lymphocytic Leukemia ?,0000001_3-4,exams and tests,"Tests that examine the blood, bone marrow, and lymph nodes are used to detect (find) and diagnose chronic lymphocytic leukemia. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Complete blood count (CBC) with differential : A procedure in which a sample of blood is drawn and checked for the following: - The number of red blood cells and platelets. - The number and type of white blood cells. - The amount of hemoglobin (the protein that carries oxygen) in the red blood cells. - The portion of the blood sample made up of red blood cells. - Immunophenotyping : A laboratory test in which the antigens or markers on the surface of a blood or bone marrow cell are checked to see if they are lymphocytes or myeloid cells. If the cells are malignant lymphocytes (cancer), they are checked to see if they are B lymphocytes or T lymphocytes. - FISH (fluorescence in situ hybridization): A laboratory technique used to look at genes or chromosomes in cells and tissues. Pieces of DNA that contain a fluorescent dye are made in the laboratory and added to cells or tissues on a glass slide. When these pieces of DNA bind to specific genes or areas of chromosomes on the slide, they light up when viewed under a microscope with a special light. - Flow cytometry : A laboratory test that measures the number of cells in a sample, the percentage of live cells in a sample, and certain characteristics of cells, such as size, shape, and the presence of tumor markers on the cell surface. The cells are stained with a light-sensitive dye, placed in a fluid, and passed in a stream before a laser or other type of light. The measurements are based on how the light-sensitive dye reacts to the light. - IgVH gene mutation test: A laboratory test done on a bone marrow or blood sample to check for an IgVH gene mutation. Patients with an IgVH gene mutation have a better prognosis. - Bone marrow aspiration and biopsy : The removal of bone marrow, blood, and a small piece of bone by inserting a hollow needle into the hipbone or breastbone. A pathologist views the bone marrow, blood, and bone under a microscope to look for abnormal cells.",Chronic Lymphocytic Leukemia,0000001_3,CancerGov,https://www.cancer.gov/types/leukemia/patient/cll-treatment-pdq,C0023434,T191,Disorders What is the outlook for Chronic Lymphocytic Leukemia ?,0000001_3-5,outlook,"Certain factors affect treatment options and prognosis (chance of recovery). Treatment options depend on: - The stage of the disease. - Red blood cell, white blood cell, and platelet blood counts. - Whether there are signs or symptoms, such as fever, chills, or weight loss. - Whether the liver, spleen, or lymph nodes are larger than normal. - The response to initial treatment. - Whether the CLL has recurred (come back). The prognosis (chance of recovery) depends on: - Whether there is a change in the DNA and the type of change, if there is one. - Whether lymphocytes are spread throughout the bone marrow. - The stage of the disease. - Whether the CLL gets better with treatment or has recurred (come back). - Whether the CLL progresses to lymphoma or prolymphocytic leukemia. - The patient's general health.",Chronic Lymphocytic Leukemia,0000001_3,CancerGov,https://www.cancer.gov/types/leukemia/patient/cll-treatment-pdq,C0023434,T191,Disorders what research (or clinical trials) is being done for Chronic Lymphocytic Leukemia ?,0000001_3-6,research,"New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Chemotherapy with stem cell transplant Chemotherapy with stem cell transplant is a method of giving chemotherapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the bodys blood cells. Biologic therapy Biologic therapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy. Chimeric antigen receptor (CAR) T-cell therapy CAR T-cell therapy is a type of immunotherapy that changes the patient's T cells (a type of immune system cell) so they will attack certain proteins on the surface of cancer cells. T cells are taken from the patient and special receptors are added to their surface in the laboratory. The changed cells are called chimeric antigen receptor (CAR) T cells. The CAR T cells are grown in the laboratory and given to the patient by infusion. The CAR T cells multiply in the patient's blood and attack cancer cells. CAR T-cell therapy is being studied in the treatment of chronic lymphocytic leukemia. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Chronic Lymphocytic Leukemia,0000001_3,CancerGov,https://www.cancer.gov/types/leukemia/patient/cll-treatment-pdq,C0023434,T191,Disorders What are the treatments for Chronic Lymphocytic Leukemia ?,0000001_3-8,treatment,"Key Points - There are different types of treatment for patients with chronic lymphocytic leukemia. - Five types of standard treatment are used: - Watchful waiting - Radiation therapy - Chemotherapy - Surgery - Targeted therapy - New types of treatment are being tested in clinical trials. - Chemotherapy with stem cell transplant - Biologic therapy - Chimeric antigen receptor (CAR) T-cell therapy - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with chronic lymphocytic leukemia. Different types of treatment are available for patients with chronic lymphocytic leukemia. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Five types of standard treatment are used: Watchful waiting Watchful waiting is closely monitoring a patients condition without giving any treatment until signs or symptoms appear or change. This is also called observation. During this time, problems caused by the disease, such as infection, are treated. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type of cancer being treated. External radiation therapy is used to treat chronic lymphocytic leukemia. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, or the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. See Drugs Approved for Chronic Lymphocytic Leukemia for more information. Surgery Splenectomy is surgery to remove the spleen. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Monoclonal antibody therapy, tyrosine kinase inhibitor therapy, and BCL2 inhibitor therapy are types of targeted therapy used in the treatment of chronic lymphocytic leukemia. Monoclonal antibody therapy is a cancer treatment that uses antibodies made in the laboratory from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances in the body that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Tyrosine kinase inhibitor therapy is a cancer treatment that blocks signals needed for tumors to grow. BCL2 inhibitor therapy is a cancer treatment that blocks a protein called BCL2. BCL2 inhibitor therapy may kill cancer cells and may make them more sensitive to other anticancer drugs. See Drugs Approved for Chronic Lymphocytic Leukemia for more information. New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Chemotherapy with stem cell transplant Chemotherapy with stem cell transplant is a method of giving chemotherapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the bodys blood cells. Biologic therapy Biologic therapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy. Chimeric antigen receptor (CAR) T-cell therapy CAR T-cell therapy is a type of immunotherapy that changes the patient's T cells (a type of immune system cell) so they will attack certain proteins on the surface of cancer cells. T cells are taken from the patient and special receptors are added to their surface in the laboratory. The changed cells are called chimeric antigen receptor (CAR) T cells. The CAR T cells are grown in the laboratory and given to the patient by infusion. The CAR T cells multiply in the patient's blood and attack cancer cells. CAR T-cell therapy is being studied in the treatment of chronic lymphocytic leukemia. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options by Stage Stage 0 Chronic Lymphocytic Leukemia Treatment of stage 0 chronic lymphocytic leukemia is usually watchful waiting. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage 0 chronic lymphocytic leukemia. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage I, Stage II, Stage III, and Stage IV Chronic Lymphocytic Leukemia Treatment of stage I, stage II, stage III, and stage IV chronic lymphocytic leukemia may include the following: - Watchful waiting when there are few or no signs or symptoms. - Targeted therapy with a monoclonal antibody, a tyrosine kinase inhibitor, or a BCL2 inhibitor. - Chemotherapy with 1 or more drugs, with or without steroids or monoclonal antibody therapy. - Low-dose external radiation therapy to areas of the body where cancer is found, such as the spleen or lymph nodes. - A clinical trial of chemotherapy and biologic therapy with stem cell transplant. - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I chronic lymphocytic leukemia, stage II chronic lymphocytic leukemia, stage III chronic lymphocytic leukemia and stage IV chronic lymphocytic leukemia. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Chronic Lymphocytic Leukemia,0000001_3,CancerGov,https://www.cancer.gov/types/leukemia/patient/cll-treatment-pdq,C0023434,T191,Disorders What is (are) Chronic Myelogenous Leukemia ?,0000001_4-1,information,"Key Points - Chronic myelogenous leukemia is a disease in which the bone marrow makes too many white blood cells. - Leukemia may affect red blood cells, white blood cells, and platelets. - Signs and symptoms of chronic myelogenous leukemia include fever, night sweats, and tiredness. - Most people with CML have a gene mutation (change) called the Philadelphia chromosome. - Tests that examine the blood and bone marrow are used to detect (find) and diagnose chronic myelogenous leukemia. - Certain factors affect prognosis (chance of recovery) and treatment options. Chronic myelogenous leukemia is a disease in which the bone marrow makes too many white blood cells. Chronic myelogenous leukemia (also called CML or chronic granulocytic leukemia) is a slowly progressing blood and bone marrow disease that usually occurs during or after middle age, and rarely occurs in children. Leukemia may affect red blood cells, white blood cells, and platelets. Normally, the bone marrow makes blood stem cells (immature cells) that become mature blood cells over time. A blood stem cell may become a myeloid stem cell or a lymphoid stem cell. A lymphoid stem cell becomes a white blood cell. A myeloid stem cell becomes one of three types of mature blood cells: - Red blood cells that carry oxygen and other substances to all tissues of the body. - Platelets that form blood clots to stop bleeding. - Granulocytes (white blood cells) that fight infection and disease. In CML, too many blood stem cells become a type of white blood cell called granulocytes. These granulocytes are abnormal and do not become healthy white blood cells. They are also called leukemia cells. The leukemia cells can build up in the blood and bone marrow so there is less room for healthy white blood cells, red blood cells, and platelets. When this happens, infection, anemia, or easy bleeding may occur. This summary is about chronic myelogenous leukemia. See the following PDQ summaries for more information about leukemia: - Adult Acute Lymphoblastic Leukemia Treatment - Childhood Acute Lymphoblastic Leukemia Treatment - Adult Acute Myeloid Leukemia Treatment - Childhood Acute Myeloid Leukemia/Other Myeloid Malignancies Treatment - Chronic Lymphocytic Leukemia Treatment - Hairy Cell Leukemia Treatment",Chronic Myelogenous Leukemia,0000001_4,CancerGov,https://www.cancer.gov/types/leukemia/patient/cml-treatment-pdq,C0023473,T191,Disorders What are the symptoms of Chronic Myelogenous Leukemia ?,0000001_4-2,symptoms,"Signs and symptoms of chronic myelogenous leukemia include fever, night sweats, and tiredness. These and other signs and symptoms may be caused by CML or by other conditions. Check with your doctor if you have any of the following: - Feeling very tired. - Weight loss for no known reason. - Night sweats. - Fever. - Pain or a feeling of fullness below the ribs on the left side. Sometimes CML does not cause any symptoms at all.",Chronic Myelogenous Leukemia,0000001_4,CancerGov,https://www.cancer.gov/types/leukemia/patient/cml-treatment-pdq,C0023473,T191,Disorders What are the genetic changes related to Chronic Myelogenous Leukemia ?,0000001_4-3,genetic changes,"Most people with CML have a gene mutation (change) called the Philadelphia chromosome. Every cell in the body contains DNA (genetic material) that determines how the cell looks and acts. DNA is contained inside chromosomes. In CML, part of the DNA from one chromosome moves to another chromosome. This change is called the Philadelphia chromosome. It results in the bone marrow making an enzyme, called tyrosine kinase, that causes too many stem cells to become white blood cells (granulocytes or blasts). The Philadelphia chromosome is not passed from parent to child.",Chronic Myelogenous Leukemia,0000001_4,CancerGov,https://www.cancer.gov/types/leukemia/patient/cml-treatment-pdq,C0023473,T191,Disorders How to diagnose Chronic Myelogenous Leukemia ?,0000001_4-4,exams and tests,"Tests that examine the blood and bone marrow are used to detect (find) and diagnose chronic myelogenous leukemia.. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease such as an enlarged spleen. A history of the patients health habits and past illnesses and treatments will also be taken. - Complete blood count (CBC) with differential : A procedure in which a sample of blood is drawn and checked for the following: - The number of red blood cells and platelets. - The number and type of white blood cells. - The amount of hemoglobin (the protein that carries oxygen) in the red blood cells. - The portion of the blood sample made up of red blood cells. - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. - Bone marrow aspiration and biopsy : The removal of bone marrow, blood, and a small piece of bone by inserting a needle into the hipbone or breastbone. A pathologist views the bone marrow, blood, and bone under a microscope to look for abnormal cells. One of the following tests may be done on the samples of blood or bone marrow tissue that are removed: - Cytogenetic analysis: A test in which cells in a sample of blood or bone marrow are viewed under a microscope to look for certain changes in the chromosomes, such as the Philadelphia chromosome. - FISH (fluorescence in situ hybridization): A laboratory technique used to look at genes or chromosomes in cells and tissues. Pieces of DNA that contain a fluorescent dye are made in the laboratory and added to cells or tissues on a glass slide. When these pieces of DNA bind to specific genes or areas of chromosomes on the slide, they light up when viewed under a microscope with a special light. - Reverse transcriptionpolymerase chain reaction (RTPCR): A laboratory test in which cells in a sample of tissue are studied using chemicals to look for certain changes in the structure or function of genes.",Chronic Myelogenous Leukemia,0000001_4,CancerGov,https://www.cancer.gov/types/leukemia/patient/cml-treatment-pdq,C0023473,T191,Disorders What is the outlook for Chronic Myelogenous Leukemia ?,0000001_4-5,outlook,Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - The patients age. - The phase of CML. - The amount of blasts in the blood or bone marrow. - The size of the spleen at diagnosis. - The patients general health.,Chronic Myelogenous Leukemia,0000001_4,CancerGov,https://www.cancer.gov/types/leukemia/patient/cml-treatment-pdq,C0023473,T191,Disorders What are the stages of Chronic Myelogenous Leukemia ?,0000001_4-6,stages,"Key Points - After chronic myelogenous leukemia has been diagnosed, tests are done to find out if the cancer has spread. - Chronic myelogenous leukemia has 3 phases. - Chronic phase - Accelerated phase - Blastic phase After chronic myelogenous leukemia has been diagnosed, tests are done to find out if the cancer has spread. Staging is the process used to find out how far the cancer has spread. There is no standard staging system for chronic myelogenous leukemia (CML). Instead, the disease is classified by phase: chronic phase, accelerated phase, or blastic phase. It is important to know the phase in order to plan treatment. The information from tests and procedures done to detect (find) and diagnose chronic myelogenous leukemia is also used to plan treatment. Chronic myelogenous leukemia has 3 phases. As the amount of blast cells increases in the blood and bone marrow, there is less room for healthy white blood cells, red blood cells, and platelets. This may result in infections, anemia, and easy bleeding, as well as bone pain and pain or a feeling of fullness below the ribs on the left side. The number of blast cells in the blood and bone marrow and the severity of signs or symptoms determine the phase of the disease. Chronic phase In chronic phase CML, fewer than 10% of the cells in the blood and bone marrow are blast cells. Accelerated phase In accelerated phase CML, 10% to 19% of the cells in the blood and bone marrow are blast cells. Blastic phase In blastic phase CML, 20% or more of the cells in the blood or bone marrow are blast cells. When tiredness, fever, and an enlarged spleen occur during the blastic phase, it is called blast crisis.",Chronic Myelogenous Leukemia,0000001_4,CancerGov,https://www.cancer.gov/types/leukemia/patient/cml-treatment-pdq,C0023473,T191,Disorders what research (or clinical trials) is being done for Chronic Myelogenous Leukemia ?,0000001_4-7,research,"New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Chronic Myelogenous Leukemia,0000001_4,CancerGov,https://www.cancer.gov/types/leukemia/patient/cml-treatment-pdq,C0023473,T191,Disorders What are the treatments for Chronic Myelogenous Leukemia ?,0000001_4-8,treatment,"Key Points - There are different types of treatment for patients with chronic myelogenous leukemia. - Six types of standard treatment are used: - Targeted therapy - Chemotherapy - Biologic therapy - High-dose chemotherapy with stem cell transplant - Donor lymphocyte infusion (DLI) - Surgery - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with chronic myelogenous leukemia. Different types of treatment are available for patients with chronic myelogenous leukemia (CML). Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information about new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Six types of standard treatment are used: Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Tyrosine kinase inhibitors are targeted therapy drugs used to treat chronic myelogenous leukemia. Imatinib mesylate, nilotinib, dasatinib, and ponatinib are tyrosine kinase inhibitors that are used to treat CML. See Drugs Approved for Chronic Myelogenous Leukemia for more information. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. See Drugs Approved for Chronic Myelogenous Leukemia for more information. Biologic therapy Biologic therapy is a treatment that uses the patients immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the bodys natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy. See Drugs Approved for Chronic Myelogenous Leukemia for more information. High-dose chemotherapy with stem cell transplant High-dose chemotherapy with stem cell transplant is a method of giving high doses of chemotherapy and replacing blood-forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the bodys blood cells. See Drugs Approved for Chronic Myelogenous Leukemia for more information. Donor lymphocyte infusion (DLI) Donor lymphocyte infusion (DLI) is a cancer treatment that may be used after stem cell transplant. Lymphocytes (a type of white blood cell) from the stem cell transplant donor are removed from the donors blood and may be frozen for storage. The donors lymphocytes are thawed if they were frozen and then given to the patient through one or more infusions. The lymphocytes see the patients cancer cells as not belonging to the body and attack them. Surgery Splenectomy is surgery to remove the spleen. New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Chronic Myelogenous Leukemia Chronic Phase Chronic Myelogenous Leukemia Treatment of chronic phase chronic myelogenous leukemia may include the following: - Targeted therapy with a tyrosine kinase inhibitor. - High-dose chemotherapy with donor stem cell transplant. - Chemotherapy. - Splenectomy. - A clinical trial of lower-dose chemotherapy with donor stem cell transplant. - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with chronic phase chronic myelogenous leukemia. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Accelerated Phase Chronic Myelogenous Leukemia Treatment of accelerated phase chronic myelogenous leukemia may include the following: - Donor stem cell transplant. - Targeted therapy with a tyrosine kinase inhibitor. - Tyrosine kinase inhibitor therapy followed by a donor stem cell transplant. - Biologic therapy (interferon) with or without chemotherapy. - High-dose chemotherapy. - Chemotherapy. - Transfusion therapy to replace red blood cells, platelets, and sometimes white blood cells, to relieve symptoms and improve quality of life. - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with accelerated phase chronic myelogenous leukemia. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Blastic Phase Chronic Myelogenous Leukemia Treatment of blastic phase chronic myelogenous leukemia may include the following: - Targeted therapy with a tyrosine kinase inhibitor. - Chemotherapy using one or more drugs. - High-dose chemotherapy. - Donor stem cell transplant. - Chemotherapy as palliative therapy to relieve symptoms and improve quality of life. - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with blastic phase chronic myelogenous leukemia. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Relapsed Chronic Myelogenous Leukemia Treatment of relapsed chronic myelogenous leukemia may include the following: - Targeted therapy with a tyrosine kinase inhibitor. - Donor stem cell transplant. - Chemotherapy. - Donor lymphocyte infusion. - Biologic therapy (interferon). - A clinical trial of new types or higher doses of targeted therapy or donor stem cell transplant. Check the list of NCI-supported cancer clinical trials that are now accepting patients with relapsing chronic myelogenous leukemia. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Chronic Myelogenous Leukemia,0000001_4,CancerGov,https://www.cancer.gov/types/leukemia/patient/cml-treatment-pdq,C0023473,T191,Disorders What is (are) Hairy Cell Leukemia ?,0000001_5-1,information,"Key Points - Hairy cell leukemia is a type of cancer in which the bone marrow makes too many lymphocytes (a type of white blood cell). - Leukemia may affect red blood cells, white blood cells, and platelets. - Gender and age may affect the risk of hairy cell leukemia. - Signs and symptoms of hairy cell leukemia include infections, tiredness, and pain below the ribs. - Tests that examine the blood and bone marrow are used to detect (find) and diagnose hairy cell leukemia. - Certain factors affect treatment options and prognosis (chance of recovery). Hairy cell leukemia is a type of cancer in which the bone marrow makes too many lymphocytes (a type of white blood cell). Hairy cell leukemia is a cancer of the blood and bone marrow. This rare type of leukemia gets worse slowly or does not get worse at all. The disease is called hairy cell leukemia because the leukemia cells look ""hairy"" when viewed under a microscope. Leukemia may affect red blood cells, white blood cells, and platelets. Normally, the bone marrow makes blood stem cells (immature cells) that become mature blood cells over time. A blood stem cell may become a myeloid stem cell or a lymphoid stem cell. A myeloid stem cell becomes one of three types of mature blood cells: - Red blood cells that carry oxygen and other substances to all tissues of the body. - White blood cells that fight infection and disease. - Platelets that form blood clots to stop bleeding. A lymphoid stem cell becomes a lymphoblast cell and then into one of three types of lymphocytes (white blood cells): - B lymphocytes that make antibodies to help fight infection. - T lymphocytes that help B lymphocytes make antibodies to help fight infection. - Natural killer cells that attack cancer cells and viruses. In hairy cell leukemia, too many blood stem cells become lymphocytes. These lymphocytes are abnormal and do not become healthy white blood cells. They are also called leukemia cells. The leukemia cells can build up in the blood and bone marrow so there is less room for healthy white blood cells, red blood cells, and platelets. This may cause infection, anemia, and easy bleeding. Some of the leukemia cells may collect in the spleen and cause it to swell. This summary is about hairy cell leukemia. See the following PDQ summaries for information about other types of leukemia: - Adult Acute Lymphoblastic Leukemia Treatment. - Childhood Acute Lymphoblastic Leukemia Treatment. - Chronic Lymphocytic Leukemia Treatment. - Adult Acute Myeloid Leukemia Treatment. - Childhood Acute Myeloid Leukemia/Other Myeloid Malignancies Treatment. - Chronic Myelogenous Leukemia Treatment.",Hairy Cell Leukemia,0000001_5,CancerGov,https://www.cancer.gov/types/leukemia/patient/hairy-cell-treatment-pdq,C0023443,T191,Disorders Who is at risk for Hairy Cell Leukemia? ?,0000001_5-2,susceptibility,Gender and age may affect the risk of hairy cell leukemia. Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesnt mean that you will not get cancer. Talk with your doctor if you think you may be at risk. The cause of hairy cell leukemia is unknown. It occurs more often in older men.,Hairy Cell Leukemia,0000001_5,CancerGov,https://www.cancer.gov/types/leukemia/patient/hairy-cell-treatment-pdq,C0023443,T191,Disorders What are the symptoms of Hairy Cell Leukemia ?,0000001_5-3,symptoms,"Signs and symptoms of hairy cell leukemia include infections, tiredness, and pain below the ribs. These and other signs and symptoms may be caused by hairy cell leukemia or by other conditions. Check with your doctor if you have any of the following: - Weakness or feeling tired. - Fever or frequent infections. - Easy bruising or bleeding. - Shortness of breath. - Weight loss for no known reason. - Pain or a feeling of fullness below the ribs. - Painless lumps in the neck, underarm, stomach, or groin.",Hairy Cell Leukemia,0000001_5,CancerGov,https://www.cancer.gov/types/leukemia/patient/hairy-cell-treatment-pdq,C0023443,T191,Disorders How to diagnose Hairy Cell Leukemia ?,0000001_5-4,exams and tests,"Tests that examine the blood and bone marrow are used to detect (find) and diagnose hairy cell leukemia. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as a swollen spleen, lumps, or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following: - The number of red blood cells, white blood cells, and platelets. - The amount of hemoglobin (the protein that carries oxygen) in the red blood cells. - The portion of the sample made up of red blood cells. - Peripheral blood smear : A procedure in which a sample of blood is checked for cells that look ""hairy,"" the number and kinds of white blood cells, the number of platelets, and changes in the shape of blood cells. - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. - Bone marrow aspiration and biopsy : The removal of bone marrow, blood, and a small piece of bone by inserting a hollow needle into the hipbone or breastbone. A pathologist views the bone marrow, blood, and bone under a microscope to look for signs of cancer. - Immunophenotyping : A laboratory test in which the antigens or markers on the surface of a blood or bone marrow cell are checked to see what type of cell it is. This test is done to diagnose the specific type of leukemia by comparing the cancer cells to normal cells of the immune system. - Flow cytometry : A laboratory test that measures the number of cells in a sample, the percentage of live cells in a sample, and certain characteristics of cells, such as size, shape, and the presence of tumor markers on the cell surface. The cells are stained with a light-sensitive dye, placed in a fluid, and passed in a stream before a laser or other type of light. The measurements are based on how the light-sensitive dye reacts to the light. - Cytogenetic analysis : A laboratory test in which cells in a sample of tissue are viewed under a microscope to look for certain changes in the chromosomes. - Gene mutation test: A laboratory test done on a bone marrow or blood sample to check for mutations in the BRAF gene. A BRAF gene mutation is often found in patients with hairy cell leukemia. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. A CT scan of the abdomen may be done to check for swollen lymph nodes or a swollen spleen.",Hairy Cell Leukemia,0000001_5,CancerGov,https://www.cancer.gov/types/leukemia/patient/hairy-cell-treatment-pdq,C0023443,T191,Disorders What is the outlook for Hairy Cell Leukemia ?,0000001_5-5,outlook,"Certain factors affect treatment options and prognosis (chance of recovery). The treatment options may depend on the following: - The number of hairy (leukemia) cells and healthy blood cells in the blood and bone marrow. - Whether the spleen is swollen. - Whether there are signs or symptoms of leukemia, such as infection. - Whether the leukemia has recurred (come back) after previous treatment. The prognosis (chance of recovery) depends on the following: - Whether the hairy cell leukemia does not grow or grows so slowly it does not need treatment. - Whether the hairy cell leukemia responds to treatment. Treatment often results in a long-lasting remission (a period during which some or all of the signs and symptoms of the leukemia are gone). If the leukemia returns after it has been in remission, retreatment often causes another remission.",Hairy Cell Leukemia,0000001_5,CancerGov,https://www.cancer.gov/types/leukemia/patient/hairy-cell-treatment-pdq,C0023443,T191,Disorders What are the stages of Hairy Cell Leukemia ?,0000001_5-6,stages,"Key Points - There is no standard staging system for hairy cell leukemia. There is no standard staging system for hairy cell leukemia. Staging is the process used to find out how far the cancer has spread. Groups are used in place of stages for hairy cell leukemia. The disease is grouped as untreated, progressive, or refractory. Untreated hairy cell leukemia The hairy cell leukemia is newly diagnosed and has not been treated except to relieve signs or symptoms such as weight loss and infections. In untreated hairy cell leukemia, some or all of the following conditions occur: - Hairy (leukemia) cells are found in the blood and bone marrow. - The number of red blood cells, white blood cells, or platelets may be lower than normal. - The spleen may be larger than normal. Progressive hairy cell leukemia In progressive hairy cell leukemia, the leukemia has been treated with either chemotherapy or splenectomy (removal of the spleen) and one or both of the following conditions occur: - There is an increase in the number of hairy cells in the blood or bone marrow. - The number of red blood cells, white blood cells, or platelets in the blood is lower than normal.",Hairy Cell Leukemia,0000001_5,CancerGov,https://www.cancer.gov/types/leukemia/patient/hairy-cell-treatment-pdq,C0023443,T191,Disorders what research (or clinical trials) is being done for Hairy Cell Leukemia ?,0000001_5-7,research,"New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Hairy Cell Leukemia,0000001_5,CancerGov,https://www.cancer.gov/types/leukemia/patient/hairy-cell-treatment-pdq,C0023443,T191,Disorders What are the treatments for Hairy Cell Leukemia ?,0000001_5-8,treatment,"Key Points - There are different types of treatment for patients with hairy cell leukemia. - Five types of standard treatment are used: - Watchful waiting - Chemotherapy - Biologic therapy - Surgery - Targeted therapy - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with hairy cell leukemia. Different types of treatment are available for patients with hairy cell leukemia. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Five types of standard treatment are used: Watchful waiting Watchful waiting is closely monitoring a patient's condition, without giving any treatment until signs or symptoms appear or change. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. Cladribine and pentostatin are anticancer drugs commonly used to treat hairy cell leukemia. These drugs may increase the risk of other types of cancer, especially Hodgkin lymphoma and non-Hodgkin lymphoma. Long-term follow up for second cancers is very important. Biologic therapy Biologic therapy is a cancer treatment that uses the patients immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the bodys natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy. Interferon alfa is a biologic agent commonly used to treat hairy cell leukemia. See Drugs Approved for Hairy Cell Leukemia for more information. Surgery Splenectomy is a surgical procedure to remove the spleen. Targeted therapy Targeted therapy is a treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Monoclonal antibody therapy is a type of targeted therapy used to treat hairy cell leukemia. Monoclonal antibody therapy uses antibodies made in the laboratory from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. A monoclonal antibody called rituximab may be used for certain patients with hairy cell leukemia. Other types of targeted therapies are being studied. New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Hairy Cell Leukemia Untreated Hairy Cell Leukemia If the patient's blood cell counts are not too low and there are no signs or symptoms, treatment may not be needed and the patient is carefully watched for changes in his or her condition. If blood cell counts become too low or if signs or symptoms appear, initial treatment may include the following: - Chemotherapy. - Splenectomy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with untreated hairy cell leukemia. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Progressive Hairy Cell Leukemia Treatment for progressive hairy cell leukemia may include the following: - Chemotherapy. - Biologic therapy. - Splenectomy. - A clinical trial of chemotherapy and targeted therapy with a monoclonal antibody (rituximab). Check the list of NCI-supported cancer clinical trials that are now accepting patients with progressive hairy cell leukemia, initial treatment. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Relapsed or Refractory Hairy Cell Leukemia Treatment of relapsed or refractory hairy cell leukemia may include the following: - Chemotherapy. - Biologic therapy. - Targeted therapy with a monoclonal antibody (rituximab). - High-dose chemotherapy. - A clinical trial of a new biologic therapy. - A clinical trial of a new targeted therapy. - A clinical trial of chemotherapy and targeted therapy with a monoclonal antibody (rituximab). Check the list of NCI-supported cancer clinical trials that are now accepting patients with refractory hairy cell leukemia. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Hairy Cell Leukemia,0000001_5,CancerGov,https://www.cancer.gov/types/leukemia/patient/hairy-cell-treatment-pdq,C0023443,T191,Disorders What is (are) Childhood Acute Lymphoblastic Leukemia ?,0000001_6-1,information,"Key Points - Childhood acute lymphoblastic leukemia (ALL) is a type of cancer in which the bone marrow makes too many immature lymphocytes (a type of white blood cell). - Leukemia may affect red blood cells, white blood cells, and platelets. - Past treatment for cancer and certain genetic conditions affect the risk of having childhood ALL. - Signs of childhood ALL include fever and bruising. - Tests that examine the blood and bone marrow are used to detect (find) and diagnose childhood ALL. - Certain factors affect prognosis (chance of recovery) and treatment options. Childhood acute lymphoblastic leukemia (ALL) is a type of cancer in which the bone marrow makes too many immature lymphocytes (a type of white blood cell). Childhood acute lymphoblastic leukemia (also called ALL or acute lymphocytic leukemia) is a cancer of the blood and bone marrow. This type of cancer usually gets worse quickly if it is not treated. ALL is the most common type of cancer in children. Leukemia may affect red blood cells, white blood cells, and platelets. In a healthy child, the bone marrow makes blood stem cells (immature cells) that become mature blood cells over time. A blood stem cell may become a myeloid stem cell or a lymphoid stem cell. A myeloid stem cell becomes one of three types of mature blood cells: - Red blood cells that carry oxygen and other substances to all tissues of the body. - Platelets that form blood clots to stop bleeding. - White blood cells that fight infection and disease. A lymphoid stem cell becomes a lymphoblast cell and then one of three types of lymphocytes (white blood cells): - B lymphocytes that make antibodies to help fight infection. - T lymphocytes that help B lymphocytes make the antibodies that help fight infection. - Natural killer cells that attack cancer cells and viruses. In a child with ALL, too many stem cells become lymphoblasts, B lymphocytes, or T lymphocytes. The cells do not work like normal lymphocytes and are not able to fight infection very well. These cells are cancer (leukemia) cells. Also, as the number of leukemia cells increases in the blood and bone marrow, there is less room for healthy white blood cells, red blood cells, and platelets. This may lead to infection, anemia, and easy bleeding. This summary is about acute lymphoblastic leukemia in children, adolescents, and young adults. See the following PDQ summaries for information about other types of leukemia: - Childhood Acute Myeloid Leukemia/Other Myeloid Malignancies Treatment - Adult Acute Lymphoblastic Leukemia Treatment - Chronic Lymphocytic Leukemia Treatment - Adult Acute Myeloid Leukemia Treatment - Chronic Myelogenous Leukemia Treatment - Hairy Cell Leukemia Treatment",Childhood Acute Lymphoblastic Leukemia,0000001_6,CancerGov,https://www.cancer.gov/types/leukemia/patient/child-all-treatment-pdq,C0023452,T191,Disorders What are the symptoms of Childhood Acute Lymphoblastic Leukemia ?,0000001_6-2,symptoms,"Signs of childhood ALL include fever and bruising. These and other signs and symptoms may be caused by childhood ALL or by other conditions. Check with your child's doctor if your child has any of the following: - Fever. - Easy bruising or bleeding. - Petechiae (flat, pinpoint, dark-red spots under the skin caused by bleeding). - Bone or joint pain. - Painless lumps in the neck, underarm, stomach, or groin. - Pain or feeling of fullness below the ribs. - Weakness, feeling tired, or looking pale. - Loss of appetite.",Childhood Acute Lymphoblastic Leukemia,0000001_6,CancerGov,https://www.cancer.gov/types/leukemia/patient/child-all-treatment-pdq,C0023452,T191,Disorders How to diagnose Childhood Acute Lymphoblastic Leukemia ?,0000001_6-3,exams and tests,"Tests that examine the blood and bone marrow are used to detect (find) and diagnose childhood ALL. The following tests and procedures may be used to diagnose childhood ALL and find out if leukemia cells have spread to other parts of the body such as the brain or testicles: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient's health habits and past illnesses and treatments will also be taken. - Complete blood count (CBC) with differential : A procedure in which a sample of blood is drawn and checked for the following: - The number of red blood cells and platelets. - The number and type of white blood cells. - The amount of hemoglobin (the protein that carries oxygen) in the red blood cells. - The portion of the sample made up of red blood cells. - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. - Bone marrow aspiration and biopsy : The removal of bone marrow and a small piece of bone by inserting a hollow needle into the hipbone or breastbone. A pathologist views the bone marrow and bone under a microscope to look for signs of cancer. The following tests are done on blood or the bone marrow tissue that is removed: - Cytogenetic analysis : A laboratory test in which the cells in a sample of blood or bone marrow are viewed under a microscope to look for certain changes in the chromosomes of lymphocytes. For example, in Philadelphia chromosome positive ALL, part of one chromosome switches places with part of another chromosome. This is called the Philadelphia chromosome. - Immunophenotyping : A laboratory test in which the antigens or markers on the surface of a blood or bone marrow cell are checked to see if they are lymphocytes or myeloid cells. If the cells are malignant lymphocytes (cancer) they are checked to see if they are B lymphocytes or T lymphocytes. - Lumbar puncture : A procedure used to collect a sample of cerebrospinal fluid (CSF) from the spinal column. This is done by placing a needle between two bones in the spine and into the CSF around the spinal cord and removing a sample of the fluid. The sample of CSF is checked under a microscope for signs that leukemia cells have spread to the brain and spinal cord. This procedure is also called an LP or spinal tap. This procedure is done after leukemia is diagnosed to find out if leukemia cells have spread to the brain and spinal cord. Intrathecal chemotherapy is given after the sample of fluid is removed to treat any leukemia cells that may have spread to the brain and spinal cord. - Chest x-ray : An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. The chest x-ray is done to see if leukemia cells have formed a mass in the middle of the chest.",Childhood Acute Lymphoblastic Leukemia,0000001_6,CancerGov,https://www.cancer.gov/types/leukemia/patient/child-all-treatment-pdq,C0023452,T191,Disorders What is the outlook for Childhood Acute Lymphoblastic Leukemia ?,0000001_6-4,outlook,"Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) depends on: - How quickly and how low the leukemia cell count drops after the first month of treatment. - Age at the time of diagnosis, gender, race, and ethnic background. - The number of white blood cells in the blood at the time of diagnosis. - Whether the leukemia cells began from B lymphocytes or T lymphocytes. - Whether there are certain changes in the chromosomes or genes of the lymphocytes with cancer. - Whether the child has Down syndrome. - Whether leukemia cells are found in the cerebrospinal fluid. - The child's weight at the time of diagnosis and during treatment. Treatment options depend on: - Whether the leukemia cells began from B lymphocytes or T lymphocytes. - Whether the child has standard-risk, high-risk, or very highrisk ALL. - The age of the child at the time of diagnosis. - Whether there are certain changes in the chromosomes of lymphocytes, such as the Philadelphia chromosome. - Whether the child was treated with steroids before the start of induction therapy. - How quickly and how low the leukemia cell count drops during treatment. For leukemia that relapses (comes back) after treatment, the prognosis and treatment options depend partly on the following: - How long it is between the time of diagnosis and when the leukemia comes back. - Whether the leukemia comes back in the bone marrow or in other parts of the body.",Childhood Acute Lymphoblastic Leukemia,0000001_6,CancerGov,https://www.cancer.gov/types/leukemia/patient/child-all-treatment-pdq,C0023452,T191,Disorders what research (or clinical trials) is being done for Childhood Acute Lymphoblastic Leukemia ?,0000001_6-5,research,"New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Chimeric antigen receptor (CAR) T-cell therapy CAR T-cell therapy is a type of immunotherapy that changes the patient's T cells (a type of immune system cell) so they will attack certain proteins on the surface of cancer cells. T cells are taken from the patient and special receptors are added to their surface in the laboratory. The changed cells are called chimeric antigen receptor (CAR) T cells. The CAR T cells are grown in the laboratory and given to the patient by infusion. The CAR T cells multiply in the patient's blood and attack cancer cells. CAR T-cell therapy is being studied in the treatment of childhood ALL that has relapsed (come back) a second time. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Childhood Acute Lymphoblastic Leukemia,0000001_6,CancerGov,https://www.cancer.gov/types/leukemia/patient/child-all-treatment-pdq,C0023452,T191,Disorders Who is at risk for Childhood Acute Lymphoblastic Leukemia? ?,0000001_6-6,susceptibility,"Key Points Past treatment for cancer and certain genetic conditions affect the risk of having childhood ALL. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesnt mean that you will not get cancer. Talk with your child's doctor if you think your child may be at risk. Possible risk factors for ALL include the following: - Being exposed to x-rays before birth. - Being exposed to radiation. - Past treatment with chemotherapy. - Having certain genetic conditions, such as: - Down syndrome. - Neurofibromatosis type 1. - Bloom syndrome. - Fanconi anemia. - Ataxia-telangiectasia. - Li-Fraumeni syndrome. - Constitutional mismatch repair deficiency (mutations in certain genes that stop DNA from repairing itself, which leads to the growth of cancers at an early age). - Having certain changes in the chromosomes or genes. Risk Groups - In childhood ALL, risk groups are used to plan treatment. - Relapsed childhood ALL is cancer that has come back after it has been treated. In childhood ALL, risk groups are used to plan treatment. There are three risk groups in childhood ALL. They are described as: - Standard (low) risk: Includes children aged 1 to younger than 10 years who have a white blood cell count of less than 50,000/L at the time of diagnosis. - High risk: Includes children 10 years and older and/or children who have a white blood cell count of 50,000/L or more at the time of diagnosis. - Very high risk: Includes children younger than age 1, children with certain changes in the genes, children who have a slow response to initial treatment, and children who have signs of leukemia after the first 4 weeks of treatment. Other factors that affect the risk group include the following: - Whether the leukemia cells began from B lymphocytes or T lymphocytes. - Whether there are certain changes in the chromosomes or genes of the lymphocytes. - How quickly and how low the leukemia cell count drops after initial treatment. - Whether leukemia cells are found in the cerebrospinal fluid at the time of diagnosis. It is important to know the risk group in order to plan treatment. Children with high-risk or very highrisk ALL usually receive more anticancer drugs and/or higher doses of anticancer drugs than children with standard-risk ALL. Relapsed childhood ALL is cancer that has come back after it has been treated. The leukemia may come back in the blood and bone marrow, brain, spinal cord, testicles, or other parts of the body. Refractory childhood ALL is cancer that does not respond to treatment.",Childhood Acute Lymphoblastic Leukemia,0000001_6,CancerGov,https://www.cancer.gov/types/leukemia/patient/child-all-treatment-pdq,C0023452,T191,Disorders What are the treatments for Childhood Acute Lymphoblastic Leukemia ?,0000001_6-7,treatment,"Key Points - There are different types of treatment for childhood acute lymphoblastic leukemia (ALL). - Children with ALL should have their treatment planned by a team of doctors who are experts in treating childhood leukemia. - Children and adolescents may have treatment-related side effects that appear months or years after treatment for acute lymphoblastic leukemia. - The treatment of childhood ALL usually has three phases. - Four types of standard treatment are used: - Chemotherapy - Radiation therapy - Chemotherapy with stem cell transplant - Targeted therapy - Treatment is given to kill leukemia cells that have spread or may spread to the brain, spinal cord, or testicles. - New types of treatment are being tested in clinical trials. - Chimeric antigen receptor (CAR) T-cell therapy - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for childhood acute lymphoblastic leukemia (ALL). Different types of treatment are available for children with acute lymphoblastic leukemia (ALL). Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment. Children with ALL should have their treatment planned by a team of doctors who are experts in treating childhood leukemia. Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other pediatric health professionals who are experts in treating children with leukemia and who specialize in certain areas of medicine. These may include the following specialists: - Pediatrician. - Hematologist. - Medical oncologist. - Pediatric surgeon. - Radiation oncologist. - Neurologist. - Pathologist. - Radiologist. - Pediatric nurse specialist. - Social worker. - Rehabilitation specialist. - Psychologist. - Child-life specialist. Children and adolescents may have treatment-related side effects that appear months or years after treatment for acute lymphoblastic leukemia. Regular follow-up exams are very important. Treatment can cause side effects long after it has ended. These are called late effects. Late effects of cancer treatment may include: - Physical problems, including problems with the heart, blood vessels, liver, or bones, and fertility. When dexrazoxane is given with chemotherapy drugs called anthracyclines, the risk of late heart effects is lessened. - Changes in mood, feelings, thinking, learning, or memory. Children younger than 4 years who have received radiation therapy to the brain have a higher risk of these effects. - Second cancers (new types of cancer) or other conditions, such as brain tumors, thyroid cancer, acute myeloid leukemia, and myelodysplastic syndrome. Some late effects may be treated or controlled. It is important to talk with your child's doctors about the possible late effects caused by some treatments. See the PDQ summary on Late Effects of Treatment for Childhood Cancer. The treatment of childhood ALL usually has three phases. The treatment of childhood ALL is done in phases: - Remission induction: This is the first phase of treatment. The goal is to kill the leukemia cells in the blood and bone marrow. This puts the leukemia into remission. - Consolidation /intensification: This is the second phase of treatment. It begins once the leukemia is in remission. The goal of consolidation/intensification therapy is to kill any leukemia cells that remain in the body and may cause a relapse. - Maintenance: This is the third phase of treatment. The goal is to kill any remaining leukemia cells that may regrow and cause a relapse. Often the cancer treatments are given in lower doses than those used during the remission induction and consolidation/intensification phases. Not taking medication as ordered by the doctor during maintenance therapy increases the chance the cancer will come back. This is also called the continuation therapy phase. Four types of standard treatment are used: Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid (intrathecal), an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is treatment using more than one anticancer drug. The way the chemotherapy is given depends on the child's risk group. Children with high-risk ALL receive more anticancer drugs and higher doses of anticancer drugs than children with standard-risk ALL. Intrathecal chemotherapy may be used to treat childhood ALL that has spread, or may spread, to the brain and spinal cord. See Drugs Approved for Acute Lymphoblastic Leukemia for more information. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type of cancer being treated. External radiation therapy may be used to treat childhood ALL that has spread, or may spread, to the brain, spinal cord, or testicles. It may also be used to prepare the bone marrow for a stem cell transplant. Chemotherapy with stem cell transplant Stem cell transplant is a method of giving high doses of chemotherapy and sometimes total-body irradiation, and then replacing the blood-forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of a donor. After the patient receives treatment, the donor's stem cells are given to the patient through an infusion. These reinfused stem cells grow into (and restore) the patient's blood cells. The stem cell donor doesn't have to be related to the patient. Stem cell transplant is rarely used as initial treatment for children and adolescents with ALL. It is used more often as part of treatment for ALL that relapses (comes back after treatment). See Drugs Approved for Acute Lymphoblastic Leukemia for more information. Targeted therapy Targeted therapy is a treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Tyrosine kinase inhibitors (TKIs) are targeted therapy drugs that block the enzyme, tyrosine kinase, which causes stem cells to become more white blood cells or blasts than the body needs. Imatinib mesylate is a TKI used in the treatment of children with Philadelphia chromosome positive ALL. Dasatinib and ruxolitinib are TKIs that are being studied in the treatment of newly diagnosed high-risk ALL. Monoclonal antibody therapy is a cancer treatment that uses antibodies made in the laboratory, from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Blinatumomab and inotuzumab are monoclonal antibodies being studied in the treatment of refractory childhood ALL. New kinds of targeted therapies are also being studied in the treatment of childhood ALL. See Drugs Approved for Acute Lymphoblastic Leukemia for more information. Treatment is given to kill leukemia cells that have spread or may spread to the brain, spinal cord, or testicles. Treatment to kill leukemia cells or prevent the spread of leukemia cells to the brain and spinal cord (central nervous system; CNS) is called CNS-directed therapy. Chemotherapy may be used to treat leukemia cells that have spread, or may spread, to the brain and spinal cord. Because standard doses of chemotherapy may not reach leukemia cells in the CNS, the cells are able to hide in the CNS. Systemic chemotherapy given in high doses or intrathecal chemotherapy (into the cerebrospinal fluid) is able to reach leukemia cells in the CNS. Sometimes external radiation therapy to the brain is also given. These treatments are given in addition to treatment that is used to kill leukemia cells in the rest of the body. All children with ALL receive CNS-directed therapy as part of induction therapy and consolidation/intensification therapy and sometimes during maintenance therapy. If the leukemia cells spread to the testicles, treatment includes high doses of systemic chemotherapy and sometimes radiation therapy. New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Chimeric antigen receptor (CAR) T-cell therapy CAR T-cell therapy is a type of immunotherapy that changes the patient's T cells (a type of immune system cell) so they will attack certain proteins on the surface of cancer cells. T cells are taken from the patient and special receptors are added to their surface in the laboratory. The changed cells are called chimeric antigen receptor (CAR) T cells. The CAR T cells are grown in the laboratory and given to the patient by infusion. The CAR T cells multiply in the patient's blood and attack cancer cells. CAR T-cell therapy is being studied in the treatment of childhood ALL that has relapsed (come back) a second time. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Bone marrow aspiration and biopsy is done during all phases of treatment to see how well the treatment is working. Treatment Options for Childhood Acute Lymphoblastic Leukemia Newly Diagnosed Childhood Acute Lymphoblastic Leukemia (Standard Risk) The treatment of standard-risk childhood acute lymphoblastic leukemia (ALL) during the remission induction, consolidation /intensification, and maintenance phases always includes combination chemotherapy. When children are in remission after remission induction therapy, a stem cell transplant using stem cells from a donor may be done. When children are not in remission after remission induction therapy, further treatment is usually the same treatment given to children with high-risk ALL. Intrathecal chemotherapy is given to prevent the spread of leukemia cells to the brain and spinal cord. Treatments being studied in clinical trials for standard-risk ALL include new chemotherapy regimens. Check the list of NCI-supported cancer clinical trials that are now accepting patients with untreated childhood acute lymphoblastic leukemia. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Newly Diagnosed Childhood Acute Lymphoblastic Leukemia (High Risk) The treatment of high-risk childhood acute lymphoblastic leukemia (ALL) during the remission induction, consolidation /intensification, and maintenance phases always includes combination chemotherapy. Children in the high-risk ALL group are given more anticancer drugs and higher doses of anticancer drugs, especially during the consolidation/intensification phase, than children in the standard-risk group. Intrathecal and systemic chemotherapy are given to prevent or treat the spread of leukemia cells to the brain and spinal cord. Sometimes radiation therapy to the brain is also given. Treatments being studied in clinical trials for high-risk ALL include new chemotherapy regimens with or without targeted therapy or stem cell transplant. Check the list of NCI-supported cancer clinical trials that are now accepting patients with untreated childhood acute lymphoblastic leukemia. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Newly Diagnosed Childhood Acute Lymphoblastic Leukemia (Very High Risk) The treatment of very highrisk childhood acute lymphoblastic leukemia (ALL) during the remission induction, consolidation /intensification, and maintenance phases always includes combination chemotherapy. Children in the very highrisk ALL group are given more anticancer drugs than children in the high-risk group. It is not clear whether a stem cell transplant during first remission will help the child live longer. Intrathecal and systemic chemotherapy are given to prevent or treat the spread of leukemia cells to the brain and spinal cord. Sometimes radiation therapy to the brain is also given. Treatments being studied in clinical trials for very highrisk ALL include new chemotherapy regimens with or without targeted therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with untreated childhood acute lymphoblastic leukemia. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Newly Diagnosed Childhood Acute Lymphoblastic Leukemia (Special Groups) T-cell childhood acute lymphoblastic leukemia The treatment of T-cell childhood acute lymphoblastic leukemia (ALL) during the remission induction, consolidation /intensification, and maintenance phases always includes combination chemotherapy. Children with T-cell ALL are given more anticancer drugs and higher doses of anticancer drugs than children in the newly diagnosed standard-risk group. Intrathecal and systemic chemotherapy are given to prevent the spread of leukemia cells to the brain and spinal cord. Sometimes radiation therapy to the brain is also given. Treatments being studied in clinical trials for T-cell ALL include new anticancer agents and chemotherapy regimens with or without targeted therapy. Infants with ALL The treatment of infants with ALL during the remission induction, consolidation /intensification, and maintenance phases always includes combination chemotherapy. Infants with ALL are given different anticancer drugs and higher doses of anticancer drugs than children 1 year and older in the standard-risk group. It is not clear whether a stem cell transplant during first remission will help the child live longer. Intrathecal and systemic chemotherapy are given to prevent the spread of leukemia cells to the brain and spinal cord. Treatments being studied in clinical trials for infants with ALL include the following: - A clinical trial of chemotherapy followed by a donor stem cell transplant for infants with certain gene changes. Children 10 years and older and adolescents with ALL The treatment of ALL in children and adolescents (10 years and older) during the remission induction, consolidation /intensification, and maintenance phases always includes combination chemotherapy. Children 10 years and older and adolescents with ALL are given more anticancer drugs and higher doses of anticancer drugs than children in the standard-risk group. Intrathecal and systemic chemotherapy are given to prevent the spread of leukemia cells to the brain and spinal cord. Sometimes radiation therapy to the brain is also given. Treatments being studied in clinical trials for children 10 years and older and adolescents with ALL include new anticancer agents and chemotherapy regimens with or without targeted therapy. Philadelphia chromosomepositive ALL The treatment of Philadelphia chromosome positive childhood ALL during the remission induction, consolidation /intensification, and maintenance phases may include the following: - Combination chemotherapy and targeted therapy with a tyrosine kinase inhibitor (imatinib mesylate) with or without a stem cell transplant using stem cells from a donor. Check the list of NCI-supported cancer clinical trials that are now accepting patients with T-cell childhood acute lymphoblastic leukemia and Philadelphia chromosome positive childhood precursor acute lymphoblastic leukemia. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Refractory Childhood Acute Lymphoblastic Leukemia There is no standard treatment for the treatment of refractory childhood acute lymphoblastic leukemia (ALL). Some of the treatments being studied in clinical trials for refractory childhood ALL include: - Targeted therapy (blinatumomab or inotuzumab). - Chimeric antigen receptor (CAR) T-cell therapy. Relapsed Childhood Acute Lymphoblastic Leukemia Standard treatment of relapsed childhood acute lymphoblastic leukemia (ALL) that comes back in the bone marrow may include the following: - Combination chemotherapy. - Chemotherapy with or without total-body irradiation followed by a stem cell transplant, using stem cells from a donor. Standard treatment of relapsed childhood acute lymphoblastic leukemia (ALL) that comes back outside the bone marrow may include the following: - Systemic chemotherapy and intrathecal chemotherapy with radiation therapy to the brain and/or spinal cord for cancer that comes back in the brain and spinal cord only. - Combination chemotherapy and radiation therapy for cancer that comes back in the testicles only. - Stem cell transplant for cancer that has recurred in the brain and/or spinal cord. Some of the treatments being studied in clinical trials for relapsed childhood ALL include: - New anticancer drugs and new combination chemotherapy treatments. - Combination chemotherapy and new kinds of targeted therapies (blinatumomab or inotuzumab). - Chimeric antigen receptor (CAR) T-cell therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent childhood acute lymphoblastic leukemia. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website.",Childhood Acute Lymphoblastic Leukemia,0000001_6,CancerGov,https://www.cancer.gov/types/leukemia/patient/child-all-treatment-pdq,C0023452,T191,Disorders What is (are) Childhood Acute Myeloid Leukemia and Other Myeloid Malignancies ?,0000001_7-1,information,"Key Points - Childhood acute myeloid leukemia (AML) is a type of cancer in which the bone marrow makes a large number of abnormal blood cells. - Leukemia and other diseases of the blood and bone marrow may affect red blood cells, white blood cells, and platelets. - Other myeloid diseases can affect the blood and bone marrow. - Chronic myelogenous leukemia - Juvenile myelomonocytic leukemia - Myelodysplastic syndromes - AML or MDS may occur after treatment with certain anticancer drugs and/or radiation therapy. - The risk factors for childhood AML, childhood CML, JMML, and MDS are similar. - Signs and symptoms of childhood AML, childhood CML, JMML, or MDS include fever, feeling tired, and easy bleeding or bruising. - Tests that examine the blood and bone marrow are used to detect (find) and diagnose childhood AML, childhood CML, JMML, and MDS. - Certain factors affect prognosis (chance of recovery) and treatment options. Childhood acute myeloid leukemia (AML) is a type of cancer in which the bone marrow makes a large number of abnormal blood cells. Childhood acute myeloid leukemia (AML) is a cancer of the blood and bone marrow. AML is also called acute myelogenous leukemia, acute myeloblastic leukemia, acute granulocytic leukemia, and acute nonlymphocytic leukemia. Cancers that are acute usually get worse quickly if they are not treated. Cancers that are chronic usually get worse slowly. Leukemia and other diseases of the blood and bone marrow may affect red blood cells, white blood cells, and platelets. Normally, the bone marrow makes blood stem cells (immature cells) that become mature blood cells over time. A blood stem cell may become a myeloid stem cell or a lymphoid stem cell. A lymphoid stem cell becomes a white blood cell. A myeloid stem cell becomes one of three types of mature blood cells: - Red blood cells that carry oxygen and other substances to all tissues of the body. - White blood cells that fight infection and disease. - Platelets that form blood clots to stop bleeding. In AML, the myeloid stem cells usually become a type of immature white blood cell called myeloblasts (or myeloid blasts). The myeloblasts, or leukemia cells, in AML are abnormal and do not become healthy white blood cells. The leukemia cells can build up in the blood and bone marrow so there is less room for healthy white blood cells, red blood cells, and platelets. When this happens, infection, anemia, or easy bleeding may occur. The leukemia cells can spread outside the blood to other parts of the body, including the central nervous system (brain and spinal cord), skin, and gums. Sometimes leukemia cells form a solid tumor called a granulocytic sarcoma or chloroma. There are subtypes of AML based on the type of blood cell that is affected. The treatment of AML is different when it is a subtype called acute promyelocytic leukemia (APL) or when the child has Down syndrome. Other myeloid diseases can affect the blood and bone marrow. Chronic myelogenous leukemia In chronic myelogenous leukemia (CML), too many bone marrow stem cells become a type of white blood cell called granulocytes. Some of these bone marrow stem cells never become mature white blood cells. These are called blasts. Over time, the granulocytes and blasts crowd out the red blood cells and platelets in the bone marrow. CML is rare in children. Juvenile myelomonocytic leukemia Juvenile myelomonocytic leukemia (JMML) is a rare childhood cancer that occurs more often in children around the age of 2 years and is more common in boys. In JMML, too many bone marrow stem cells become 2 types of white blood cells called myelocytes and monocytes. Some of these bone marrow stem cells never become mature white blood cells. These immature cells, called blasts, are unable to do their usual work. Over time, the myelocytes, monocytes, and blasts crowd out the red blood cells and platelets in the bone marrow. When this happens, infection, anemia, or easy bleeding may occur. Myelodysplastic syndromes Myelodysplastic syndromes (MDS) occur less often in children than in adults. In MDS, the bone marrow makes too few red blood cells, white blood cells, and platelets. These blood cells may not mature and enter the blood. The treatment for MDS depends on how low the numbers of red blood cells, white blood cells, or platelets are. Over time, MDS may become AML. Transient myeloproliferative disorder (TMD) is a type of MDS. This disorder of the bone marrow can develop in newborns who have Down syndrome. It usually goes away on its own within the first 3 weeks of life. Infants who have Down syndrome and TMD have an increased chance of developing AML before the age of 3 years. This summary is about childhood AML, childhood CML, JMML, and MDS. See the following PDQ summaries for more information about other types of leukemia and diseases of the blood and bone marrow in children and adults: - Childhood Acute Lymphoblastic Leukemia Treatment - Adult Acute Myeloid Leukemia Treatment - Adult Acute Lymphoblastic Leukemia Treatment - Chronic Myelogenous Leukemia Treatment - Chronic Lymphocytic Leukemia Treatment - Hairy Cell Leukemia Treatment - Myelodysplastic Syndromes Treatment - Myelodysplastic/Myeloproliferative Neoplasms Treatment AML or MDS may occur after treatment with certain anticancer drugs and/or radiation therapy. Cancer treatment with certain anticancer drugs and/or radiation therapy may cause therapy -related AML (t-AML) or therapy-related MDS (t-MDS). The risk of these therapy-related myeloid diseases depends on the total dose of the anticancer drugs used and the radiation dose and treatment field. Some patients also have an inherited risk for t-AML and t-MDS. These therapy-related diseases usually occur within 7 years after treatment, but are rare in children.",Childhood Acute Myeloid Leukemia and Other Myeloid Malignancies,0000001_7,CancerGov,https://www.cancer.gov/types/leukemia/patient/child-aml-treatment-pdq,C0220621,T191,Disorders Who is at risk for Childhood Acute Myeloid Leukemia and Other Myeloid Malignancies? ?,0000001_7-2,susceptibility,"The risk factors for childhood AML, childhood CML, JMML, and MDS are similar. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesnt mean that you will not get cancer. Talk with your childs doctor if you think your child may be at risk. These and other factors may increase the risk of childhood AML, childhood CML, JMML, and MDS: - Having a brother or sister, especially a twin, with leukemia. - Being Hispanic. - Being exposed to cigarette smoke or alcohol before birth. - Having a personal history of aplastic anemia. - Having a personal or family history of MDS. - Having a family history of AML. - Past treatment with chemotherapy or radiation therapy. - Being exposed to ionizing radiation or chemicals such as benzene. - Having certain genetic disorders, such as: - Down syndrome. - Fanconi anemia. - Neurofibromatosis type 1. - Noonan syndrome. - Shwachman-Diamond syndrome.",Childhood Acute Myeloid Leukemia and Other Myeloid Malignancies,0000001_7,CancerGov,https://www.cancer.gov/types/leukemia/patient/child-aml-treatment-pdq,C0220621,T191,Disorders What are the symptoms of Childhood Acute Myeloid Leukemia and Other Myeloid Malignancies ?,0000001_7-3,symptoms,"Signs and symptoms of childhood AML, childhood CML, JMML, or MDS include fever, feeling tired, and easy bleeding or bruising. These and other signs and symptoms may be caused by childhood AML, childhood CML, JMML, or MDS or by other conditions. Check with a doctor if your child has any of the following: - Fever with or without an infection. - Night sweats. - Shortness of breath. - Weakness or feeling tired. - Easy bruising or bleeding. - Petechiae (flat, pinpoint spots under the skin caused by bleeding). - Pain in the bones or joints. - Pain or feeling of fullness below the ribs. - Painless lumps in the neck, underarm, stomach, groin, or other parts of the body. In childhood AML, these lumps, called leukemia cutis, may be blue or purple. - Painless lumps that are sometimes around the eyes. These lumps, called chloromas, are sometimes seen in childhood AML and may be blue-green. - An eczema -like skin rash. The signs and symptoms of TMD may include the following: - Swelling all over the body. - Shortness of breath. - Trouble breathing. - Weakness or feeling tired. - Pain below the ribs.",Childhood Acute Myeloid Leukemia and Other Myeloid Malignancies,0000001_7,CancerGov,https://www.cancer.gov/types/leukemia/patient/child-aml-treatment-pdq,C0220621,T191,Disorders How to diagnose Childhood Acute Myeloid Leukemia and Other Myeloid Malignancies ?,0000001_7-4,exams and tests,"Tests that examine the blood and bone marrow are used to detect (find) and diagnose childhood AML, childhood CML, JMML, and MDS. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Complete blood count (CBC) with differential: A procedure in which a sample of blood is drawn and checked for the following: - The number of red blood cells and platelets. - The number and type of white blood cells. - The amount of hemoglobin (the protein that carries oxygen) in the red blood cells. - The portion of the blood sample made up of red blood cells. - Peripheral blood smear : A procedure in which a sample of blood is checked for blast cells, the number and kinds of white blood cells, number of platelets, and changes in the shape of the blood cells. - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. - Chest x-ray : An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. - Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. Biopsies that may be done include the following: - Bone marrow aspiration and biopsy : The removal of bone marrow, blood, and a small piece of bone by inserting a hollow needle into the hipbone or breastbone. - Tumor biopsy: A biopsy of a chloroma may be done. - Lymph node biopsy: The removal of all or part of a lymph node. - Cytogenetic analysis : A laboratory test in which cells in a sample of blood or bone marrow are viewed under a microscope to look for certain changes in the chromosomes. Changes in the chromosomes may include when part of one chromosome is switched with part of another chromosome, part of one chromosome is missing or repeated, or part of one chromosome is turned upside down. The following test is a type of cytogenetic analysis: - FISH (fluorescence in situ hybridization): A laboratory technique used to look at genes or chromosomes in cells and tissues. Pieces of DNA that contain a fluorescent dye are made in the laboratory and added to cells or tissues on a glass slide. When these pieces of DNA bind to specific genes or areas of chromosomes on the slide, they light up when viewed under a microscope with a special light. - Reverse transcriptionpolymerase chain reaction (RTPCR) test: A laboratory test in which cells in a sample of tissue are studied using chemicals to look for certain changes in the structure or function of genes. - Immunophenotyping : A process used to identify cells, based on the types of antigens or markers on the surface of the cell, that may include special staining of the blood and bone marrow cells. This process is used to diagnose the subtype of AML by comparing the cancer cells to normal cells of the immune system. - Molecular testing : A laboratory test to check for certain genes, proteins, or other molecules in a sample of blood or bone marrow. Molecular tests also check for certain changes in a gene or chromosome that may cause or affect the chance of developing AML. A molecular test may be used to help plan treatment, find out how well treatment is working, or make a prognosis. - Lumbar puncture : A procedure used to collect a sample of cerebrospinal fluid (CSF) from the spinal column. This is done by placing a needle between two bones in the spine and into the CSF around the spinal cord and removing a sample of the fluid. The sample of CSF is checked under a microscope for signs that leukemia cells have spread to the brain and spinal cord. This procedure is also called an LP or spinal tap.",Childhood Acute Myeloid Leukemia and Other Myeloid Malignancies,0000001_7,CancerGov,https://www.cancer.gov/types/leukemia/patient/child-aml-treatment-pdq,C0220621,T191,Disorders What is the outlook for Childhood Acute Myeloid Leukemia and Other Myeloid Malignancies ?,0000001_7-5,outlook,"Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options for childhood AML depend on the following: - The age of the child when the cancer is diagnosed. - The race or ethnic group of the child. - Whether the child is greatly overweight. - Number of white blood cells in the blood at diagnosis. - Whether the AML occurred after previous cancer treatment. - The subtype of AML. - Whether there are certain chromosome or gene changes in the leukemia cells. - Whether the child has Down syndrome. Most children with AML and Down syndrome can be cured of their leukemia. - Whether the leukemia is in the central nervous system (brain and spinal cord). - How quickly the leukemia responds to treatment. - Whether the AML is newly diagnosed (untreated) or has recurred (come back) after being treated. - The length of time since treatment ended, for AML that has recurred. The prognosis and treatment options for childhood CML depend on how long it has been since the patient was diagnosed and how many blast cells are in the blood. The prognosis (chance of recovery) and treatment options for JMML depend on the following: - The age of the child when the cancer is diagnosed. - The type of gene affected and the number of genes that have changes. - How many red blood cells, white blood cells, or platelets are in the blood. - Whether the JMML is newly diagnosed (untreated) or has recurred after treatment. The prognosis (chance of recovery) and treatment options for MDS depend on the following: - Whether the MDS was caused by previous cancer treatment. - How low the numbers of red blood cells, white blood cells, or platelets are. - Whether the MDS is newly diagnosed (untreated) or has recurred after treatment.",Childhood Acute Myeloid Leukemia and Other Myeloid Malignancies,0000001_7,CancerGov,https://www.cancer.gov/types/leukemia/patient/child-aml-treatment-pdq,C0220621,T191,Disorders What are the stages of Childhood Acute Myeloid Leukemia and Other Myeloid Malignancies ?,0000001_7-6,stages,"Key Points - Once childhood acute myeloid leukemia (AML) has been diagnosed, tests are done to find out if the cancer has spread to other parts of the body. - There is no standard staging system for childhood AML, childhood chronic myelogenous leukemia (CML), juvenile myelomonocytic leukemia (JMML), or myelodysplastic syndromes (MDS). Once childhood acute myeloid leukemia (AML) has been diagnosed, tests are done to find out if the cancer has spread to other parts of the body. The following tests and procedures may be used to determine if the leukemia has spread: - Lumbar puncture : A procedure used to collect a sample of cerebrospinal fluid (CSF) from the spinal column. This is done by placing a needle between two bones in the spine and into the CSF around the spinal cord and removing a sample of the fluid. The sample of CSF is checked under a microscope for signs that leukemia cells have spread to the brain and spinal cord. This procedure is also called an LP or spinal tap. - Biopsy of the testicles, ovaries, or skin: The removal of cells or tissues from the testicles, ovaries, or skin so they can be viewed under a microscope to check for signs of cancer. This is done only if something unusual about the testicles, ovaries, or skin is found during the physical exam. There is no standard staging system for childhood AML, childhood chronic myelogenous leukemia (CML), juvenile myelomonocytic leukemia (JMML), or myelodysplastic syndromes (MDS). The extent or spread of cancer is usually described as stages. Instead of stages, treatment of childhood AML, childhood CML, JMML, and MDS is based on one or more of the following: - The type of disease or the subtype of AML. - Whether leukemia has spread outside the blood and bone marrow. - Whether the disease is newly diagnosed, in remission, or recurrent. Newly diagnosed childhood AML Newly diagnosed childhood AML has not been treated except to relieve signs and symptoms such as fever, bleeding, or pain, and one of the following is true: - More than 20% of the cells in the bone marrow are blasts (leukemia cells). or - Less than 20% of the cells in the bone marrow are blasts and there is a specific change in the chromosome. Childhood AML in remission In childhood AML in remission, the disease has been treated and the following are true: - The complete blood count is almost normal. - Less than 5% of the cells in the bone marrow are blasts (leukemia cells). - There are no signs or symptoms of leukemia in the brain, spinal cord, or other parts of the body.",Childhood Acute Myeloid Leukemia and Other Myeloid Malignancies,0000001_7,CancerGov,https://www.cancer.gov/types/leukemia/patient/child-aml-treatment-pdq,C0220621,T191,Disorders what research (or clinical trials) is being done for Childhood Acute Myeloid Leukemia and Other Myeloid Malignancies ?,0000001_7-7,research,"New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Biologic therapy Biologic therapy is a treatment that uses the patients immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the bodys natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy. Natural killer (NK) cells are a type of biologic therapy. NK cells are white blood cells that can kill tumor cells. These may be taken from a donor and given to the patient by infusion to help kill leukemia cells. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Childhood Acute Myeloid Leukemia and Other Myeloid Malignancies,0000001_7,CancerGov,https://www.cancer.gov/types/leukemia/patient/child-aml-treatment-pdq,C0220621,T191,Disorders What are the treatments for Childhood Acute Myeloid Leukemia and Other Myeloid Malignancies ?,0000001_7-8,treatment,"Key Points - There are different types of treatment for children with acute myeloid leukemia (AML), chronic myelogenous leukemia (CML), juvenile myelomonocytic leukemia (JMML), or myelodysplastic syndromes (MDS). - Treatment is planned by a team of health care providers who are experts in treating childhood leukemia and other diseases of the blood. - Some cancer treatments cause side effects months or years after treatment has ended. - The treatment of childhood AML usually has two phases. - Seven types of standard treatment are used for childhood AML, childhood CML, JMML, or MDS. - Chemotherapy - Radiation therapy - Stem cell transplant - Targeted therapy - Other drug therapy - Watchful waiting - Supportive care - New types of treatment are being tested in clinical trials. - Biologic therapy - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for children with acute myeloid leukemia (AML), chronic myelogenous leukemia (CML), juvenile myelomonocytic leukemia (JMML), or myelodysplastic syndromes (MDS). Different types of treatment are available for children with AML, CML, JMML, or MDS. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment. Treatment is planned by a team of health care providers who are experts in treating childhood leukemia and other diseases of the blood. Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other healthcare providers who are experts in treating children with leukemia and who specialize in certain areas of medicine. These may include the following specialists: - Pediatrician. - Hematologist. - Medical oncologist. - Pediatric surgeon. - Radiation oncologist. - Neurologist. - Neuropathologist. - Neuroradiologist. - Pediatric nurse specialist. - Social worker. - Rehabilitation specialist. - Psychologist. Some cancer treatments cause side effects months or years after treatment has ended. Regular follow-up exams are very important. Some cancer treatments cause side effects that continue or appear months or years after cancer treatment has ended. These are called late effects. Late effects of cancer treatment may include: - Physical problems. - Changes in mood, feelings, thinking, learning, or memory. - Second cancers (new types of cancer). Some late effects may be treated or controlled. It is important that parents of children who are treated for AML or other blood diseases talk with their doctors about the effects cancer treatment can have on their child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information). The treatment of childhood AML usually has two phases. The treatment of childhood AML is done in phases: - Induction therapy: This is the first phase of treatment. The goal is to kill the leukemia cells in the blood and bone marrow. This puts the leukemia into remission. - Consolidation /intensification therapy: This is the second phase of treatment. It begins once the leukemia is in remission. The goal of therapy is to kill any remaining leukemia cells that may not be active but could begin to regrow and cause a relapse. Treatment called central nervous system (CNS) sanctuary therapy may be given during the induction phase of therapy. Because standard doses of chemotherapy may not reach leukemia cells in the CNS (brain and spinal cord), the cells are able to find sanctuary (hide) in the CNS. Intrathecal chemotherapy is able to reach leukemia cells in the CNS. It is given to kill the leukemia cells and lessen the chance the leukemia will recur (come back). CNS sanctuary therapy is also called CNS prophylaxis. Seven types of standard treatment are used for childhood AML, childhood CML, JMML, or MDS. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid (intrathecal chemotherapy), an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is treatment using more than one anticancer drug. The way the chemotherapy is given depends on the type of cancer being treated. In AML, the leukemia cells may spread to the brain and/or spinal cord. Chemotherapy given by mouth or vein to treat AML may not cross the blood-brain barrier to get into the fluid that surrounds the brain and spinal cord. Instead, chemotherapy is injected into the fluid-filled space to kill leukemia cells that may have spread there (intrathecal chemotherapy). See Drugs Approved for Acute Myeloid Leukemia for more information. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type of the cancer being treated. In childhood AML, external radiation therapy may be used to treat a chloroma that does not respond to chemotherapy. Stem cell transplant Stem cell transplant is a way of giving chemotherapy and replacing blood-forming cells that are abnormal or destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Types of targeted therapy include the following: - Tyrosine kinase inhibitor therapy: Tyrosine kinase inhibitor (TKI) therapy blocks signals needed for tumors to grow. TKIs block the enzyme (tyrosine kinase) that causes stem cells to become more white blood cells (granulocytes or blasts) than the body needs. TKIs may be used with other anticancer drugs as adjuvant therapy (treatment given after the initial treatment, to lower the risk that the cancer will come back). - Imatinib is a type of TKI that is approved to treat childhood CML. - Sorafenib, dasatinib, and nilotinib are being studied in the treatment of childhood leukemia. - Monoclonal antibody therapy: Monoclonal antibody therapy uses antibodies made in the laboratory, from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. - Gemtuzumab is a type of monoclonal antibody used in the treatment of a subtype of AML called acute promyelocytic leukemia (APL). Gemtuzumab is not available in the United States unless special approval is given. Monoclonal antibodies may be used with chemotherapy as adjuvant therapy. - Proteasome inhibitor therapy: Proteasome inhibitors break down proteins in cancer cells and kill them. - Bortezomib is a proteasome inhibitor used to treat childhood APL. See Drugs Approved for Leukemia for more information. Other drug therapy Lenalidomide may be used to lessen the need for transfusions in patients who have myelodysplastic syndromes caused by a specific chromosome change. Arsenic trioxide and all-trans retinoic acid (ATRA) are anticancer drugs that kill leukemia cells, stop the leukemia cells from dividing, or help the leukemia cells mature into white blood cells. These drugs are used in the treatment of acute promyelocytic leukemia. See Drugs Approved for Acute Myeloid Leukemia for more information. Watchful waiting Watchful waiting is closely monitoring a patients condition without giving any treatment until signs or symptoms appear or change. It is sometimes used to treat MDS or TMD. Supportive care Supportive care is given to lessen the problems caused by the disease or its treatment. Supportive care may include the following: - Transfusion therapy: A way of giving red blood cells, white blood cells, or platelets to replace blood cells destroyed by disease or cancer treatment. The blood may be donated from another person or it may have been taken from the patient earlier and stored until needed. - Drug therapy, such as antibiotics or antifungal agents. - Leukapheresis: A procedure in which a special machine is used to remove white blood cells from the blood. Blood is taken from the patient and put through a blood cell separator where the white blood cells are removed. The rest of the blood is then returned to the patient's bloodstream. New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Biologic therapy Biologic therapy is a treatment that uses the patients immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the bodys natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy. Natural killer (NK) cells are a type of biologic therapy. NK cells are white blood cells that can kill tumor cells. These may be taken from a donor and given to the patient by infusion to help kill leukemia cells. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Childhood Acute Myeloid Leukemia, Childhood Chronic Myelogenous Leukemia, Juvenile Myelomonocytic Leukemia, and Myelodysplastic Syndromes Newly Diagnosed Childhood Acute Myeloid Leukemia Treatment of newly diagnosed childhood acute myeloid leukemia may include the following: - Combination chemotherapy plus central nervous system sanctuary therapy with intrathecal chemotherapy. - A clinical trial comparing different chemotherapy regimens (doses and schedules of treatment). - A clinical trial of combination chemotherapy and targeted therapy with a proteasome inhibitor or a tyrosine kinase inhibitor with or without stem cell transplant. Treatment of newly diagnosed childhood acute leukemia with a granulocytic sarcoma (chloroma) may include chemotherapy with or without radiation therapy. Treatment of therapy -related AML is usually the same as for newly diagnosed AML, followed by stem cell transplant. Check the list of NCI-supported cancer clinical trials that are now accepting patients with untreated childhood acute myeloid leukemia and other myeloid malignancies. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Children with Newly Diagnosed Childhood AML and Down Syndrome Treatment of acute myeloid leukemia (AML) in children aged 4 years or younger who have Down syndrome may include the following: - Combination chemotherapy plus central nervous system sanctuary therapy with intrathecal chemotherapy. Treatment of AML in children older than 4 years who have Down syndrome may be the same as treatment for children without Down syndrome. Childhood Acute Myeloid Leukemia in Remission Treatment of childhood acute myeloid leukemia (AML) during the remission phase (consolidation /intensification therapy) depends on the subtype of AML and may include the following: - Combination chemotherapy. - High-dose chemotherapy followed by stem cell transplant using blood stem cells from a donor. - A clinical trial of chemotherapy followed by an infusion of natural killer cells. - A clinical trial of combination chemotherapy and targeted therapy with a proteasome inhibitor or a tyrosine kinase inhibitor with or without stem cell transplant. Check the list of NCI-supported cancer clinical trials that are now accepting patients with childhood acute myeloid leukemia in remission. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Recurrent Childhood Acute Myeloid Leukemia Treatment of recurrent childhood acute myeloid leukemia (AML) may include the following: - Combination chemotherapy. - Combination chemotherapy and stem cell transplant. - A second stem cell transplant. - A clinical trial of combinations of new anticancer drugs, new biologic agents, and stem cell transplant using different sources of stem cells. Treatment of recurrent AML in children with Down syndrome is chemotherapy. It is not clear if stem cell transplant after chemotherapy is helpful in treating these children. Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent childhood acute myeloid leukemia. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Acute Promyelocytic Leukemia Treatment of acute promyelocytic leukemia may include the following: - All-trans retinoic acid (ATRA) plus chemotherapy. - Arsenic trioxide therapy. - Central nervous system sanctuary therapy with intrathecal chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with childhood acute promyelocytic leukemia (M3). For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Recurrent Acute Promyelocytic Leukemia Treatment of recurrent acute promyelocytic leukemia may include the following: - All-trans retinoic acid therapy (ATRA) plus chemotherapy. - Arsenic trioxide therapy. - Targeted therapy with a monoclonal antibody (gemtuzumab), if special approval is given. - Stem cell transplant using blood stem cells from the patient or a donor. Childhood Chronic Myelogenous Leukemia Treatment for childhood chronic myelogenous leukemia may include the following: - Targeted therapy with a tyrosine kinase inhibitor (imatinib). - A clinical trial of targeted therapy with other tyrosine kinase inhibitors. For patients whose disease does not respond to therapy with imatinib or whose disease comes back after treatment, treatment may include the following: - Stem cell transplant using blood stem cells from a donor. - A clinical trial of targeted therapy with other tyrosine kinase inhibitors. Check the list of NCI-supported cancer clinical trials that are now accepting patients with childhood chronic myelogenous leukemia. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Juvenile Myelomonocytic Leukemia Treatment of juvenile myelomonocytic leukemia (JMML) may include the following: - Combination chemotherapy followed by stem cell transplant. If JMML recurs after stem cell transplant, a second stem cell transplant may be done. Check the list of NCI-supported cancer clinical trials that are now accepting patients with juvenile myelomonocytic leukemia. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Myelodysplastic Syndromes Treatment of myelodysplastic syndromes (MDS) may include the following: - Watchful waiting. - Stem cell transplant using blood stem cells from a donor. - Combination chemotherapy. - Lenalidomide therapy. - A clinical trial of stem cell transplant using lower doses of chemotherapy. - A clinical trial of a new anticancer drug or targeted therapy. If the MDS becomes acute myeloid leukemia (AML), treatment will be the same as treatment for newly diagnosed AML. Treatment of therapy-related MDS is usually the same as for newly diagnosed AML, followed by stem cell transplant. Transient myeloproliferative disorder (TMD), a type of MDS, usually goes away on its own. For TMD that does not go away on its own, treatment may include the following: - Transfusion therapy. - Leukapheresis. - Chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with childhood myelodysplastic syndromes. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website.",Childhood Acute Myeloid Leukemia and Other Myeloid Malignancies,0000001_7,CancerGov,https://www.cancer.gov/types/leukemia/patient/child-aml-treatment-pdq,C0220621,T191,Disorders What is (are) Adult Soft Tissue Sarcoma ?,0000003_1-1,information,"Key Points - Adult soft tissue sarcoma is a disease in which malignant (cancer) cells form in the soft tissues of the body. - Having certain inherited disorders can increase the risk of adult soft tissue sarcoma. - A sign of adult soft tissue sarcoma is a lump or swelling in soft tissue of the body. - Adult soft tissue sarcoma is diagnosed with a biopsy. - Certain factors affect treatment options and prognosis (chance of recovery). Adult soft tissue sarcoma is a disease in which malignant (cancer) cells form in the soft tissues of the body. The soft tissues of the body include the muscles, tendons (bands of fiber that connect muscles to bones), fat, blood vessels, lymph vessels, nerves, and tissues around joints. Adult soft tissue sarcomas can form almost anywhere in the body, but are most common in the head, neck, arms, legs, trunk, and abdomen. There are many types of soft tissue sarcoma. The cells of each type of sarcoma look different under a microscope, based on the type of soft tissue in which the cancer began. See the following PDQ summaries for more information on soft tissue sarcomas: - Childhood Soft Tissue Sarcoma Treatment - Ewing Sarcoma Family of Tumors Treatment - Gastrointestinal Stromal Tumors Treatment - Kaposi Sarcoma Treatment - Uterine Sarcoma Treatment",Adult Soft Tissue Sarcoma,0000003_1,CancerGov,https://www.cancer.gov/types/soft-tissue-sarcoma/patient/adult-soft-tissue-treatment-pdq,C0220613,T191,Disorders Who is at risk for Adult Soft Tissue Sarcoma? ?,0000003_1-2,susceptibility,"Having certain inherited disorders can increase the risk of adult soft tissue sarcoma. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for soft tissue sarcoma include the following inherited disorders: - Retinoblastoma. - Neurofibromatosis type 1 (NF1; von Recklinghausen disease). - Tuberous sclerosis (Bourneville disease). - Familial adenomatous polyposis (FAP; Gardner syndrome). - Li-Fraumeni syndrome. - Werner syndrome (adult progeria). - Nevoid basal cell carcinoma syndrome (Gorlin syndrome). Other risk factors for soft tissue sarcoma include the following: - Past treatment with radiation therapy for certain cancers. - Being exposed to certain chemicals, such as Thorotrast (thorium dioxide), vinyl chloride, or arsenic. - Having swelling (lymphedema) in the arms or legs for a long time.",Adult Soft Tissue Sarcoma,0000003_1,CancerGov,https://www.cancer.gov/types/soft-tissue-sarcoma/patient/adult-soft-tissue-treatment-pdq,C0220613,T191,Disorders What are the symptoms of Adult Soft Tissue Sarcoma ?,0000003_1-3,symptoms,"A sign of adult soft tissue sarcoma is a lump or swelling in soft tissue of the body. A sarcoma may appear as a painless lump under the skin, often on an arm or a leg. Sarcomas that begin in the abdomen may not cause signs or symptoms until they get very big. As the sarcoma grows bigger and presses on nearby organs, nerves, muscles, or blood vessels, signs and symptoms may include: - Pain. - Trouble breathing. Other conditions may cause the same signs and symptoms. Check with your doctor if you have any of these problems.",Adult Soft Tissue Sarcoma,0000003_1,CancerGov,https://www.cancer.gov/types/soft-tissue-sarcoma/patient/adult-soft-tissue-treatment-pdq,C0220613,T191,Disorders How to diagnose Adult Soft Tissue Sarcoma ?,0000003_1-4,exams and tests,"Adult soft tissue sarcoma is diagnosed with a biopsy. If your doctor thinks you may have a soft tissue sarcoma, a biopsy will be done. The type of biopsy will be based on the size of the tumor and where it is in the body. There are three types of biopsy that may be used: - Incisional biopsy : The removal of part of a lump or a sample of tissue. - Core biopsy : The removal of tissue using a wide needle. - Excisional biopsy : The removal of an entire lump or area of tissue that doesnt look normal. Samples will be taken from the primary tumor, lymph nodes, and other suspicious areas. A pathologist views the tissue under a microscope to look for cancer cells and to find out the grade of the tumor. The grade of a tumor depends on how abnormal the cancer cells look under a microscope and how quickly the cells are dividing. High-grade tumors usually grow and spread more quickly than low-grade tumors. Because soft tissue sarcoma can be hard to diagnose, patients should ask to have tissue samples checked by a pathologist who has experience in diagnosing soft tissue sarcoma. The following tests may be done on the tissue that was removed: - Immunohistochemistry : A test that uses antibodies to check for certain antigens in a sample of tissue. The antibody is usually linked to a radioactive substance or a dye that causes the tissue to light up under a microscope. This type of test may be used to tell the difference between different types of cancer. - Light and electron microscopy : A laboratory test in which cells in a sample of tissue are viewed under regular and high-powered microscopes to look for certain changes in the cells. - Cytogenetic analysis : A laboratory test in which cells in a sample of tissue are viewed under a microscope to look for certain changes in the chromosomes. - FISH (fluorescence in situ hybridization): A laboratory test used to look at genes or chromosomes in cells and tissues. Pieces of DNA that contain a fluorescent dye are made in the laboratory and added to cells or tissues on a glass slide. When these pieces of DNA attach to certain genes or areas of chromosomes on the slide, they light up when viewed under a microscope with a special light. - Flow cytometry : A laboratory test that measures the number of cells in a sample, the percentage of live cells in a sample, and certain characteristics of cells, such as size, shape, and the presence of tumor markers on the cell surface. The cells are stained with a light-sensitive dye, placed in a fluid, and passed in a stream before a laser or other type of light. The measurements are based on how the light-sensitive dye reacts to the light.",Adult Soft Tissue Sarcoma,0000003_1,CancerGov,https://www.cancer.gov/types/soft-tissue-sarcoma/patient/adult-soft-tissue-treatment-pdq,C0220613,T191,Disorders What is the outlook for Adult Soft Tissue Sarcoma ?,0000003_1-5,outlook,"Certain factors affect treatment options and prognosis (chance of recovery). The treatment options and prognosis (chance of recovery) depend on the following: - The type of soft tissue sarcoma. - The size, grade, and stage of the tumor. - How fast the cancer cells are growing and dividing. - Where the tumor is in the body. - Whether all of the tumor is removed by surgery. - The patient's age and general health. - Whether the cancer has recurred (come back).",Adult Soft Tissue Sarcoma,0000003_1,CancerGov,https://www.cancer.gov/types/soft-tissue-sarcoma/patient/adult-soft-tissue-treatment-pdq,C0220613,T191,Disorders What are the stages of Adult Soft Tissue Sarcoma ?,0000003_1-6,stages,"Key Points - After adult soft tissue sarcoma has been diagnosed, tests are done to find out if cancer cells have spread within the soft tissue or to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - The following stages are used for adult soft tissue sarcoma: - Stage I - Stage II - Stage III - Stage IV After adult soft tissue sarcoma has been diagnosed, tests are done to find out if cancer cells have spread within the soft tissue or to other parts of the body. The process used to find out if cancer has spread within the soft tissue or to other parts of the body is called staging. Staging of soft tissue sarcoma is also based on the grade and size of the tumor, whether it is superficial (close to the skin's surface) or deep, and whether it has spread to the lymph nodes or other parts of the body. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following tests and procedures may be used in the staging process: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Chest x-ray : An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. - Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following: - The number of red blood cells, white blood cells, and platelets. - The amount of hemoglobin (the protein that carries oxygen) in the red blood cells. - The portion of the blood sample made up of red blood cells. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside of the body, such as the lung and abdomen, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. The results of these tests are viewed together with the results of the tumor biopsy to find out the stage of the soft tissue sarcoma before treatment is given. Sometimes chemotherapy or radiation therapy is given as the initial treatment and afterwards the soft tissue sarcoma is staged again. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if soft tissue sarcoma spreads to the lung, the cancer cells in the lung are actually soft tissue sarcoma cells. The disease is metastatic soft tissue sarcoma, not lung cancer. The following stages are used for adult soft tissue sarcoma: Stage I Stage I is divided into stages IA and IB: - In stage IA, the tumor is low-grade (likely to grow and spread slowly) and 5 centimeters or smaller. It may be either superficial (in subcutaneous tissue with no spread into connective tissue or muscle below) or deep (in the muscle and may be in connective or subcutaneous tissue). - In stage IB, the tumor is low-grade (likely to grow and spread slowly) and larger than 5 centimeters. It may be either superficial (in subcutaneous tissue with no spread into connective tissue or muscle below) or deep (in the muscle and may be in connective or subcutaneous tissue). Stage II Stage II is divided into stages IIA and IIB: - In stage IIA, the tumor is mid-grade (somewhat likely to grow and spread quickly) or high-grade (likely to grow and spread quickly) and 5 centimeters or smaller. It may be either superficial (in subcutaneous tissue with no spread into connective tissue or muscle below) or deep (in the muscle and may be in connective or subcutaneous tissue). - In stage IIB, the tumor is mid-grade (somewhat likely to grow and spread quickly) and larger than 5 centimeters. It may be either superficial (in subcutaneous tissue with no spread into connective tissue or muscle below) or deep (in the muscle and may be in connective or subcutaneous tissue). Stage III In stage III, the tumor is either: - high-grade (likely to grow and spread quickly), larger than 5 centimeters, and either superficial (in subcutaneous tissue with no spread into connective tissue or muscle below) or deep (in the muscle and may be in connective or subcutaneous tissue); or - any grade, any size, and has spread to nearby lymph nodes. Stage III cancer that has spread to the lymph nodes is advanced stage III. Stage IV In stage IV, the tumor is any grade, any size, and may have spread to nearby lymph nodes. Cancer has spread to distant parts of the body, such as the lungs.",Adult Soft Tissue Sarcoma,0000003_1,CancerGov,https://www.cancer.gov/types/soft-tissue-sarcoma/patient/adult-soft-tissue-treatment-pdq,C0220613,T191,Disorders what research (or clinical trials) is being done for Adult Soft Tissue Sarcoma ?,0000003_1-7,research,"Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Adult Soft Tissue Sarcoma,0000003_1,CancerGov,https://www.cancer.gov/types/soft-tissue-sarcoma/patient/adult-soft-tissue-treatment-pdq,C0220613,T191,Disorders What are the treatments for Adult Soft Tissue Sarcoma ?,0000003_1-8,treatment,"Key Points - There are different types of treatment for patients with adult soft tissue sarcoma. - Three types of standard treatment are used: - Surgery - Radiation therapy - Chemotherapy - New types of treatment are being tested in clinical trials. - Regional chemotherapy - Treatment for adult soft tissue sarcoma may cause side effects. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with adult soft tissue sarcoma. Different types of treatments are available for patients with adult soft tissue sarcoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Three types of standard treatment are used: Surgery Surgery is the most common treatment for adult soft tissue sarcoma. For some soft-tissue sarcomas, removal of the tumor in surgery may be the only treatment needed. The following surgical procedures may be used: - Mohs microsurgery: A procedure in which the tumor is cut from the skin in thin layers. During surgery, the edges of the tumor and each layer of tumor removed are viewed through a microscope to check for cancer cells. Layers continue to be removed until no more cancer cells are seen. This type of surgery removes as little normal tissue as possible and is often used where appearance is important, such as on the skin. - Wide local excision: Removal of the tumor along with some normal tissue around it. For tumors of the head, neck, abdomen, and trunk, as little normal tissue as possible is removed. - Limb-sparing surgery: Removal of the tumor in an arm or leg without amputation, so the use and appearance of the limb is saved. Radiation therapy or chemotherapy may be given first to shrink the tumor. The tumor is then removed in a wide local excision. Tissue and bone that are removed may be replaced with a graft using tissue and bone taken from another part of the patient's body, or with an implant such as artificial bone. - Amputation: Surgery to remove part or all of a limb or appendage, such as an arm or leg. Amputation is rarely used to treat soft tissue sarcoma of the arm or leg. - Lymphadenectomy: A surgical procedure in which lymph nodes are removed and a sample of tissue is checked under a microscope for signs of cancer. This procedure is also called a lymph node dissection. Radiation therapy or chemotherapy may be given before or after surgery to remove the tumor. When given before surgery, radiation therapy or chemotherapy will make the tumor smaller and reduce the amount of tissue that needs to be removed during surgery. Treatment given before surgery is called neoadjuvant therapy. When given after surgery, radiation therapy or chemotherapy will kill any remaining cancer cells. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. Intensity-modulated radiation therapy (IMRT) is a type of 3-dimensional (3-D) radiation therapy that uses a computer to make pictures of the size and shape of the tumor. Thin beams of radiation of different intensities (strengths) are aimed at the tumor from many angles. This type of external radiation therapy causes less damage to nearby healthy tissue and is less likely to cause dry mouth, trouble swallowing, and damage to the skin. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External radiation therapy and internal radiation therapy may be used to treat adult soft tissue sarcoma. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. See Drugs Approved for Soft Tissue Sarcoma for more information. New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Regional chemotherapy Clinical trials are studying ways to improve the effect of chemotherapy on tumor cells, including the following: - Regional hyperthermia therapy: A treatment in which tissue around the tumor is exposed to high temperatures to damage and kill cancer cells or to make cancer cells more sensitive to chemotherapy. - Isolated limb perfusion: A procedure that sends chemotherapy directly to an arm or leg in which the cancer has formed. The flow of blood to and from the limb is temporarily stopped with a tourniquet, and anticancer drugs are put directly into the blood of the limb. This sends a high dose of drugs to the tumor. Treatment for adult soft tissue sarcoma may cause side effects. For information about side effects caused by treatment for cancer, see our Side Effects page. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Adult Soft Tissue Sarcoma Stage I Adult Soft Tissue Sarcoma Treatment of stage I soft tissue sarcoma may include the following: - Surgery to remove the tumor, such as Mohs microsurgery for small sarcomas of the skin, wide local excision, or limb-sparing surgery. - Radiation therapy before and/or after surgery. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I adult soft tissue sarcoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage II Adult Soft Tissue Sarcoma and Stage III Adult Soft Tissue Sarcoma That Has Not Spread to Lymph Nodes Treatment of stage II adult soft tissue sarcoma and stage III adult soft tissue sarcoma that has not spread to lymph nodes may include the following: - Surgery to remove the tumor, such as wide local excision or limb-sparing surgery. - Radiation therapy before or after surgery. - Radiation therapy or chemotherapy before limb-sparing surgery. Radiation therapy may also be given after surgery. - High-dose radiation therapy for tumors that cannot be removed by surgery. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage II adult soft tissue sarcoma and stage III adult soft tissue sarcoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage III Adult Soft Tissue Sarcoma That Has Spread to Lymph Nodes (Advanced) Treatment of stage III adult soft tissue sarcoma that has spread to lymph nodes (advanced) may include the following: - Surgery (wide local excision) with lymphadenectomy. Radiation therapy may also be given after surgery. - A clinical trial of surgery followed by chemotherapy. - A clinical trial of regional hyperthermia therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III adult soft tissue sarcoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage IV Adult Soft Tissue Sarcoma Treatment of stage IV adult soft tissue sarcoma may include the following: - Chemotherapy. - Surgery to remove cancer that has spread to the lungs. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage IV adult soft tissue sarcoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Adult Soft Tissue Sarcoma,0000003_1,CancerGov,https://www.cancer.gov/types/soft-tissue-sarcoma/patient/adult-soft-tissue-treatment-pdq,C0220613,T191,Disorders What is (are) Gastrointestinal Stromal Tumors ?,0000003_2-1,information,"Key Points - Gastrointestinal stromal tumor is a disease in which abnormal cells form in the tissues of the gastrointestinal tract. - Genetic factors can increase the risk of having a gastrointestinal stromal tumor. - Signs of gastrointestinal stromal tumors include blood in the stool or vomit. - Tests that examine the GI tract are used to detect (find) and diagnose gastrointestinal stromal tumors. - Very small GISTs are common. - Certain factors affect prognosis (chance of recovery) and treatment options. Gastrointestinal stromal tumor is a disease in which abnormal cells form in the tissues of the gastrointestinal tract. The gastrointestinal (GI) tract is part of the bodys digestive system. It helps to digest food and takes nutrients (vitamins, minerals, carbohydrates, fats, proteins, and water) from food so they can be used by the body. The GI tract is made up of the following organs: - Stomach. - Small intestine. - Large intestine (colon). Gastrointestinal stromal tumors (GISTs) may be malignant (cancer) or benign (not cancer). They are most common in the stomach and small intestine but may be found anywhere in or near the GI tract. Some scientists believe that GISTs begin in cells called interstitial cells of Cajal (ICC), in the wall of the GI tract. See the PDQ summary about Unusual Cancers of Childhood Treatment for information on the treatment of GIST in children. Very small GISTs are common. Sometimes GISTs are smaller than the eraser on top of a pencil. Tumors may be found during a procedure that is done for another reason, such as an x-ray or surgery. Some of these small tumors will not grow and cause signs or symptoms or spread to the abdomen or other parts of the body. Doctors do not agree on whether these small tumors should be removed or whether they should be watched to see if they begin to grow.",Gastrointestinal Stromal Tumors,0000003_2,CancerGov,https://www.cancer.gov/types/soft-tissue-sarcoma/patient/gist-treatment-pdq,C0238198,T191,Disorders Who is at risk for Gastrointestinal Stromal Tumors? ?,0000003_2-2,susceptibility,"Genetic factors can increase the risk of having a gastrointestinal stromal tumor. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. The genes in cells carry the hereditary information received from a persons parents. The risk of GIST is increased in people who have inherited a mutation (change) in a certain gene. In rare cases, GISTs can be found in several members of the same family. GIST may be part of a genetic syndrome, but this is rare. A genetic syndrome is a set of symptoms or conditions that occur together and is usually caused by abnormal genes. The following genetic syndromes have been linked to GIST: - Neurofibromatosis type 1 (NF1). - Carney triad.",Gastrointestinal Stromal Tumors,0000003_2,CancerGov,https://www.cancer.gov/types/soft-tissue-sarcoma/patient/gist-treatment-pdq,C0238198,T191,Disorders What are the symptoms of Gastrointestinal Stromal Tumors ?,0000003_2-3,symptoms,"Signs of gastrointestinal stromal tumors include blood in the stool or vomit. These and other signs and symptoms may be caused by a GIST or by other conditions. Check with your doctor if you have any of the following: - Blood (either bright red or very dark) in the stool or vomit. - Pain in the abdomen, which may be severe. - Feeling very tired. - Trouble or pain when swallowing. - Feeling full after only a little food is eaten.",Gastrointestinal Stromal Tumors,0000003_2,CancerGov,https://www.cancer.gov/types/soft-tissue-sarcoma/patient/gist-treatment-pdq,C0238198,T191,Disorders What are the stages of Gastrointestinal Stromal Tumors ?,0000003_2-6,stages,"Key Points - After a gastrointestinal stromal tumor has been diagnosed, tests are done to find out if cancer cells have spread within the gastrointestinal tract or to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - The results of diagnostic and staging tests are used to plan treatment. After a gastrointestinal stromal tumor has been diagnosed, tests are done to find out if cancer cells have spread within the gastrointestinal tract or to other parts of the body. The process used to find out if cancer has spread within the gastrointestinal (GI) tract or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. The following tests and procedures may be used in the staging process: - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Chest x-ray : An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. - Bone scan : A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones and is detected by a scanner. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of tumor as the primary tumor. For example, if a gastrointestinal stromal tumor (GIST) spreads to the liver, the tumor cells in the liver are actually GIST cells. The disease is metastatic GIST, not liver cancer. The results of diagnostic and staging tests are used to plan treatment. For many cancers it is important to know the stage of the cancer in order to plan treatment. However, the treatment of GIST is not based on the stage of the cancer. Treatment is based on whether the tumor can be removed by surgery and if the tumor has spread to other parts of the abdomen or to distant parts of the body. Treatment is based on whether the tumor is: - Resectable: These tumors can be removed by surgery . - Unresectable: These tumors cannot be completely removed by surgery. - Metastatic and recurrent: Metastatic tumors have spread to other parts of the body. Recurrent tumors have recurred (come back) after treatment. Recurrent GISTs may come back in the gastrointestinal tract or in other parts of the body. They are usually found in the abdomen, peritoneum, and/or liver. - Refractory: These tumors have not gotten better with treatment.",Gastrointestinal Stromal Tumors,0000003_2,CancerGov,https://www.cancer.gov/types/soft-tissue-sarcoma/patient/gist-treatment-pdq,C0238198,T191,Disorders what research (or clinical trials) is being done for Gastrointestinal Stromal Tumors ?,0000003_2-7,research,"New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Gastrointestinal Stromal Tumors,0000003_2,CancerGov,https://www.cancer.gov/types/soft-tissue-sarcoma/patient/gist-treatment-pdq,C0238198,T191,Disorders What are the treatments for Gastrointestinal Stromal Tumors ?,0000003_2-8,treatment,"Key Points - There are different types of treatment for patients with gastrointestinal stromal tumors. - Four types of standard treatment are used: - Surgery - Targeted therapy - Watchful waiting - Supportive care - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with gastrointestinal stromal tumors. Different types of treatments are available for patients with gastrointestinal stromal tumors (GISTs). Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Four types of standard treatment are used: Surgery If the GIST has not spread and is in a place where surgery can be safely done, the tumor and some of the tissue around it may be removed. Sometimes surgery is done using a laparoscope (a thin, lighted tube) to see inside the body. Small incisions (cuts) are made in the wall of the abdomen and a laparoscope is inserted into one of the incisions. Instruments may be inserted through the same incision or through other incisions to remove organs or tissues. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Tyrosine kinase inhibitors (TKIs) are targeted therapy drugs that block signals needed for tumors to grow. TKIs may be used to treat GISTs that cannot be removed by surgery or to shrink GISTs so they become small enough to be removed by surgery. Imatinib mesylate and sunitinib are two TKIs used to treat GISTs. TKIs are sometimes given for as long as the tumor does not grow and serious side effects do not occur. See Drugs Approved for Gastrointestinal Stromal Tumors for more information. Watchful waiting Watchful waiting is closely monitoring a patients condition without giving any treatment until signs or symptoms appear or change. Supportive care If a GIST gets worse during treatment or there are side effects, supportive care is usually given. The goal of supportive care is to prevent or treat the symptoms of a disease, side effects caused by treatment, and psychological, social, and spiritual problems related to a disease or its treatment. Supportive care helps improve the quality of life of patients who have a serious or life-threatening disease. Radiation therapy is sometimes given as supportive care to relieve pain in patients with large tumors that have spread. New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Follow-up for GISTs that were removed by surgery may include CT scan of the liver and pelvis or watchful waiting. For GISTs that are treated with tyrosine kinase inhibitors, follow-up tests, such as CT, MRI, or PET scans, may be done to check how well the targeted therapy is working. Treatment Options for Gastrointestinal Stromal Tumors Resectable Gastrointestinal Stromal Tumors Resectable gastrointestinal stromal tumors (GISTs) can be completely or almost completely removed by surgery. Treatment may include the following: - Surgery to remove tumors that are 2 centimeters or larger. Laparoscopic surgery may be done if the tumor is 5 cm or smaller. If there are cancer cells remaining at the edges of the area where the tumor was removed, watchful waiting or targeted therapy with imatinib mesylate may follow. - A clinical trial of targeted therapy with imatinib mesylate following surgery, to decrease the chance the tumor will recur (come back). Unresectable Gastrointestinal Stromal Tumors Unresectable GISTs cannot be completely removed by surgery because they are too large or in a place where there would be too much damage to nearby organs if the tumor is removed. Treatment is usually a clinical trial of targeted therapy with imatinib mesylate to shrink the tumor, followed by surgery to remove as much of the tumor as possible. Metastatic and Recurrent Gastrointestinal Stromal Tumors Treatment of GISTs that are metastatic (spread to other parts of the body) or recurrent (came back after treatment) may include the following: - Targeted therapy with imatinib mesylate. - Targeted therapy with sunitinib, if the tumor begins to grow during imatinib mesylate therapy or if the side effects are too bad. - Surgery to remove tumors that have been treated with targeted therapy and are shrinking, stable (not changing), or that have slightly increased in size. Targeted therapy may continue after surgery. - Surgery to remove tumors when there are serious complications, such as bleeding, a hole in the gastrointestinal (GI) tract, a blocked GI tract, or infection. - A clinical trial of a new treatment. Refractory Gastrointestinal Stromal Tumors Many GISTs treated with a tyrosine kinase inhibitor (TKI) become refractory (stop responding) to the drug after a while. Treatment is usually a clinical trial with a different TKI or a clinical trial of a new drug. Treatment Options in Clinical Trials Check the list of NCI-supported cancer clinical trials that are now accepting patients with gastrointestinal stromal tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Gastrointestinal Stromal Tumors,0000003_2,CancerGov,https://www.cancer.gov/types/soft-tissue-sarcoma/patient/gist-treatment-pdq,C0238198,T191,Disorders What is (are) Kaposi Sarcoma ?,0000003_3-1,information,"Key Points - Kaposi sarcoma is a disease in which malignant tumors (cancer) can form in the skin, mucous membranes, lymph nodes, and other organs. - Tests that examine the skin, lungs, and gastrointestinal tract are used to detect (find) and diagnose Kaposi sarcoma. - After Kaposi sarcoma has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body. - Certain factors affect prognosis (chance of recovery) and treatment options. Kaposi sarcoma is a disease in which malignant tumors (cancer) can form in the skin, mucous membranes, lymph nodes, and other organs. Kaposi sarcoma is a cancer that causes lesions (abnormal tissue) to grow in the skin; the mucous membranes lining the mouth, nose, and throat; lymph nodes; or other organs. The lesions are usually purple and are made of cancer cells, new blood vessels, red blood cells, and white blood cells. Kaposi sarcoma is different from other cancers in that lesions may begin in more than one place in the body at the same time. Human herpesvirus-8 (HHV-8) is found in the lesions of all patients with Kaposi sarcoma. This virus is also called Kaposi sarcoma herpesvirus (KSHV). Most people infected with HHV-8 do not get Kaposi sarcoma. Those infected with HHV-8 who are most likely to develop Kaposi sarcoma have immune systems weakened by disease or by drugs given after an organ transplant. There are several types of Kaposi sarcoma, including: - Classic Kaposi sarcoma. - African Kaposi sarcoma. - Immunosuppressive therapyrelated Kaposi sarcoma. - Epidemic Kaposi sarcoma. - Nonepidemic Kaposi sarcoma. Tests that examine the skin, lungs, and gastrointestinal tract are used to detect (find) and diagnose Kaposi sarcoma. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking skin and lymph nodes for signs of disease, such as lumps or anything else that seems unusual. A history of the patient's health habits and past illnesses and treatments will also be taken. - Chest x-ray : An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. This is used to find Kaposi sarcoma in the lungs. - Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. One of the following types of biopsies may be done to check for Kaposi sarcoma lesions in the skin: - Excisional biopsy : A scalpel is used to remove the entire skin growth. - Incisional biopsy : A scalpel is used to remove part of a skin growth. - Core biopsy : A wide needle is used to remove part of a skin growth. - Fine-needle aspiration (FNA) biopsy : A thin needle is used to remove part of a skin growth. An endoscopy or bronchoscopy may be done to check for Kaposi sarcoma lesions in the gastrointestinal tract or lungs. - Endoscopy for biopsy: A procedure to look at organs and tissues inside the body to check for abnormal areas. An endoscope is inserted through an incision (cut) in the skin or opening in the body, such as the mouth. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of disease. This is used to find Kaposi sarcoma lesions in the gastrointestinal tract. - Bronchoscopy for biopsy: A procedure to look inside the trachea and large airways in the lung for abnormal areas. A bronchoscope is inserted through the nose or mouth into the trachea and lungs. A bronchoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of disease. This is used to find Kaposi sarcoma lesions in the lungs. After Kaposi sarcoma has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body. The following tests and procedures may be used to find out if cancer has spread to other parts of the body: - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the lung, liver, and spleen, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. This imaging test checks for signs of cancer in the lung, liver, and spleen. - CD34 lymphocyte count: A procedure in which a blood sample is checked to measure the amount of CD34 cells (a type of white blood cell). A lower than normal amount of CD34 cells can be a sign the immune system is not working well. Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - The type of Kaposi sarcoma. - The general health of the patient, especially the patient's immune system. - Whether the cancer has just been diagnosed or has recurred (come back). Classic Kaposi Sarcoma Key Points - Classic Kaposi sarcoma is found most often in older men of Italian or Eastern European Jewish origin. - Signs of classic Kaposi sarcoma may include slow-growing lesions on the legs and feet. - Another cancer may develop. Classic Kaposi sarcoma is found most often in older men of Italian or Eastern European Jewish origin. Classic Kaposi sarcoma is a rare disease that gets worse slowly over many years. Signs of classic Kaposi sarcoma may include slow-growing lesions on the legs and feet. Patients may have one or more red, purple, or brown skin lesions on the legs and feet, most often on the ankles or soles of the feet. Over time, lesions may form in other parts of the body, such as the stomach, intestines, or lymph nodes. The lesions usually don't cause any symptoms, but may grow in size and number over a period of 10 years or more. Pressure from the lesions may block the flow of lymph and blood in the legs and cause painful swelling. Lesions in the digestive tract may cause gastrointestinal bleeding. Another cancer may develop. Some patients with classic Kaposi sarcoma may develop another type of cancer before the Kaposi sarcoma lesions appear or later in life. Most often, this second cancer is non-Hodgkin lymphoma. Frequent follow-up is needed to watch for these second cancers. Epidemic Kaposi Sarcoma Key Points - Epidemic Kaposi sarcoma is found in patients who have acquired immunodeficiency syndrome (AIDS). - Signs of epidemic Kaposi sarcoma can include lesions that form in many parts of the body. - The use of drug therapy called cART reduces the risk of epidemic Kaposi sarcoma in patients infected with HIV. Epidemic Kaposi sarcoma is found in patients who have acquired immunodeficiency syndrome (AIDS). Epidemic Kaposi sarcoma occurs in patients who have acquired immunodeficiency syndrome (AIDS). AIDS is caused by the human immunodeficiency virus (HIV), which attacks and weakens the immune system. When the body's immune system is weakened by HIV, infections and cancers such as Kaposi sarcoma can develop. Most cases of epidemic Kaposi sarcoma in the United States have been diagnosed in homosexual or bisexual men infected with HIV. Signs of epidemic Kaposi sarcoma can include lesions that form in many parts of the body. The signs of epidemic Kaposi sarcoma can include lesions in different parts of the body, including any of the following: - Skin. - Lining of the mouth. - Lymph nodes. - Stomach and intestines. - Lungs and lining of the chest. - Liver. - Spleen. Kaposi sarcoma is sometimes found in the lining of the mouth during a regular dental check-up. In most patients with epidemic Kaposi sarcoma, the disease will spread to other parts of the body over time. Fever, weight loss, or diarrhea can occur. In the later stages of epidemic Kaposi sarcoma, life-threatening infections are common. The use of drug therapy called cART reduces the risk of epidemic Kaposi sarcoma in patients infected with HIV. Combined antiretroviral therapy (cART) is a combination of several drugs that block HIV and slow down the development of AIDS and AIDS-related Kaposi sarcoma. For information about AIDS and its treatment, see the AIDSinfo website. Tests that examine the skin, lungs, and gastrointestinal tract are used to detect (find) and diagnose Kaposi sarcoma. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking skin and lymph nodes for signs of disease, such as lumps or anything else that seems unusual. A history of the patient's health habits and past illnesses and treatments will also be taken. - Chest x-ray : An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. This is used to find Kaposi sarcoma in the lungs. - Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. One of the following types of biopsies may be done to check for Kaposi sarcoma lesions in the skin: - Excisional biopsy : A scalpel is used to remove the entire skin growth. - Incisional biopsy : A scalpel is used to remove part of a skin growth. - Core biopsy : A wide needle is used to remove part of a skin growth. - Fine-needle aspiration (FNA) biopsy : A thin needle is used to remove part of a skin growth. An endoscopy or bronchoscopy may be done to check for Kaposi sarcoma lesions in the gastrointestinal tract or lungs. - Endoscopy for biopsy: A procedure to look at organs and tissues inside the body to check for abnormal areas. An endoscope is inserted through an incision (cut) in the skin or opening in the body, such as the mouth. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of disease. This is used to find Kaposi sarcoma lesions in the gastrointestinal tract. - Bronchoscopy for biopsy: A procedure to look inside the trachea and large airways in the lung for abnormal areas. A bronchoscope is inserted through the nose or mouth into the trachea and lungs. A bronchoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of disease. This is used to find Kaposi sarcoma lesions in the lungs. After Kaposi sarcoma has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body. The following tests and procedures may be used to find out if cancer has spread to other parts of the body: - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the lung, liver, and spleen, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. This imaging test checks for signs of cancer in the lung, liver, and spleen. - CD34 lymphocyte count: A procedure in which a blood sample is checked to measure the amount of CD34 cells (a type of white blood cell). A lower than normal amount of CD34 cells can be a sign the immune system is not working well. Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - The type of Kaposi sarcoma. - The general health of the patient, especially the patient's immune system. - Whether the cancer has just been diagnosed or has recurred (come back).",Kaposi Sarcoma,0000003_3,CancerGov,https://www.cancer.gov/types/soft-tissue-sarcoma/patient/kaposi-treatment-pdq,C0036220,T191,Disorders How to diagnose Kaposi Sarcoma ?,0000003_3-2,exams and tests,"Tests that examine the skin, lungs, and gastrointestinal tract are used to detect (find) and diagnose Kaposi sarcoma. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking skin and lymph nodes for signs of disease, such as lumps or anything else that seems unusual. A history of the patient's health habits and past illnesses and treatments will also be taken. - Chest x-ray : An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. This is used to find Kaposi sarcoma in the lungs. - Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. One of the following types of biopsies may be done to check for Kaposi sarcoma lesions in the skin: - Excisional biopsy : A scalpel is used to remove the entire skin growth. - Incisional biopsy : A scalpel is used to remove part of a skin growth. - Core biopsy : A wide needle is used to remove part of a skin growth. - Fine-needle aspiration (FNA) biopsy : A thin needle is used to remove part of a skin growth. An endoscopy or bronchoscopy may be done to check for Kaposi sarcoma lesions in the gastrointestinal tract or lungs. - Endoscopy for biopsy: A procedure to look at organs and tissues inside the body to check for abnormal areas. An endoscope is inserted through an incision (cut) in the skin or opening in the body, such as the mouth. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of disease. This is used to find Kaposi sarcoma lesions in the gastrointestinal tract. - Bronchoscopy for biopsy: A procedure to look inside the trachea and large airways in the lung for abnormal areas. A bronchoscope is inserted through the nose or mouth into the trachea and lungs. A bronchoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of disease. This is used to find Kaposi sarcoma lesions in the lungs. - After Kaposi sarcoma has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body. The following tests and procedures may be used to find out if cancer has spread to other parts of the body: - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the lung, liver, and spleen, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. This imaging test checks for signs of cancer in the lung, liver, and spleen. - CD34 lymphocyte count: A procedure in which a blood sample is checked to measure the amount of CD34 cells (a type of white blood cell). A lower than normal amount of CD34 cells can be a sign the immune system is not working well.",Kaposi Sarcoma,0000003_3,CancerGov,https://www.cancer.gov/types/soft-tissue-sarcoma/patient/kaposi-treatment-pdq,C0036220,T191,Disorders what research (or clinical trials) is being done for Kaposi Sarcoma ?,0000003_3-4,research,"New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Monoclonal antibody therapy and tyrosine kinase inhibitors (TKIs) are types of targeted therapy being studied in the treatment of Kaposi sarcoma. Monoclonal antibody therapy is a cancer treatment that uses antibodies made in the laboratory from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. These may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Bevacizumab is a monoclonal antibody that is being studied in the treatment of Kaposi sarcoma. TKIs are targeted therapy drugs that block signals needed for tumors to grow. Imatinib mesylate is a TKI being studied in the treatment of Kaposi sarcoma. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Kaposi Sarcoma,0000003_3,CancerGov,https://www.cancer.gov/types/soft-tissue-sarcoma/patient/kaposi-treatment-pdq,C0036220,T191,Disorders What are the treatments for Kaposi Sarcoma ?,0000003_3-5,treatment,"Key Points - There are different types of treatment for patients with Kaposi sarcoma. - Treatment of epidemic Kaposi sarcoma combines treatment for Kaposi sarcoma with treatment for AIDS. - Four types of standard treatment are used to treat Kaposi sarcoma: - Radiation therapy - Surgery - Chemotherapy - Biologic therapy - New types of treatment are being tested in clinical trials. - Targeted therapy - Treatment for Kaposi sarcoma may cause side effects. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with Kaposi sarcoma. Different types of treatments are available for patients with Kaposi sarcoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Treatment of epidemic Kaposi sarcoma combines treatment for Kaposi sarcoma with treatment for AIDS. For the treatment of epidemic Kaposi sarcoma, combined antiretroviral therapy (cART) is used to slow the progression of AIDS. cART may be combined with anticancer drugs and medicines that prevent and treat infections. Four types of standard treatment are used to treat Kaposi sarcoma: Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type of the cancer being treated. Certain types of external radiation therapy are used to treat Kaposi sarcoma lesions. Photon radiation therapy treats lesions with high-energy light. Electron beam radiation therapy uses tiny negatively charged particles called electrons. Surgery The following surgical procedures may be used for Kaposi sarcoma to treat small, surface lesions: - Local excision: The cancer is cut from the skin along with a small amount of normal tissue around it. - Electrodesiccation and curettage: The tumor is cut from the skin with a curette (a sharp, spoon-shaped tool). A needle-shaped electrode is then used to treat the area with an electric current that stops the bleeding and destroys cancer cells that remain around the edge of the wound. The process may be repeated one to three times during the surgery to remove all of the cancer. - Cryosurgery: A treatment that uses an instrument to freeze and destroy abnormal tissue. This type of treatment is also called cryotherapy. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, tissue, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). In electrochemotherapy, intravenous chemotherapy is given and a probe is used to send electric pulses to the tumor. The pulses make an opening in the membrane around the tumor cell and allow the chemotherapy to get inside. Electrochemotherapy is being studied in the treatment of Kaposi sarcoma. The way the chemotherapy is given depends on where the Kaposi sarcoma lesions occur in the body. In Kaposi sarcoma, chemotherapy may be given in the following ways: - For local Kaposi sarcoma lesions, such as in the mouth, anticancer drugs may be injected directly into the lesion (intralesional chemotherapy). - For local lesions on the skin, a topical agent may be applied to the skin as a gel. Electrochemotherapy may also be used. - For widespread lesions on the skin, intravenous chemotherapy may be given. Liposomal chemotherapy uses liposomes (very tiny fat particles) to carry anticancer drugs. Liposomal doxorubicin is used to treat Kaposi sarcoma. The liposomes build up in Kaposi sarcoma tissue more than in healthy tissue, and the doxorubicin is released slowly. This increases the effect of the doxorubicin and causes less damage to healthy tissue. See Drugs Approved for Kaposi Sarcoma for more information. Biologic therapy Biologic therapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy. Interferon alfa is a biologic agent used to treat Kaposi sarcoma. See Drugs Approved for Kaposi Sarcoma for more information. Treatment for Kaposi sarcoma may cause side effects. For information about side effects caused by treatment for cancer, see our Side Effects page. New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Monoclonal antibody therapy and tyrosine kinase inhibitors (TKIs) are types of targeted therapy being studied in the treatment of Kaposi sarcoma. Monoclonal antibody therapy is a cancer treatment that uses antibodies made in the laboratory from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. These may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Bevacizumab is a monoclonal antibody that is being studied in the treatment of Kaposi sarcoma. TKIs are targeted therapy drugs that block signals needed for tumors to grow. Imatinib mesylate is a TKI being studied in the treatment of Kaposi sarcoma. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Kaposi Sarcoma Classic Kaposi Sarcoma Treatment for single lesions may include the following: - Radiation therapy. - Surgery. Treatment for lesions all over the body may include the following: - Radiation therapy. - Chemotherapy. - A clinical trial of electrochemotherapy. Treatment for Kaposi sarcoma that affects lymph nodes or the gastrointestinal tract usually includes chemotherapy with or without radiation therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with classic Kaposi sarcoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Immunosuppressive Therapyrelated Kaposi Sarcoma Treatment for immunosuppressive therapyrelated Kaposi sarcoma may include the following: - Stopping or reducing immunosuppressive drug therapy. - Radiation therapy. - Chemotherapy using one or more anticancer drugs. Check the list of NCI-supported cancer clinical trials that are now accepting patients with immunosuppressive treatment related Kaposi sarcoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Epidemic Kaposi Sarcoma Treatment for epidemic Kaposi sarcoma may include the following: - Surgery, including local excision or electrodesiccation and curettage. - Cryosurgery. - Radiation therapy. - Chemotherapy using one or more anticancer drugs. - Biologic therapy. - A clinical trial of new drug therapy, biologic therapy, or targeted therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with AIDS-related Kaposi sarcoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Recurrent Kaposi Sarcoma Treatment for recurrent Kaposi sarcoma depends on which type of Kaposi sarcoma the patient has. Treatment may include a clinical trial of a new therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent Kaposi sarcoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Kaposi Sarcoma,0000003_3,CancerGov,https://www.cancer.gov/types/soft-tissue-sarcoma/patient/kaposi-treatment-pdq,C0036220,T191,Disorders Who is at risk for Childhood Rhabdomyosarcoma? ?,0000003_4-1,susceptibility,"Certain genetic conditions increase the risk of childhood rhabdomyosarcoma. Anything that increases the risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesnt mean that you will not get cancer. Talk with your childs doctor if you think your child may be at risk. Risk factors for rhabdomyosarcoma include having the following inherited diseases: - Li-Fraumeni syndrome. - Pleuropulmonary blastoma. - Neurofibromatosis type 1 (NF1). - Costello syndrome. - Beckwith-Wiedemann syndrome. - Noonan syndrome. Children who had a high birth weight or were larger than expected at birth may have an increased risk of embryonal rhabdomyosarcoma. In most cases, the cause of rhabdomyosarcoma is not known.",Childhood Rhabdomyosarcoma,0000003_4,CancerGov,https://www.cancer.gov/types/soft-tissue-sarcoma/patient/rhabdomyosarcoma-treatment-pdq,C0220611,T191,Disorders What are the symptoms of Childhood Rhabdomyosarcoma ?,0000003_4-2,symptoms,"A sign of childhood rhabdomyosarcoma is a lump or swelling that keeps getting bigger. Signs and symptoms may be caused by childhood rhabdomyosarcoma or by other conditions. The signs and symptoms that occur depend on where the cancer forms. Check with your child's doctor if your child has any of the following: - A lump or swelling that keeps getting bigger or does not go away. It may be painful. - Bulging of the eye. - Headache. - Trouble urinating or having bowel movements. - Blood in the urine. - Bleeding in the nose, throat, vagina, or rectum.",Childhood Rhabdomyosarcoma,0000003_4,CancerGov,https://www.cancer.gov/types/soft-tissue-sarcoma/patient/rhabdomyosarcoma-treatment-pdq,C0220611,T191,Disorders How to diagnose Childhood Rhabdomyosarcoma ?,0000003_4-3,exams and tests,"Diagnostic tests and a biopsy are used to detect (find) and diagnose childhood rhabdomyosarcoma. The diagnostic tests that are done depend in part on where the cancer forms. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - X-ray : An x-ray of the organs and bones inside the body, such as the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the abdomen, pelvis, or lymph nodes, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas of the body, such as the skull, brain, and lymph nodes. This procedure is also called nuclear magnetic resonance imaging (NMRI). - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. - Bone scan : A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones with cancer and is detected by a scanner. - Bone marrow aspiration and biopsy : The removal of bone marrow, blood, and a small piece of bone by inserting a hollow needle into the hipbone. Samples are removed from both hipbones. A pathologist views the bone marrow, blood, and bone under a microscope to look for signs of cancer. - Lumbar puncture : A procedure used to collect cerebrospinal fluid (CSF) from the spinal column. This is done by placing a needle between two bones in the spine and into the CSF around the spinal cord and removing a sample of the fluid. The sample of CSF is checked under a microscope for signs of cancer cells. This procedure is also called an LP or spinal tap. If these tests show there may be a rhabdomyosarcoma, a biopsy is done. A biopsy is the removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. Because treatment depends on the type of rhabdomyosarcoma, biopsy samples should be checked by a pathologist who has experience in diagnosing rhabdomyosarcoma. One of the following types of biopsies may be used: - Fine-needle aspiration (FNA) biopsy : The removal of tissue or fluid using a thin needle. - Core needle biopsy : The removal of tissue using a wide needle. This procedure may be guided using ultrasound, CT scan, or MRI. - Open biopsy : The removal of tissue through an incision (cut) made in the skin. - Sentinel lymph node biopsy : The removal of the sentinel lymph node during surgery. The sentinel lymph node is the first lymph node to receive lymphatic drainage from a tumor. It is the first lymph node the cancer is likely to spread to from the tumor. A radioactive substance and/or blue dye is injected near the tumor. The substance or dye flows through the lymph ducts to the lymph nodes. The first lymph node to receive the substance or dye is removed. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are not found, it may not be necessary to remove more lymph nodes. The following tests may be done on the sample of tissue that is removed: - Light microscopy: A laboratory test in which cells in a sample of tissue are viewed under regular and high-powered microscopes to look for certain changes in the cells. - Immunohistochemistry : A test that uses antibodies to check for certain antigens in a sample of tissue. The antibody is usually linked to a radioactive substance or a dye that causes the tissue to light up under a microscope. This type of test may be used to tell the difference between different types of cancer. - FISH (fluorescence in situ hybridization): A laboratory test used to look at genes or chromosomes in cells and tissues. Pieces of DNA that contain a fluorescent dye are made in the laboratory and added to cells or tissues on a glass slide. When these pieces of DNA attach to certain genes or areas of chromosomes on the slide, they light up when viewed under a microscope with a special light. This type of test is used to find certain gene changes. - Reverse transcriptionpolymerase chain reaction (RTPCR) test: A laboratory test in which cells in a sample of tissue are studied using chemicals to look for certain changes in the structure or function of genes. - Cytogenetic analysis : A laboratory test in which cells in a sample of tissue are viewed under a microscope to look for certain changes in the chromosomes.",Childhood Rhabdomyosarcoma,0000003_4,CancerGov,https://www.cancer.gov/types/soft-tissue-sarcoma/patient/rhabdomyosarcoma-treatment-pdq,C0220611,T191,Disorders What is the outlook for Childhood Rhabdomyosarcoma ?,0000003_4-4,outlook,"Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - The patient's age. - Where in the body the tumor started. - The size of the tumor at the time of diagnosis. - Whether the tumor has been completely removed by surgery. - The type of rhabdomyosarcoma (embryonal, alveolar, or anaplastic). - Whether there are certain changes in the genes. - Whether the tumor had spread to other parts of the body at the time of diagnosis. - Whether the tumor was in the lymph nodes at the time of diagnosis. - Whether the tumor responds to chemotherapy and/or radiation therapy. For patients with recurrent cancer, prognosis and treatment also depend on the following: - Where in the body the tumor recurred (came back). - How much time passed between the end of cancer treatment and when the cancer recurred. - Whether the tumor was treated with radiation therapy.",Childhood Rhabdomyosarcoma,0000003_4,CancerGov,https://www.cancer.gov/types/soft-tissue-sarcoma/patient/rhabdomyosarcoma-treatment-pdq,C0220611,T191,Disorders What are the stages of Childhood Rhabdomyosarcoma ?,0000003_4-5,stages,"Key Points - After childhood rhabdomyosarcoma has been diagnosed, treatment is based in part on the stage of the cancer and sometimes it is based on whether all the cancer was removed by surgery. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - Staging of childhood rhabdomyosarcoma is done in three parts. - The staging system is based on the size of the tumor, where it is in the body, and whether it has spread to other parts of the body: - Stage 1 - Stage 2 - Stage 3 - Stage 4 - The grouping system is based on whether the cancer has spread and whether all the cancer was removed by surgery: - Group I - Group II - Group III - Group IV - The risk group is based on the staging system and the grouping system. - Low-risk childhood rhabdomyosarcoma - Intermediate-risk childhood rhabdomyosarcoma - High-risk childhood rhabdomyosarcoma After childhood rhabdomyosarcoma has been diagnosed, treatment is based in part on the stage of the cancer and sometimes it is based on whether all the cancer was removed by surgery. The process used to find out if cancer has spread within the tissue or to other parts of the body is called staging. It is important to know the stage in order to plan treatment. The doctor will use results of the diagnostic tests to help find out the stage of the disease. Treatment for childhood rhabdomyosarcoma is based in part on the stage and sometimes on the amount of cancer that remains after surgery to remove the tumor. The pathologist will use a microscope to check the tissues removed during surgery, including tissue samples from the edges of the areas where the cancer was removed and the lymph nodes. This is done to see if all the cancer cells were taken out during the surgery. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if rhabdomyosarcoma spreads to the lung, the cancer cells in the lung are actually rhabdomyosarcoma cells. The disease is metastatic rhabdomyosarcoma, not lung cancer. Staging of childhood rhabdomyosarcoma is done in three parts. Childhood rhabdomyosarcoma is staged by using three different ways to describe the cancer: - A staging system. - A grouping system. - A risk group. The staging system is based on the size of the tumor, where it is in the body, and whether it has spread to other parts of the body: Stage 1 In stage 1, the tumor is any size, may have spread to lymph nodes, and is found in only one of the following ""favorable"" sites: - Eye or area around the eye. - Head and neck (but not in the tissue next to the brain and spinal cord). - Gallbladder and bile ducts. - Ureters or urethra. - Testes, ovary, vagina, or uterus. Rhabdomyosarcoma that forms in a ""favorable"" site has a better prognosis. If the site where cancer occurs is not one of the favorable sites listed above, it is said to be an ""unfavorable"" site. Stage 2 In stage 2, cancer is found in an ""unfavorable"" site (any one area not described as ""favorable"" in stage 1). The tumor is no larger than 5 centimeters and has not spread to lymph nodes. Stage 3 In stage 3, cancer is found in an ""unfavorable"" site (any one area not described as ""favorable"" in stage 1) and one of the following is true: - The tumor is no larger than 5 centimeters and cancer has spread to nearby lymph nodes. - The tumor is larger than 5 centimeters and cancer may have spread to nearby lymph nodes. Stage 4 In stage 4, the tumor may be any size and cancer may have spread to nearby lymph nodes. Cancer has spread to distant parts of the body, such as the lung, bone marrow, or bone. The grouping system is based on whether the cancer has spread and whether all the cancer was removed by surgery: Group I Cancer was found only in the place where it started and it was completely removed by surgery. Tissue was taken from the edges of where the tumor was removed. The tissue was checked under a microscope by a pathologist and no cancer cells were found. Group II Group II is divided into groups IIA, IIB, and IIC. - IIA: Cancer was removed by surgery but cancer cells were seen when the tissue, taken from the edges of where the tumor was removed, was viewed under a microscope by a pathologist. - IIB: Cancer had spread to nearby lymph nodes and the cancer and lymph nodes were removed by surgery. - IIC: Cancer had spread to nearby lymph nodes, the cancer and lymph nodes were removed by surgery, and at least one of the following is true: - Tissue taken from the edges of where the tumor was removed was checked under a microscope by a pathologist and cancer cells were seen. - The furthest lymph node from the tumor that was removed was checked under a microscope by a pathologist and cancer cells were seen. Group III Cancer was partly removed by biopsy or surgery but there is tumor remaining that can be seen with the eye. Group IV Cancer had spread to distant parts of the body when the cancer was diagnosed. - Cancer cells are found by an imaging test; or - There are cancer cells in the fluid around the brain, spinal cord, or lungs, or in fluid in the abdomen; or tumors are found in those areas. The risk group is based on the staging system and the grouping system. The risk group describes the chance that rhabdomyosarcoma will recur (come back). Every child treated for rhabdomyosarcoma should receive chemotherapy to decrease the chance cancer will recur. The type of anticancer drug, dose, and the number of treatments given depends on whether the child has low-risk, intermediate-risk, or high-risk rhabdomyosarcoma. The following risk groups are used: Low-risk childhood rhabdomyosarcoma Low-risk childhood rhabdomyosarcoma is one of the following: - An embryonal tumor of any size that is found in a ""favorable"" site. There may be tumor remaining after surgery that can be seen with or without a microscope. The cancer may have spread to nearby lymph nodes. The following areas are ""favorable"" sites: - Eye or area around the eye. - Head or neck (but not in the tissue near the ear, nose, sinuses, or base of the skull). - Gallbladder and bile ducts. - Ureter or urethra. - Testes, ovary, vagina, or uterus. - An embryonal tumor of any size that is not found in a ""favorable"" site. There may be tumor remaining after surgery that can be seen only with a microscope. The cancer may have spread to nearby lymph nodes. Intermediate-risk childhood rhabdomyosarcoma Intermediate-risk childhood rhabdomyosarcoma is one of the following: - An embryonal tumor of any size that is not found in one of the ""favorable"" sites listed above. There is tumor remaining after surgery, that can be seen with or without a microscope. The cancer may have spread to nearby lymph nodes. - An alveolar tumor of any size in a ""favorable"" or ""unfavorable"" site. There may be tumor remaining after surgery that can be seen with or without a microscope. The cancer may have spread to nearby lymph nodes. High-risk childhood rhabdomyosarcoma High-risk childhood rhabdomyosarcoma may be the embryonal type or the alveolar type. It may have spread to nearby lymph nodes and has spread to one or more of the following: - Other parts of the body that are not near where the tumor first formed. - Fluid around the brain or spinal cord. - Fluid in the lung or abdomen.",Childhood Rhabdomyosarcoma,0000003_4,CancerGov,https://www.cancer.gov/types/soft-tissue-sarcoma/patient/rhabdomyosarcoma-treatment-pdq,C0220611,T191,Disorders what research (or clinical trials) is being done for Childhood Rhabdomyosarcoma ?,0000003_4-6,research,"New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. High-dose chemotherapy with stem cell transplant High-dose chemotherapy with stem cell transplant is a way of giving high doses of chemotherapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. Immunotherapy Immunotherapy is a treatment that uses the patients immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the bodys natural defenses against cancer. This type of cancer treatment is also called biologic therapy or biotherapy. There are different types of immunotherapy: - Immune checkpoint inhibitor therapy uses the body's immune system to kill cancer cells. Two types of immune checkpoint inhibitors are being studied in the treatment of childhood rhabdomyosarcoma that has come back after treatment: - CTLA-4 is a protein on the surface of T cells that helps keep the bodys immune responses in check. When CTLA-4 attaches to another protein called B7 on a cancer cell, it stops the T cell from killing the cancer cell. CTLA-4 inhibitors attach to CTLA-4 and allow the T cells to kill cancer cells. Ipilimumab is a type of CTLA-4 inhibitor. - PD-1 is a protein on the surface of T cells that helps keep the bodys immune responses in check. When PD-1 attaches to another protein called PDL-1 on a cancer cell, it stops the T cell from killing the cancer cell. PD-1 inhibitors attach to PDL-1 and allow the T cells to kill cancer cells. Nivolumab and pembrolizumab are PD-1 inhibitors. - Vaccine therapy is a type of immunotherapy being studied to treat metastatic rhabdomyosarcoma. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to attack cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation do. There are different types of targeted therapy: - mTOR inhibitors stop the protein that helps cells divide and survive. Sirolimus is a type of mTOR inhibitor therapy being studied in the treatment of recurrent rhabdomyosarcoma. - Tyrosine kinase inhibitors are small-molecule drugs that go through the cell membrane and work inside cancer cells to block signals that cancer cells need to grow and divide. MK-1775 is a tyrosine kinase inhibitor being studied in the treatment of recurrent rhabdomyosarcoma. - Antibody-drug conjugates are made up of a monoclonal antibody attached to a drug. The monoclonal antibody binds to specific proteins or receptors found on certain cells, including cancer cells. The drug enters these cells and kills them without harming other cells. Lorvotuzumab mertansine is an antibody-drug conjugate being studied in the treatment of recurrent rhabdomyosarcoma. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Childhood Rhabdomyosarcoma,0000003_4,CancerGov,https://www.cancer.gov/types/soft-tissue-sarcoma/patient/rhabdomyosarcoma-treatment-pdq,C0220611,T191,Disorders What are the treatments for Childhood Rhabdomyosarcoma ?,0000003_4-7,treatment,"Key Points - There are different types of treatment for patients with childhood rhabdomyosarcoma. - Children with rhabdomyosarcoma should have their treatment planned by a team of health care providers who are experts in treating cancer in children. - Treatment for childhood rhabdomyosarcoma may cause side effects. - Three types of standard treatment are used: - Surgery - Radiation therapy - Chemotherapy - New types of treatment are being tested in clinical trials. - High-dose chemotherapy with stem cell transplant - Immunotherapy - Targeted therapy - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with childhood rhabdomyosarcoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment. Children with rhabdomyosarcoma should have their treatment planned by a team of health care providers who are experts in treating cancer in children. Because rhabdomyosarcoma can form in many different parts of the body, many different kinds of treatments are used. Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other health care providers who are experts in treating children with rhabdomyosarcoma and who specialize in certain areas of medicine. These may include the following specialists: - Pediatrician. - Pediatric surgeon. - Radiation oncologist. - Pediatric hematologist. - Pediatric radiologist. - Pediatric nurse specialist. - Geneticist or cancer genetics risk counselor. - Social worker. - Rehabilitation specialist. Treatment for childhood rhabdomyosarcoma may cause side effects. For information about side effects that begin during treatment for cancer, see our Side Effects page. Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of cancer treatment for rhabdomyosarcoma may include: - Physical problems. - Changes in mood, feelings, thinking, learning, or memory. - Second cancers (new types of cancer). Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information.) Three types of standard treatment are used: Surgery Surgery (removing the cancer in an operation) is used to treat childhood rhabdomyosarcoma. A type of surgery called wide local excision is often done. A wide local excision is the removal of tumor and some of the tissue around it, including the lymph nodes. A second surgery may be needed to remove all the cancer. Whether surgery is done and the type of surgery done depends on the following: - Where in the body the tumor started. - The effect the surgery will have on the way the child will look. - The effect the surgery will have on the child's important body functions. - How the tumor responded to chemotherapy or radiation therapy that may have been given first. In most children with rhabdomyosarcoma, it is not possible to remove all of the tumor by surgery. Rhabdomyosarcoma can form in many different places in the body and the surgery will be different for each site. Surgery to treat rhabdomyosarcoma of the eye or genital areas is usually a biopsy. Chemotherapy, and sometimes radiation therapy, may be given before surgery to shrink large tumors. Even if the doctor removes all the cancer that can be seen at the time of the surgery, patients will be given chemotherapy after surgery to kill any cancer cells that are left. Radiation therapy may also be given. Treatment given after the surgery to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or stop them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. Certain ways of giving radiation therapy can help keep radiation from damaging nearby healthy tissue. These types of external radiation therapy include the following: - Conformal radiation therapy: Conformal radiation therapy is a type of external radiation therapy that uses a computer to make a 3-dimensional (3-D) picture of the tumor and shapes the radiation beams to fit the tumor. This allows a high dose of radiation to reach the tumor and causes less damage to nearby healthy tissue. - Intensity-modulated radiation therapy (IMRT): IMRT is a type of 3-dimensional (3-D) radiation therapy that uses a computer to make pictures of the size and shape of the tumor. Thin beams of radiation of different intensities (strengths) are aimed at the tumor from many angles. - Volumetrical modulated arc therapy (VMAT): VMAT is type of 3-D radiation therapy that uses a computer to make pictures of the size and shape of the tumor. The radiation machine moves in a circle around the patient once during treatment and sends thin beams of radiation of different intensities (strengths) at the tumor. Treatment with VMAT is delivered faster than treatment with IMRT. - Stereotactic body radiation therapy: Stereotactic body radiation therapy is a type of external radiation therapy. Special equipment is used to place the patient in the same position for each radiation treatment. Once a day for several days, a radiation machine aims a larger than usual dose of radiation directly at the tumor. By having the patient in the same position for each treatment, there is less damage to nearby healthy tissue. This procedure is also called stereotactic external-beam radiation therapy and stereotaxic radiation therapy. - Proton beam radiation therapy: Proton-beam therapy is a type of high-energy, external radiation therapy. A radiation therapy machine aims streams of protons (tiny, invisible, positively-charged particles) at the cancer cells to kill them. This type of treatment causes less damage to nearby healthy tissue. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. It is used to treat cancer in areas such as the vagina, vulva, uterus, bladder, prostate, head, or neck. Internal radiation therapy is also called brachytherapy, internal radiation, implant radiation, or interstitial radiation therapy. The type and amount of radiation therapy and when it is given depends on the age of the child, the type of rhabdomyosarcoma, where in the body the tumor started, how much tumor remained after surgery, and whether there is tumor in the nearby lymph nodes. External radiation therapy is usually used to treat childhood rhabdomyosarcoma but in certain cases internal radiation therapy is used. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Chemotherapy may also be given to shrink the tumor before surgery in order to save as much healthy tissue as possible. This is called neoadjuvant chemotherapy. Every child treated for rhabdomyosarcoma should receive systemic chemotherapy to decrease the chance the cancer will recur. The type of anticancer drug, dose, and the number of treatments given depends on whether the child has low-risk, intermediate-risk, or high-risk rhabdomyosarcoma. See Drugs Approved for Rhabdomyosarcoma for more information. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Childhood Rhabdomyosarcoma Previously Untreated Childhood Rhabdomyosarcoma The treatment of childhood rhabdomyosarcoma often includes surgery, radiation therapy, and chemotherapy. The order that these treatments are given depends on where in the body the tumor started, the size of the tumor, the type of tumor, and whether the tumor has spread to lymph nodes or other parts of the body. See the Treatment Option Overview section of this summary for more information about surgery, radiation therapy, and chemotherapy used to treat children with rhabdomyosarcoma. Rhabdomyosarcoma of the brain and head and neck - For tumors of the brain: Treatment may include surgery to remove the tumor, radiation therapy, and chemotherapy. - For tumors of the head and neck that are in or near the eye: Treatment may include chemotherapy and radiation therapy. If the tumor remains or comes back after treatment with chemotherapy and radiation therapy, surgery to remove the eye and some tissues around the eye may be needed. - For tumors of the head and neck that are near the ear, nose, sinuses, or base of the skull but not in or near the eye: Treatment may include radiation therapy and chemotherapy. - For tumors of the head and neck that are not in or near the eye and not near the ear, nose, sinuses, or base of the skull: Treatment may include chemotherapy, radiation therapy, and surgery to remove the tumor. - For tumors of the head and neck that cannot be removed by surgery: Treatment may include chemotherapy and radiation therapy including stereotactic body radiation therapy. - For tumors of the larynx (voice box): Treatment may include chemotherapy and radiation therapy. Surgery to remove the larynx is usually not done, so that the voice is not harmed. Rhabdomyosarcoma of the arms or legs - Chemotherapy followed by surgery to remove the tumor. If the tumor was not completely removed, a second surgery to remove the tumor may be done. Radiation therapy may also be given. - For tumors of the hand or foot, radiation therapy and chemotherapy may be given. The tumor may not be removed because it would affect the function of the hand or foot. - Lymph node dissection (one or more lymph nodes are removed and a sample of tissue is checked under a microscope for signs of cancer). - For tumors in the arms, lymph nodes near the tumor and in the armpit area are removed. - For tumors in the legs, lymph nodes near the tumor and in the groin area are removed. Rhabdomyosarcoma of the chest, abdomen, or pelvis - For tumors in the chest or abdomen (including the chest wall or abdominal wall): Surgery (wide local excision) may be done. If the tumor is large, chemotherapy and radiation therapy are given to shrink the tumor before surgery. - For tumors of the pelvis: Surgery (wide local excision) may be done. If the tumor is large, chemotherapy is given to shrink the tumor before surgery. Radiation therapy may be given after surgery. - For tumors of the diaphragm: A biopsy of the tumor is followed by chemotherapy and radiation therapy to shrink the tumor. Surgery may be done later to remove any remaining cancer cells. - For tumors of the gallbladder or bile ducts: A biopsy of the tumor is followed by chemotherapy and radiation therapy. - For tumors of the muscles or tissues around the anus or between the vulva and the anus or the scrotum and the anus: Surgery is done to remove as much of the tumor as possible and some nearby lymph nodes, followed by chemotherapy and radiation therapy. Rhabdomyosarcoma of the kidney - For tumors of the kidney: Surgery to remove as much of the tumor as possible. Chemotherapy and radiation therapy may also be given. Rhabdomyosarcoma of the bladder and prostate - For tumors that are only at the top of the bladder: Surgery (wide local excision) is done. - For tumors of the prostate or bladder (other than the top of the bladder): - Chemotherapy and radiation therapy are given first to shrink the tumor. If cancer cells remain after chemotherapy and radiation therapy, the tumor is removed by surgery. Surgery may include removal of the prostate, part of the bladder, or pelvic exenteration without removal of the rectum. (This may include removal of the lower colon and bladder. In girls, the cervix, vagina, ovaries, and nearby lymph nodes may be removed). - Chemotherapy is given first to shrink the tumor. Surgery to remove the tumor, but not the bladder or prostate, is done. Internal or external radiation therapy may be given after surgery. Rhabdomyosarcoma of the area near the testicles - Surgery to remove the testicle and spermatic cord. The lymph nodes in the back of the abdomen may be checked for cancer, especially if the lymph nodes are large or the child is 10 years or older. - Radiation therapy may be given if the tumor cannot be completely removed by surgery. Rhabdomyosarcoma of the vulva, vagina, uterus, cervix, or ovary - For tumors of the vulva and vagina: Treatment may include chemotherapy followed by surgery to remove the tumor. Internal or external radiation therapy may be given after surgery. - For tumors of the uterus: Treatment may include chemotherapy with or without radiation therapy. Sometimes surgery may be needed to remove any remaining cancer cells. - For tumors of the cervix: Treatment may include chemotherapy followed by surgery to remove any remaining tumor. - For tumors of the ovary: Treatment may include chemotherapy followed by surgery to remove any remaining tumor. Metastatic rhabdomyosarcoma Treatment, such as chemotherapy, radiation therapy, or surgery to remove the tumor, is given to the site where the tumor first formed. If the cancer has spread to the brain, spinal cord, or lungs, radiation therapy may also be given to the sites where the cancer has spread. The following treatment is being studied for metastatic rhabdomyosarcoma: - A clinical trial of immunotherapy (vaccine therapy). Check the list of NCI-supported cancer clinical trials that are now accepting patients with previously untreated childhood rhabdomyosarcoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Recurrent Childhood Rhabdomyosarcoma Treatment options for recurrent childhood rhabdomyosarcoma are based on many factors, including where in the body the cancer has come back, what type of treatment the child had before, and the needs of the child. Treatment of recurrent rhabdomyosarcoma may include one or more of the following: - Surgery. - Radiation therapy. - Chemotherapy. - A clinical trial of high-dose chemotherapy followed by stem cell transplant using the patient's own stem cells. - A clinical trial of targeted therapy or immunotherapy (sirolimus, lorvotuzumab, ipilimumab, nivolumab, or pembrolizumab). - A clinical trial of targeted therapy with a tyrosine kinase inhibitor (MK-1775) and chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent childhood rhabdomyosarcoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. High-dose chemotherapy with stem cell transplant High-dose chemotherapy with stem cell transplant is a way of giving high doses of chemotherapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. Immunotherapy Immunotherapy is a treatment that uses the patients immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the bodys natural defenses against cancer. This type of cancer treatment is also called biologic therapy or biotherapy. There are different types of immunotherapy: - Immune checkpoint inhibitor therapy uses the body's immune system to kill cancer cells. Two types of immune checkpoint inhibitors are being studied in the treatment of childhood rhabdomyosarcoma that has come back after treatment: - CTLA-4 is a protein on the surface of T cells that helps keep the bodys immune responses in check. When CTLA-4 attaches to another protein called B7 on a cancer cell, it stops the T cell from killing the cancer cell. CTLA-4 inhibitors attach to CTLA-4 and allow the T cells to kill cancer cells. Ipilimumab is a type of CTLA-4 inhibitor. - PD-1 is a protein on the surface of T cells that helps keep the bodys immune responses in check. When PD-1 attaches to another protein called PDL-1 on a cancer cell, it stops the T cell from killing the cancer cell. PD-1 inhibitors attach to PDL-1 and allow the T cells to kill cancer cells. Nivolumab and pembrolizumab are PD-1 inhibitors. - Vaccine therapy is a type of immunotherapy being studied to treat metastatic rhabdomyosarcoma. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to attack cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation do. There are different types of targeted therapy: - mTOR inhibitors stop the protein that helps cells divide and survive. Sirolimus is a type of mTOR inhibitor therapy being studied in the treatment of recurrent rhabdomyosarcoma. - Tyrosine kinase inhibitors are small-molecule drugs that go through the cell membrane and work inside cancer cells to block signals that cancer cells need to grow and divide. MK-1775 is a tyrosine kinase inhibitor being studied in the treatment of recurrent rhabdomyosarcoma. - Antibody-drug conjugates are made up of a monoclonal antibody attached to a drug. The monoclonal antibody binds to specific proteins or receptors found on certain cells, including cancer cells. The drug enters these cells and kills them without harming other cells. Lorvotuzumab mertansine is an antibody-drug conjugate being studied in the treatment of recurrent rhabdomyosarcoma. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Childhood Rhabdomyosarcoma,0000003_4,CancerGov,https://www.cancer.gov/types/soft-tissue-sarcoma/patient/rhabdomyosarcoma-treatment-pdq,C0220611,T191,Disorders What is (are) Childhood Soft Tissue Sarcoma ?,0000003_5-1,information,"Key Points - Childhood soft tissue sarcoma is a disease in which malignant (cancer) cells form in soft tissues of the body. - Soft tissue sarcoma occurs in children and adults. - Having certain diseases and inherited disorders can increase the risk of childhood soft tissue sarcoma. - The most common sign of childhood soft tissue sarcoma is a painless lump or swelling in soft tissues of the body. - Diagnostic tests are used to detect (find) and diagnose childhood soft tissue sarcoma. - If tests show there may be a soft tissue sarcoma, a biopsy is done. - There are many different types of soft tissue sarcomas. - Fat tissue tumors - Bone and cartilage tumors - Fibrous (connective) tissue tumors - Skeletal muscle tumors - Smooth muscle tumors - So-called fibrohistiocytic tumors - Peripheral nervous system tumors - Pericytic (Perivascular) Tumors - Tumors of unknown origin - Blood vessel tumors - Certain factors affect prognosis (chance of recovery) and treatment options. Childhood soft tissue sarcoma is a disease in which malignant (cancer) cells form in soft tissues of the body. Soft tissues of the body connect, support, and surround other body parts and organs. The soft tissues include the following: - Fat. - A mix of bone and cartilage. - Fibrous tissue. - Muscles. - Nerves. - Tendons (bands of tissue that connect muscles to bones). - Synovial tissues (tissues around joints). - Blood vessels. - Lymph vessels. Soft tissue sarcoma may be found anywhere in the body. In children, the tumors form most often in the arms, legs, or trunk (chest and abdomen). Soft tissue sarcoma occurs in children and adults. Soft tissue sarcoma in children may respond differently to treatment, and may have a better prognosis than soft tissue sarcoma in adults. (See the PDQ summary on Adult Soft Tissue Sarcoma Treatment for information on treatment in adults.) There are many different types of soft tissue sarcomas. The cells of each type of sarcoma look different under a microscope. The soft tissue tumors are grouped based on the type of soft tissue cell where they first formed. This summary is about the following types of soft tissue sarcoma: Fat tissue tumors - Liposarcoma . This is a rare cancer of the fat cells. Liposarcoma usually forms in the fat layer just under the skin. In children and adolescents, liposarcoma is often low grade (likely to grow and spread slowly). There are several different types of liposarcoma. Myxoid liposarcoma is usually low grade and responds well to treatment. The cells of myxoid liposarcoma have a certain genetic change called a translocation (part of one chromosome switches places with part of another chromosome). In order to diagnose myxoid liposarcoma, the tumor cells are checked for this genetic change. Pleomorphic liposarcoma is usually high grade (likely to grow and spread quickly) and is less likely to respond well to treatment. Bone and cartilage tumors Bone and cartilage tumors are a mix of bone cells and cartilage cells. Bone and cartilage tumors include the following types: - Extraskeletal mesenchymal chondrosarcoma . This type of bone and cartilage tumor often affects young adults and occurs in the head and neck. - Extraskeletal osteosarcoma . This type of bone and cartilage tumor is very rare in children and adolescents. It is likely to come back after treatment and may spread to the lungs. Fibrous (connective) tissue tumors Fibrous (connective) tissue tumors include the following types: - Desmoid-type fibromatosis (also called desmoid tumor or aggressive fibromatosis). This fibrous tissue tumor is low grade (likely to grow slowly). It may come back in nearby tissues but usually does not spread to distant parts of the body. Rarely, the tumor may disappear without treatment. Desmoid tumors sometimes occur in children with changes in the adenomatous polyposis coli (APC) gene. Changes in this gene cause familial adenomatous polyposis (FAP). FAP is an inherited condition in which many polyps (growths on mucous membranes) form on the inside walls of the colon and rectum. Genetic counseling (a discussion with a trained professional about inherited diseases and a possible need for gene testing) may be needed. - Dermatofibrosarcoma protuberans . This is a rare tumor of the deep layers of the skin found in children and adults. The cells of this tumor have a certain genetic change called a translocation (part of one chromosome switches places with part of another chromosome). In order to diagnose dermatofibrosarcoma protuberans, the tumor cells are checked for this genetic change. - Fibrosarcoma . There are two types of fibrosarcoma in children and adolescents: - Infantile fibrosarcoma (also called congenital fibrosarcoma). This type of fibrosarcoma is found in children aged 4 years and younger. It most often occurs in infants and may be seen in a prenatal ultrasound exam. This tumor is often large and fast growing, but rarely spreads to distant parts of the body. The cells of this tumor usually have a certain genetic change called a translocation (part of one chromosome switches places with part of another chromosome). In order to diagnose infantile fibrosarcoma, the tumor cells are checked for this genetic change. - Adult-type fibrosarcoma. This is the same type of fibrosarcoma found in adults. The cells of this tumor do not have the genetic change found in infantile fibrosarcoma. See the PDQ summary on Adult Soft Tissue Sarcoma Treatment for more information. - Inflammatory myofibroblastic tumor . This is a fibrous tissue tumor that occurs in children and adolescents. It is likely to come back after treatment but rarely spreads to distant parts of the body. A certain genetic change has been found in about half of these tumors. - Low-grade fibromyxoid sarcoma . This is a slow-growing tumor that affects young and middle-aged adults. The cells of this tumor usually have a certain genetic change called a translocation (part of one chromosome switches places with part of another chromosome). In order to diagnose low-grade fibromyxoid sarcoma, the tumor cells are checked for this genetic change. The tumor may come back many years after treatment and spread to the lungs and the lining of the wall of the chest cavity. Lifelong follow-up is needed. - Myxofibrosarcoma . This is a rare fibrous tissue tumor that is found less often in children than in adults. - Sclerosing epithelioid fibrosarcoma . This is a rare fibrous tissue tumor that can come back and spread to other places years after treatment. Long-term follow-up is needed. Skeletal muscle tumors Skeletal muscle is attached to bones and helps the body move. - Rhabdomyosarcoma . Rhabdomyosarcoma is the most common childhood soft tissue sarcoma in children 14 years and younger. See the PDQ summary on Childhood Rhabdomyosarcoma Treatment for more information. Smooth muscle tumors Smooth muscle lines the inside of blood vessels and hollow internal organs such as the stomach, intestines, bladder, and uterus. - Leiomyosarcoma . This smooth muscle tumor has been linked with Epstein-Barr virus in children who also have HIV disease or AIDS. Leiomyosarcoma may also form as a second cancer in survivors of inherited retinoblastoma, sometimes many years after the initial treatment for retinoblastoma. So-called fibrohistiocytic tumors - Plexiform fibrohistiocytic tumor . This is a rare tumor that usually affects children and young adults. The tumor usually starts as a painless growth on or just under the skin on the arm, hand, or wrist. It may rarely spread to nearby lymph nodes or to the lungs. Peripheral nervous system tumors Peripheral nervous system tumors include the following types: - Ectomesenchymoma . This is a rare, fast-growing tumor of the nerve sheath (protective covering of nerves that are not part of the brain or spinal cord) that occurs mainly in children. Ectomesenchymomas may form in the head and neck, abdomen, perineum, scrotum, arms, or legs. - Malignant peripheral nerve sheath tumor . This is a tumor that forms in the nerve sheath. Some children who have a malignant peripheral nerve sheath tumor have a rare genetic condition called neurofibromatosis type 1 (NF1). This tumor may be low grade or high grade. - Malignant triton tumor . These are very rare, fast-growing tumors that occur most often in children with NF1. Pericytic (Perivascular) Tumors Pericytic tumors form in cells that wrap around blood vessels. Pericytic tumors include the following types: - Myopericytoma . Infantile hemangiopericytoma is a type of myopericytoma. Children younger than 1 year at the time of diagnosis may have a better prognosis. In patients older than 1 year, infantile hemangiopericytoma is more likely to spread to other parts of the body, including the lymph nodes and lungs. - Infantile myofibromatosis . Infantile myofibromatosis is another type of myopericytoma. It is a fibrous tumor that often forms in the first 2 years of life. There may be one nodule under the skin, usually in the head and neck area (myofibroma), or nodules in several skin areas, muscle, and bone (myofibromatosis). These tumors may go away without treatment. Tumors of unknown origin Tumors of unknown origin (the place where the tumor first formed is not known) include the following types: - Alveolar soft part sarcoma . This is a rare tumor of the soft supporting tissue that connects and surrounds the organs and other tissues. It is most commonly found in the limbs but can occur in the tissues of the mouth, jaws, and face. It may grow slowly and may have spread to other parts of the body at the time of diagnosis. Alveolar soft part sarcoma may have a better prognosis when the tumor is 5 centimeters or smaller or when the tumor is completely removed by surgery. The cells of this tumor usually have a certain genetic change called a translocation (part of one chromosome switches places with part of another chromosome). In order to diagnose alveolar soft part sarcoma, the tumor cells are checked for this genetic change. - Clear cell sarcoma of soft tissue . This is a slow-growing soft tissue tumor that begins in a tendon (tough, fibrous, cord-like tissue that connects muscle to bone or to another part of the body). Clear cell sarcoma most commonly occurs in deep tissue of the foot, heel, and ankle. It may spread to nearby lymph nodes. The cells of this tumor usually have a certain genetic change called a translocation (part of one chromosome switches places with part of another chromosome). In order to diagnose clear cell sarcoma of soft tissue, the tumor cells are checked for this genetic change. - Desmoplastic small round cell tumor . This tumor most often forms in the abdomen, pelvis or tissues around the testes, but it may form in the kidney. Desmoplastic small round cell tumor may also spread to the lungs and other parts of the body. The cells of this tumor usually have a certain genetic change called a translocation (part of one chromosome switches places with part of another chromosome). In order to diagnose desmoplastic small round cell tumor, the tumor cells are checked for this genetic change. - Epithelioid sarcoma . This is a rare sarcoma that usually starts deep in soft tissue as a slow growing, firm lump and may spread to the lymph nodes. - Extrarenal (extracranial) rhabdoid tumor . This is a rare, fast-growing tumor of soft tissues such as the liver and peritoneum. It usually occurs in young children, including newborns, but it can occur in older children and adults. Rhabdoid tumors may be linked to a change in a tumor suppressor gene called SMARCB1. This type of gene makes a protein that helps control cell growth. Changes in the SMARCB1 gene may be inherited (passed on from parents to offspring). Genetic counseling (a discussion with a trained professional about inherited diseases and a possible need for gene testing) may be needed. - Extraskeletal myxoid chondrosarcoma . This is a rare soft tissue sarcoma that may be found in children and adolescents. Over time, it tends to spread to other parts of the body, including the lymph nodes and lungs. The cells of this tumor usually have a genetic change, often a translocation (part of one chromosome switches places with part of another chromosome). In order to diagnose extraskeletal myxoid chondrosarcoma, the tumor cells are checked for this genetic change. The tumor may come back many years after treatment. - Perivascular epithelioid cell tumors (PEComas). Benign (not cancer) PEComas may be found in children with an inherited condition called tuberous sclerosis. They occur in the stomach, intestines, lungs, female reproductive organs, and genitourinary organs. - Primitive neuroectodermal tumor (PNET)/extraskeletal Ewing tumor . See the PDQ summary on Ewing Sarcoma Treatment for information. - Synovial sarcoma . Synovial sarcoma is a common type of soft tissue sarcoma in children and adolescents. Synovial sarcoma usually forms in the tissues around the joints in the arms or legs, but may also form in the trunk, head, or neck. The cells of this tumor usually have a certain genetic change called a translocation (part of one chromosome switches places with part of another chromosome). Larger tumors have a greater risk of spreading to other parts of the body, including the lungs. Children younger than 10 years and those whose tumor is 5 centimeters or smaller have a better prognosis. - Undifferentiated /unclassified sarcoma . These tumors usually occur in the muscles that are attached to bones and that help the body move. - Undifferentiated pleomorphic sarcoma /malignant fibrous histiocytoma (high-grade). This type of soft tissue tumor may form in parts of the body where patients have received radiation therapy in the past, or as a second cancer in children with retinoblastoma. The tumor is usually found on the arms or legs and may spread to other parts of the body. See the PDQ summary on Osteosarcoma and Malignant Fibrous Histiocytoma of Bone Treatment for information about malignant fibrous histiocytoma of bone. Blood vessel tumors Blood vessel tumors include the following types: - Angiosarcoma of the soft tissue. Angiosarcoma of the soft tissue is a fast-growing tumor that forms in blood vessels or lymph vessels in any part of the body. Most angiosarcomas are in or just under the skin. Those in deeper soft tissue can form in the liver, spleen, and lung. They are very rare in children, who sometimes have more than one tumor in the skin or liver. Rarely, infantile hemangioma may become angiosarcoma of the soft tissue. (See the PDQ summary on Childhood Vascular Tumors Treatment for more information.) - Epithelioid hemangioendothelioma. Epithelioid hemangioendotheliomas can occur in children, but are most common in adults between 30 and 50 years of age. They usually occur in the liver, lung, or bone. They may be either fast growing or slow growing. In about a third of cases, the tumor spreads to other parts of the body very quickly. (See the PDQ summary on Childhood Vascular Tumors Treatment for more information.) See the following PDQ summaries for information about types of soft tissue sarcoma not included in this summary: - Childhood Rhabdomyosarcoma Treatment. - Ewing Sarcoma Treatment. - Unusual Cancers of Childhood Treatment (gastrointestinal stromal tumors).",Childhood Soft Tissue Sarcoma,0000003_5,CancerGov,https://www.cancer.gov/types/soft-tissue-sarcoma/patient/child-soft-tissue-treatment-pdq,C0220645,T191,Disorders Who is at risk for Childhood Soft Tissue Sarcoma? ?,0000003_5-2,susceptibility,Having certain diseases and inherited disorders can increase the risk of childhood soft tissue sarcoma. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesnt mean that you will not get cancer. Talk with your childs doctor if you think your child may be at risk. Risk factors for childhood soft tissue sarcoma include having the following inherited disorders: - Li-Fraumeni syndrome. - Familial adenomatous polyposis (FAP). - Retinoblastoma 1 gene changes. - Neurofibromatosis type 1 (NF1). - Werner syndrome. Other risk factors include the following: - Past treatment with radiation therapy. - Having AIDS (acquired immune deficiency syndrome) and Epstein-Barr virus infection at the same time.,Childhood Soft Tissue Sarcoma,0000003_5,CancerGov,https://www.cancer.gov/types/soft-tissue-sarcoma/patient/child-soft-tissue-treatment-pdq,C0220645,T191,Disorders What are the symptoms of Childhood Soft Tissue Sarcoma ?,0000003_5-3,symptoms,"The most common sign of childhood soft tissue sarcoma is a painless lump or swelling in soft tissues of the body. A sarcoma may appear as a painless lump under the skin, often on an arm, a leg, or the trunk. There may be no other signs or symptoms at first. As the sarcoma gets bigger and presses on nearby organs, nerves, muscles, or blood vessels, it may cause signs or symptoms, such as pain or weakness. Other conditions may cause the same signs and symptoms. Check with your childs doctor if your child has any of these problems.",Childhood Soft Tissue Sarcoma,0000003_5,CancerGov,https://www.cancer.gov/types/soft-tissue-sarcoma/patient/child-soft-tissue-treatment-pdq,C0220645,T191,Disorders How to diagnose Childhood Soft Tissue Sarcoma ?,0000003_5-4,exams and tests,"Diagnostic tests are used to detect (find) and diagnose childhood soft tissue sarcoma. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - X-rays : An x-ray is a type of energy beam that can go through the body onto film, making pictures of areas inside the body. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas of the body, such as the chest, abdomen, arms, or legs. This procedure is also called nuclear magnetic resonance imaging (NMRI). - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the chest or abdomen, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later. - If tests show there may be a soft tissue sarcoma, a biopsy is done. One of the following types of biopsies is usually used: - Core needle biopsy : The removal of tissue using a wide needle. This procedure may be guided using ultrasound, CT scan, or MRI. - Incisional biopsy : The removal of part of a lump or a sample of tissue. - Excisional biopsy : The removal of an entire lump or area of tissue that doesnt look normal. A pathologist views the tissue under a microscope to look for cancer cells. An excisional biopsy may be used to completely remove smaller tumors that are near the surface of the skin. This type of biopsy is rarely used because cancer cells may remain after the biopsy. If cancer cells remain, the cancer may come back or it may spread to other parts of the body. An MRI of the tumor is done before the excisional biopsy. This is done to show where the original tumor is and may be used to guide future surgery or radiation therapy. The placement of needles or incisions for the biopsy can affect the success of later surgery to remove the tumor. If possible, the surgeon who will remove any tumor that is found should be involved in planning the biopsy. In order to plan the best treatment, the sample of tissue removed during the biopsy must be large enough to find out the type of soft tissue sarcoma and do other laboratory tests. Tissue samples will be taken from the primary tumor, lymph nodes, and other areas that may have cancer cells. A pathologist views the tissue under a microscope to look for cancer cells and to find out the type and grade of the tumor. The grade of a tumor depends on how abnormal the cancer cells look under a microscope and how quickly the cells are dividing. High-grade and mid-grade tumors usually grow and spread more quickly than low-grade tumors. Because soft tissue sarcoma can be hard to diagnose, the tissue sample should be checked by a pathologist who has experience in diagnosing soft tissue sarcoma. One or more of the following laboratory tests may be done to study the tissue samples: - Molecular test : A laboratory test to check for certain genes, proteins, or other molecules in a sample of tissue, blood, or other body fluid. A molecular test may be done with other procedures, such as biopsies, to help diagnose some types of cancer. Molecular tests check for certain gene or chromosome changes that occur in some soft tissue sarcomas. - Reverse transcriptionpolymerase chain reaction (RTPCR) test: A laboratory test in which cells in a sample of tissue are studied using chemicals to look for changes in the expression of certain genes. When genes are expressed they make specific proteins that are needed for the structure, function, and monitoring of the bodys tissues and organs. This test is done in order to identify the type of tumor. - Cytogenetic analysis : A laboratory test in which cells in a sample of bone marrow, blood, amniotic fluid, tumor or other tissue is viewed under a microscope to look for changes in the chromosomes. Fluorescence in situ hybridization (FISH) is a type of cytogenetic analysis. - Immunocytochemistry : A test that uses antibodies to check for certain antigens (markers) in a sample of cells. The antibody is usually linked to an enzyme or fluorescent dye that causes the cells that have that marker to become visible under a microscope. This type of test may be used to tell the difference between different types of soft tissue sarcoma.",Childhood Soft Tissue Sarcoma,0000003_5,CancerGov,https://www.cancer.gov/types/soft-tissue-sarcoma/patient/child-soft-tissue-treatment-pdq,C0220645,T191,Disorders What is the outlook for Childhood Soft Tissue Sarcoma ?,0000003_5-5,outlook,Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - The part of the body where the tumor first formed. - The size and grade of the tumor. - The type of soft tissue sarcoma. - How deep the tumor is under the skin. - Whether the tumor has spread to other places in the body. - The amount of tumor remaining after surgery to remove it. - Whether radiation therapy was used to treat the tumor. - The age and gender of the patient. - Whether the cancer has just been diagnosed or has recurred (come back).,Childhood Soft Tissue Sarcoma,0000003_5,CancerGov,https://www.cancer.gov/types/soft-tissue-sarcoma/patient/child-soft-tissue-treatment-pdq,C0220645,T191,Disorders What are the stages of Childhood Soft Tissue Sarcoma ?,0000003_5-6,stages,"Key Points - After childhood soft tissue sarcoma has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. After childhood soft tissue sarcoma has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body. The process used to find out if cancer has spread within the soft tissue or to other parts of the body is called staging. There is no standard staging system for childhood soft tissue sarcoma. In order to plan treatment, it is important to know the type of soft tissue sarcoma, whether the tumor can be removed by surgery, and whether cancer has spread to other parts of the body. The following procedures may be used to find out if cancer has spread: - Sentinel lymph node biopsy: A sentinel lymph node biopsy is done to check if cancer has spread to the lymph nodes. The sentinel lymph node is the first lymph node to receive lymphatic drainage from a tumor. It is the first lymph node the cancer is likely to spread to from the tumor. A small amount of a radioactive substance and/or blue dye is injected near the tumor. The radioactive substance or dye flows through the lymph ducts to the lymph nodes. The first lymph node to receive the substance or dye is removed. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are not found, it may not be necessary to remove more lymph nodes. This procedure is used for epithelioid and clear cell sarcoma. - PET scan: A PET scan is a procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. This procedure is also called positron emission tomography (PET) scan. - PET-CT scan: A procedure that combines the pictures from a PET scan and a computed tomography (CT) scan. The PET and CT scans are done at the same time on the same machine. The pictures from both scans are combined to make a more detailed picture than either test would make by itself. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if soft tissue sarcoma spreads to the lung, the cancer cells in the lung are soft tissue sarcoma cells. The disease is metastatic soft tissue sarcoma, not lung cancer.",Childhood Soft Tissue Sarcoma,0000003_5,CancerGov,https://www.cancer.gov/types/soft-tissue-sarcoma/patient/child-soft-tissue-treatment-pdq,C0220645,T191,Disorders what research (or clinical trials) is being done for Childhood Soft Tissue Sarcoma ?,0000003_5-7,research,"New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Gene therapy Gene therapy is being studied for childhood synovial sarcoma that has recurred, spread, or cannot be removed by surgery. Some of the patient's T cells (a type of white blood cell) are removed and the genes in the cells are changed in a laboratory (genetically engineered) so that they will attack specific cancer cells. They are then given back to the patient by infusion. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Childhood Soft Tissue Sarcoma,0000003_5,CancerGov,https://www.cancer.gov/types/soft-tissue-sarcoma/patient/child-soft-tissue-treatment-pdq,C0220645,T191,Disorders What are the treatments for Childhood Soft Tissue Sarcoma ?,0000003_5-8,treatment,"Key Points - There are different types of treatment for patients with childhood soft tissue sarcoma. - Children with childhood soft tissue sarcoma should have their treatment planned by a team of health care providers who are experts in treating cancer in children. - Treatment for childhood soft tissue sarcoma may cause side effects. - Eight types of standard treatment are used: - Surgery - Radiation therapy - Chemotherapy - Observation - Hormone therapy - Nonsteroidal anti-inflammatory drugs - Targeted therapy - Immunotherapy - New types of treatment are being tested in clinical trials. - Gene therapy - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with childhood soft tissue sarcoma. Different types of treatments are available for patients with childhood soft tissue sarcoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment. Children with childhood soft tissue sarcoma should have their treatment planned by a team of health care providers who are experts in treating cancer in children. Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other health care providers who are experts in treating children with soft tissue sarcoma and who specialize in certain areas of medicine. These may include a pediatric surgeon with special training in the removal of soft tissue sarcomas. The following specialists may also be included: - Pediatrician. - Radiation oncologist. - Pediatric hematologist. - Pediatric nurse specialist. - Rehabilitation specialist. - Psychologist. - Social worker. - Child-life specialist. Treatment for childhood soft tissue sarcoma may cause side effects. For information about side effects that begin during treatment for cancer, see our Side Effects page. Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include: - Physical problems. - Changes in mood, feelings, thinking, learning, or memory. - Second cancers (new types of cancer). Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information.) Eight types of standard treatment are used: Surgery Surgery to completely remove the soft tissue sarcoma is done when possible. If the tumor is very large, radiation therapy or chemotherapy may be given first, to make the tumor smaller and decrease the amount of tissue that needs to be removed during surgery. This is called neoadjuvant therapy. The following types of surgery may be used: - Wide local excision: Removal of the tumor along with some normal tissue around it. - Amputation: Surgery to remove all or part of the limb or appendage with cancer, such as the arm or hand. - Lymphadenectomy: Removal of the lymph nodes with cancer. - Mohs surgery: A surgical procedure used to treat cancer in the skin. Individual layers of cancer tissue are removed and checked under a microscope one at a time until all cancer tissue has been removed. This type of surgery is used to treat dermatofibrosarcoma protuberans. It is also called Mohs micrographic surgery. - Hepatectomy: Surgery to remove all or part of the liver. A second surgery may be needed to: - Remove any remaining cancer cells. - Check the area around where the tumor was removed for cancer cells and then remove more tissue if needed. If cancer is in the liver, a hepatectomy and liver transplant may be done (the liver is removed and replaced with a healthy one from a donor). Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given radiation therapy or chemotherapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. Certain ways of giving radiation therapy can help keep radiation from damaging nearby healthy tissue. This type of radiation therapy may include the following: - Stereotactic body radiation therapy: Stereotactic body radiation therapy is a type of external radiation therapy. Special equipment is used to place the patient in the same position for each radiation treatment. Once a day for several days, a radiation machine aims a larger than usual dose of radiation directly at the tumor. By having the patient in the same position for each treatment, there is less damage to nearby healthy tissue. This procedure is also called stereotactic external-beam radiation therapy and stereotaxic radiation therapy. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated and whether the tumor was completely removed by surgery. External and internal radiation therapy are used to treat childhood soft tissue sarcoma. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is the use of more than one anticancer drug. The way the chemotherapy is given depends on the type of cancer being treated. Most types of soft tissue sarcoma do not respond to treatment with chemotherapy. See Drugs Approved for Soft Tissue Sarcoma for more information. Observation Observation is closely monitoring a patients condition without giving any treatment until signs or symptoms appear or change. Observation may be done when: - Complete removal of the tumor is not possible. - No other treatments are available. - The tumor is not likely to damage any vital organs. Hormone therapy Hormone therapy is a cancer treatment that removes hormones or blocks their action and stops cancer cells from growing. Hormones are substances made by glands in the body and circulated in the bloodstream. Some hormones can cause certain cancers to grow. If tests show that the cancer cells have places where hormones can attach (receptors), drugs, surgery, or radiation therapy is used to reduce the production of hormones or block them from working. Antiestrogens (drugs that block estrogen), such as tamoxifen, may be used to treat desmoid-type fibromatosis. Nonsteroidal anti-inflammatory drugs Nonsteroidal anti-inflammatory drugs (NSAIDs) are drugs (such as aspirin, ibuprofen, and naproxen) that are commonly used to decrease fever, swelling, pain, and redness. In the treatment of desmoid-type fibromatosis, an NSAID called sulindac may be used to help block the growth of cancer cells. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to attack cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation do. Kinase inhibitors are a type of targeted therapy that block an enzyme called kinase (a type of protein). There are different types of kinases in the body that have different actions. - ALK inhibitors may stop the cancer from growing and spreading: - Crizotinib may be used to treat inflammatory myofibroblastic tumor. - Tyrosine kinase inhibitors (TKIs) block signals needed for tumors to grow: - Imatinib is used to treat dermatofibrosarcoma protuberans. - Pazopanib may be used to treat recurrent and progressive soft tissue sarcoma. It is being studied for many types of newly diagnosed soft tissue sarcoma. - Sorafenib may be used to treat desmoid-type fibromatosis. New types of tyrosine kinase inhibitors are being studied such as LOXO-101 and entrectinib for infantile fibrosarcoma. Other types of targeted therapy are being studied in clinical trials, including the following: - mTOR inhibitors are a type of targeted therapy that stops the protein that helps cells divide and survive. mTOR inhibitors are being studied to treat perivascular epithelioid cell tumors (PEComas) and epithelioid hemangioendothelioma. Sirolimus is a type of mTOR inhibitor therapy. - Angiogenesis inhibitors are a type of targeted therapy that prevent the growth of new blood vessels needed for tumors to grow. Angiogenesis inhibitors, such as cediranib, sunitinib, and thalidomide are being studied to treat alveolar soft part sarcoma and epithelioid hemangioendothelioma. Bevacizumab is being studied for blood vessel tumors. - Histone methyltransferase (HMT) inhibitors are targeted therapy drugs that work inside cancer cells and block signals needed for tumors to grow. HMT inhibitors are being studied for the treatment of epithelioid sarcoma, malignant peripheral nerve sheath tumor, extrarenal (extracranial) rhabdoid tumor, extraskeletal myxoid chondrosarcoma, and synovial sarcoma. - Heat-shock protein inhibitors block certain proteins that protect tumor cells and help them grow. Ganetespib is a heat shock protein inhibitor being studied in combination with the mTOR inhibitor sirolimus for malignant peripheral nerve sheath tumors that cannot be removed by surgery. - Antibody-drug conjugates are made up of a monoclonal antibody attached to a drug. The monoclonal antibody binds to specific proteins or receptors found on certain cells, including cancer cells. The drug enters these cells and kills them without harming other cells. Lorvotuzumab mertansine is an antibody-drug conjugate being studied for the treatment of rhabdomyosarcoma, malignant peripheral nerve sheath tumor, and synovial sarcoma. See Drugs Approved for Soft Tissue Sarcoma for more information. Immunotherapy Immunotherapy is a treatment that uses the patients immune system to fight disease. Substances made by the body or made in a laboratory are used to boost, direct, or restore the bodys natural defenses against disease. Interferon is a type of immunotherapy used to treat epithelioid hemangioendothelioma. It interferes with the division of tumor cells and can slow tumor growth. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Childhood Soft Tissue Sarcoma Newly Diagnosed Childhood Soft Tissue Sarcoma Fat Tissue Tumors Liposarcoma Treatment of liposarcoma may include the following: - Surgery to completely remove the tumor. If the cancer is not completely removed, a second surgery may be done. - Chemotherapy to shrink the tumor, followed by surgery. - Radiation therapy before or after surgery. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. Bone and Cartilage Tumors Extraskeletal mesenchymal chondrosarcoma Treatment of extraskeletal mesenchymal chondrosarcoma may include the following: - Surgery to completely remove the tumor. Radiation therapy may be given before and/or after surgery. - Chemotherapy followed by surgery. Chemotherapy with or without radiation therapy is given after surgery. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. Extraskeletal osteosarcoma Treatment of extraskeletal osteosarcoma may include the following: - Surgery to completely remove the tumor, followed by chemotherapy. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. See the PDQ summary on Osteosarcoma and Malignant Fibrous Histiocytoma of Bone Treatment for more information. Fibrous (Connective) Tissue Tumors Desmoid-type fibromatosis Treatment of desmoid-type fibromatosis may include the following: - Surgery to completely remove the tumor. Treatment before surgery may include the following: - Observation. - Chemotherapy. - Radiation therapy. - Antiestrogen drug therapy. - Nonsteroidal anti-inflammatory drug (NSAID) therapy. If the tumor is not completely removed by surgery, treatment may include the following: - Observation, if other treatment options are not possible. - Radiation therapy. - Radiation therapy or chemotherapy for tumors that cannot be removed by surgery. - A clinical trial of targeted therapy. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. Treatment of desmoid-type fibromatosis that has come back may include the following: - Observation and possibly surgery at a later time. - Chemotherapy. Dermatofibrosarcoma protuberans Treatment of dermatofibrosarcoma protuberans may include the following: - Surgery to completely remove the tumor when possible. This may include Mohs surgery. - Radiation therapy before or after surgery. - Targeted therapy (imatinib) if the tumor cannot be removed or has come back. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. Fibrosarcoma Infantile fibrosarcoma Treatment of infantile fibrosarcoma may include the following: - Surgery to remove the tumor when possible, followed by observation. - Surgery followed by chemotherapy. - Chemotherapy to shrink the tumor, followed by surgery. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. - A clinical trial of targeted therapy (tyrosine kinase inhibitor). Adult-type fibrosarcoma Treatment of adult-type fibrosarcoma may include the following: - Surgery to completely remove the tumor when possible. Inflammatory myofibroblastic tumor Treatment of inflammatory myofibroblastic tumor may include the following: - Surgery to completely remove the tumor when possible. - Chemotherapy. - Steroid therapy. - Nonsteroidal anti-inflammatory drug (NSAID) therapy. - Targeted therapy (ALK inhibitors). - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. Low-grade fibromyxoid sarcoma Treatment of low-grade fibromyxoid sarcoma may include the following: - Surgery to completely remove the tumor. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. Myxofibrosarcoma Treatment of myxofibrosarcoma may include the following: - Surgery to completely remove the tumor. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. Sclerosing epithelioid fibrosarcoma Treatment of sclerosing epithelioid fibrosarcoma may include the following: - Surgery to completely remove the tumor. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. Skeletal Muscle Tumors Rhabdomyosarcoma See the PDQ summary on Childhood Rhabdomyosarcoma Treatment. Smooth Muscle Tumors Leiomyosarcoma Treatment of leiomyosarcoma may include the following: - Chemotherapy. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. So-called Fibrohistiocytic Tumors Plexiform fibrohistiocytic tumor Treatment of plexiform fibrohistiocytic tumor may include the following: - Surgery to completely remove the tumor. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. Peripheral Nervous System Tumors Ectomesenchymoma Treatment of ectomesenchymoma may include the following: - Surgery and chemotherapy. - Radiation therapy. Malignant peripheral nerve sheath tumor Treatment of malignant peripheral nerve sheath tumor may include the following: - Surgery to completely remove the tumor when possible. - Radiation therapy before or after surgery. - Chemotherapy, for tumors that cannot be removed by surgery. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. - A clinical trial of targeted therapy, for tumors that cannot be removed by surgery. - A clinical trial of targeted therapy (histone methyltransferase inhibitor). - A clinical trial of an antibody-drug conjugate. It is not clear whether giving radiation therapy or chemotherapy after surgery improves the tumor's response to treatment. Malignant triton tumor Malignant triton tumors may be treated the same as rhabdomyosarcomas and include surgery, chemotherapy, or radiation therapy. A regimen of targeted therapy, radiation therapy, and surgery with or without chemotherapy is being studied. Pericytic (Perivascular) Tumors Infantile hemangiopericytoma Treatment of infantile hemangiopericytoma may include the following: - Chemotherapy. Infantile myofibromatosis Treatment of infantile myofibromatosis may include the following: - Combination chemotherapy. Tumors of Unknown Origin (the place where the tumor first formed is not known) Alveolar soft part sarcoma Treatment of alveolar soft part sarcoma may include the following: - Surgery to completely remove the tumor when possible. - Radiation therapy before or after surgery, if the tumor cannot be completely removed by surgery. - Targeted therapy (angiogenesis inhibitor). - A clinical trial of targeted therapy (angiogenesis inhibitor) for children. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. Clear cell sarcoma of soft tissue Treatment of clear cell sarcoma of soft tissue may include the following: - Surgery to remove the tumor when possible. - Radiation therapy before or after surgery. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. Desmoplastic small round cell tumor There is no standard treatment for desmoplastic small round cell tumor. Treatment may include the following: - Surgery to completely remove the tumor when possible. - Chemotherapy followed by surgery. - Radiation therapy. Epithelioid sarcoma Treatment of epithelioid sarcoma may include the following: - Surgery to remove the tumor when possible. - Chemotherapy before or after surgery. - Radiation therapy before or after surgery. - A clinical trial of targeted therapy (histone methyltransferase inhibitor). Extrarenal (extracranial) rhabdoid tumor Treatment of extrarenal (extracranial) rhabdoid tumor may include the following: - A combination of surgery to remove the tumor when possible, chemotherapy, and radiation therapy. - A clinical trial of targeted therapy (histone methyltransferase inhibitor). Extraskeletal myxoid chondrosarcoma Treatment of extraskeletal myxoid chondrosarcoma may include the following: - Surgery to remove the tumor when possible. - Radiation therapy. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. - A clinical trial of targeted therapy (histone methyltransferase inhibitor). Perivascular epithelioid cell tumors (PEComas) Treatment of perivascular epithelioid cell tumors may include the following: - Surgery to remove the tumor. - Observation followed by surgery. - Targeted therapy (mTOR inhibitor), for tumors that have certain gene changes and cannot be removed by surgery. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. Primitive neuroectodermal tumor (PNET)/extraskeletal Ewing tumor See the PDQ summary on Ewing Sarcoma Treatment. Synovial sarcoma Treatment of synovial sarcoma may include the following: - Chemotherapy. - Surgery. Radiation therapy and/or chemotherapy may be given before or after surgery. - A clinical trial of gene therapy. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. - A clinical trial of targeted therapy (histone methyltransferase inhibitor). - A clinical trial of an antibody-drug conjugate. Undifferentiated/unclassified sarcoma These tumors include undifferentiated pleomorphic sarcoma /malignant fibrous histiocytoma (high-grade). There is no standard treatment for these tumors. Treatment may include the following: - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. See the PDQ summary on Osteosarcoma and Malignant Fibrous Histiocytoma of Bone Treatment for information about the treatment of malignant fibrous histiocytoma of bone. Blood Vessel Tumors Angiosarcoma of soft tissue Treatment of angiosarcoma may include the following: - Surgery to completely remove the tumor. - A combination of surgery, chemotherapy, and radiation therapy for angiosarcomas that have spread. - Targeted therapy (bevacizumab) and chemotherapy for angiosarcomas that began as infantile hemangiomas. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy. Epithelioid hemangioendothelioma Treatment of epithelioid hemangioendothelioma may include the following: - Surgery to remove the tumor when possible. - Immunotherapy (interferon) and targeted therapy (thalidomide, sorafenib, pazopanib, sirolimus) for tumors that are likely to spread. - Chemotherapy. - Total hepatectomy and liver transplant when the tumor is in the liver. Metastatic Childhood Soft Tissue Sarcoma Treatment of childhood soft tissue sarcoma that has spread to other parts of the body at diagnosis may include the following: - Chemotherapy and radiation therapy. Surgery may be done to remove tumors that have spread to the lung. - Stereotactic body radiation therapy for tumors that have spread to the lung. For treatment of specific tumor types, see the Treatment Options for Childhood Soft Tissue Sarcoma section. Check the list of NCI-supported cancer clinical trials that are now accepting patients with nonmetastatic childhood soft tissue sarcoma and metastatic childhood soft tissue sarcoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Recurrent and Progressive Childhood Soft Tissue Sarcoma Treatment of recurrent or progressive childhood soft tissue sarcoma may include the following: - Surgery to remove cancer that has come back where it first formed or that has spread to the lung. - Surgery followed by external or internal radiation therapy, if radiation therapy has not already been given. - Surgery to remove the arm or leg with cancer, if radiation therapy was already given. - Chemotherapy. - Targeted therapy (tyrosine kinase inhibitor). - A clinical trial of a new chemotherapy regimen. Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent childhood soft tissue sarcoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website.",Childhood Soft Tissue Sarcoma,0000003_5,CancerGov,https://www.cancer.gov/types/soft-tissue-sarcoma/patient/child-soft-tissue-treatment-pdq,C0220645,T191,Disorders What is (are) Childhood Vascular Tumors ?,0000003_6-1,information,"Key Points - Childhood vascular tumors form from cells that make blood vessels or lymph vessels. - Tests are used to detect (find) and diagnose childhood vascular tumors. - Childhood vascular tumors may be classified into four groups. - Benign tumors - Intermediate (locally aggressive) tumors - Intermediate (rarely metastasizing) tumors - Malignant tumors Childhood vascular tumors form from cells that make blood vessels or lymph vessels. Vascular tumors can form from abnormal blood vessel or lymph vessel cells anywhere in the body. They may be benign (not cancer) or malignant (cancer). There are many types of vascular tumors. The most common type of childhood vascular tumor is hemangioma, which is a benign tumor that usually goes away on its own. Because malignant vascular tumors are rare in children, there is not a lot of information about what treatment works best. Childhood vascular tumors may be classified into four groups. Benign tumors Benign tumors are not cancer. This summary has information about the following benign vascular tumors: - Infantile hemangioma. - Congenital hemangioma. - Benign vascular tumors of the liver. - Spindle cell hemangioma. - Epithelioid hemangioma. - Pyogenic granuloma (lobular capillary hemangioma). - Angiofibroma. - Juvenile nasopharyngeal angiofibroma. Intermediate (locally aggressive) tumors Intermediate tumors that are locally aggressive often spread to the area around the tumor. This summary has information about the following locally aggressive vascular tumors: - Kaposiform hemangioendothelioma and tufted angioma. Intermediate (rarely metastasizing) tumors Intermediate (rarely metastasizing) tumors sometimes spread to other parts of the body. This summary has information about the following vascular tumors that rarely metastasize: - Retiform hemangioendothelioma. - Papillary intralymphatic angioendothelioma. - Composite hemangioendothelioma. - Kaposi sarcoma. Malignant tumors Malignant tumors are cancer. This summary has information about the following malignant vascular tumors: - Epithelioid hemangioendothelioma. - Angiosarcoma of soft tissue. Benign Tumors Infantile Hemangioma Infantile hemangiomas are the most common type of benign vascular tumor in children. An infantile hemangioma may also be called a ""strawberry mark."" Immature cells that are meant to form blood vessels form a tumor instead. These tumors are not usually seen at birth but appear when the infant is 3 to 6 weeks old. Most hemangiomas get bigger for about 5 months, then stop growing and slowly fade away completely during the next several years. It is rare for them to come back. Hemangiomas may be on the skin, in the tissue below the skin, and/or in an organ. They are usually on the head and neck but can be anywhere on or in the body. Hemangiomas may appear as a single lesion, one or more lesions spread over a larger area of the body, or multiple lesions in more than one part of the body. Lesions that are spread over a larger area of the body or multiple lesions are more likely to cause problems. Risk Factors Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get the disease; not having risk factors doesnt mean that you will not get the disease. Talk with your child's doctor if you think your child may be at risk. Infantile hemangiomas are more common in the following: - Girls. - Whites. - Premature babies. - Twins, triplets, or other multiple births. - Babies of mothers who are older at time of the pregnancy or who have problems with the placenta during pregnancy. Other risk factors for infantile hemangiomas include the following: - Having certain syndromes. - PHACE syndrome: A syndrome in which the hemangioma spreads across a large area of the body (usually the head or face). Other health problems involving the large blood vessels, heart, eyes, and/or brain may also occur. - LUMBAR/PELVIS/SACRAL syndrome: A syndrome in which the hemangioma spreads across a large area of the lower back. Other health problems that affect the urinary system, genitals, rectum, anus, brain, spinal cord, and nerve function may also occur. Having more than one hemangioma or an airway hemangioma increases the risk of having other health problems. - Multiple hemangiomas: Having more than five hemangiomas on the skin is a sign that there may be hemangiomas in an organ, most commonly the liver. Heart, muscle, and thyroid gland problems can also occur. - Airway hemangiomas: Hemangiomas in the airway usually occur along with a large, beard-shaped area of hemangioma on the face (from the ears, around the mouth, lower chin, and front of neck). It is important for airway hemangiomas to be treated before the child has trouble breathing. Signs and Symptoms Infantile hemangiomas may cause any of the following signs and symptoms. Check with your childs doctor if your child has any of the following: - Skin lesions: An area of spidery veins or lightened or discolored skin may appear before the hemangioma does. Hemangiomas occur as firm, warm, bright red-blue lesions on the skin. Lesions that form ulcers are also painful. Later, as the hemangiomas go away, they begin fading in the center before flattening and losing color. - Lesions below the skin: Lesions that grow under the skin in the fat may appear blue or purple. If the lesions are deep enough under the skin surface, they may not be seen. - Lesions in an organ: There may be no signs that hemangiomas have formed on an organ. Although most infantile hemangiomas are nothing to worry about, if your child develops any lumps or red or blue marks on the skin check with your child's doctor. He or she can recommend a specialist if needed. Diagnostic Tests A physical exam and history are usually all that are needed to diagnose infantile hemangiomas. If there is something about the tumor that looks unusual, a biopsy may be done. If the hemangioma is deeper inside the body with no change to the skin, or the lesions are spread across a large area of the body, an ultrasound or MRI may be done. See the General Information section for a description of these tests and procedures. If the hemangiomas are part of a syndrome, more tests may be done such as an echocardiogram, magnetic resonance angiogram, and eye exam. Treatment Most hemangiomas fade and shrink without treatment. If the hemangioma is large or causing other health problems, treatment may include the following: - Propranolol or other beta-blocker therapy. - Steroid therapy, before beta-blocker therapy is begun or when beta-blockers cannot be used. - Pulsed dye laser surgery, for hemangiomas that have ulcers or have not gone away. - Surgery (excision) for hemangiomas that have ulcers, cause vision problems, or have not gone away. - Topical beta-blocker therapy for hemangiomas that are in one area of the skin. - Combined therapy, such as propranolol and steroid therapy or propranolol and topical beta-blocker therapy. Congenital Hemangioma Congenital hemangioma is a benign vascular tumor that begins forming before birth and is fully formed when the baby is born. They're usually on the skin but can be in another organ. There are three types of congenital hemangiomas: - Rapidly Involuting Congenital Hemangioma: These tumors go away on their own 12 to 15 months after birth. They can form ulcers, bleed, and cause temporary heart and blood clotting problems. The skin may look a little different even after the hemangiomas go away. - Partial Involuting Congenital Hemangioma: These tumors do not go away completely. - Non-Involuting Congenital Hemangioma: These tumors never go away on their own. Diagnostic Tests See the General Information section for a description of tests and procedures used to diagnose congenital hemangioma. Treatment Treatment of rapidly involuting congenital hemangioma and partial involuting congenital hemangioma may include the following: - Observation only. Treatment of non-involuting congenital hemangioma may include the following: - Surgery to remove the tumor depending on where it is and whether it is causing symptoms. Benign Vascular Tumors of the Liver Benign vascular tumors of the liver may be focal (a single lesion in one area of the liver), multifocal (multiple lesions in one area of the liver), or diffuse (multiple lesions in more than one area of the liver). The liver has many functions, including filtering blood and making proteins needed for blood clotting. Sometimes, blood that normally flows through the liver is blocked or slowed by the tumor. This sends blood directly to the heart without going through the liver and is called a liver shunt. This can cause heart failure and problems with blood clotting. Focal Tumors Focal tumors are usually rapidly involuting congenital hemangiomas or non-involuting congenital hemangiomas. Diagnostic Tests See the General Information section for a description of tests and procedures used to diagnose focal benign vascular tumors. Treatment Treatment of focal tumors of the liver depends on whether symptoms occur and may include the following: - Observation. - Drugs to manage symptoms, including heart failure and blood clotting problems. - Embolization of the liver to manage symptoms, including heart failure. Multifocal and Diffuse Tumors Multifocal and diffuse tumors of the liver are usually infantile hemangiomas. Diffuse tumors of the liver can cause serious effects, including problems with the thyroid gland and heart. The liver can enlarge, press on other organs, and cause more symptoms. Diagnostic Tests See the General Information section for a description of tests and procedures used to diagnose multifocal or diffuse benign vascular tumors. Treatment Treatment of multifocal and diffuse liver tumors may include the following: - Observation for multifocal tumors of the liver that do not cause symptoms. - Beta-blocker therapy (propranolol). - Chemotherapy. - Steroid therapy. - Total hepatectomy and liver transplant, when the tumors do not respond to drug therapy. This is only done when the tumors have spread widely in the liver and more than one organ has failed. If a vascular tumor of the liver does not respond to standard treatments, a biopsy may be done to see if the tumor has become malignant. Spindle Cell Hemangioma Spindle cell hemangiomas contain cells called spindle cells. Under a microscope, spindle cells look long and slender. Risk Factors Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get the disease; not having risk factors doesnt mean that you will not get the disease. Talk with your child's doctor if you think your child may be at risk. Spindle cell hemangiomas are likely to occur in children with the following syndromes: - Maffucci syndrome, which affects cartilage and skin. - Klippel-Trenaunay syndrome, which affects blood vessels, soft tissues, and bones. Signs Spindle cell hemangiomas appear on or under the skin. They are painful red-brown or bluish lesions that usually appear on the arms or legs. They can begin as one lesion and develop into more lesions over years. Diagnostic Tests See the General Information section for a description of tests and procedures used to diagnose spindle cell hemangioma. Treatment There is no standard treatment for spindle cell hemangiomas. Treatment may include the following: - Surgery to remove the tumor. Spindle cell hemangiomas may come back after surgery. Epithelioid Hemangioma Epithelioid hemangiomas usually form on or in the skin, especially the head, but can occur in other areas, such as bone. Signs and Symptoms Epithelioid hemangiomas are sometimes caused by injury. On the skin, they may appear as firm pink to red bumps and may be itchy. Epithelioid hemangioma of the bone may cause swelling, pain, and weakened bone in the affected area. Diagnostic Tests See the General Information section for a description of tests and procedures used to diagnose epithelioid hemangioma. Treatment There is no standard treatment for epithelioid hemangiomas. Treatment may include the following: - Surgery (curettage or resection). - Sclerotherapy. - Radiation therapy in rare cases. Epithelioid hemangiomas often come back after treatment. Pyogenic Granuloma Pyogenic granuloma is also called lobular capillary hemangioma. It is most common in older children and young adults but may occur at any age. The lesions are sometimes caused by injury or from the use of certain medicines, including birth control pills and retinoids. They may also form for no known reason inside capillaries (the smallest blood vessels) or other places on the body. Usually there is only one lesion, but sometimes multiple lesions occur in the same area or the lesions may spread to other areas of the body. Signs Pyogenic granulomas are raised, bright red lesions that may be small or large and smooth or bumpy. They grow quickly over weeks to months and may bleed a lot. Diagnostic Tests See the General Information section for a description of tests and procedures used to diagnose pyogenic granuloma. Treatment Some pyogenic granulomas go away without treatment. Other pyogenic granulomas need treatment that may include the following: - Surgery (excision or curettage) to remove the lesion. - Laser photocoagulation. Pyogenic granulomas often come back after treatment. Angiofibroma Angiofibromas are rare. They are benign skin lesions that usually occur with a condition called tuberous sclerosis (an inherited disorder that causes skin lesions, seizures, and mental disabilities). Signs Angiofibromas appear as red bumps on the face. Diagnostic Tests See the General Information section for a description of tests and procedures used to diagnose angiofibroma. Treatment Treatment of angiofibromas may include the following: - Surgery (excision) to remove the tumor. - Laser therapy. - Targeted therapy (sirolimus). Juvenile Nasopharyngeal Angiofibroma Juvenile nasopharyngeal angiofibromas are benign tumors but they can invade nearby tissue. They begin in the nasal cavity and may spread to the nasopharynx, the paranasal sinuses, the bone around the eyes, and sometimes to the brain. Diagnostic Tests See the General Information section for a description of tests and procedures used to diagnose juvenile nasopharyngeal angiofibroma. Treatment Treatment of juvenile nasopharyngeal angiofibromas may include the following: - Surgery (excision) to remove the tumor. - Radiation therapy. - Chemotherapy. - Immunotherapy (interferon). - Targeted therapy (sirolimus). Intermediate Tumors that Spread Locally Kaposiform Hemangioendothelioma and Tufted Angioma Kaposiform hemangioendotheliomas and tufted angiomas are blood vessel tumors that occur in infants or during early childhood. These tumors can cause Kasabach-Merritt phenomenon, a condition in which the blood is not able to clot and serious bleeding may occur. In Kasabach-Merritt phenomenon the tumor traps and destroys platelets (blood-clotting cells). Then there aren't enough platelets in the blood when needed to stop bleeding. This type of vascular tumor is not related to Kaposi sarcoma. Signs and Symptoms Kaposiform hemangioendotheliomas and tufted angiomas usually occur on the skin of the arms and legs, but may also form in deeper tissues, such as muscle or bone. Signs and symptoms may include the following: - Firm, painful areas of skin that look bruised. - Purple or brownish-red areas of skin. - Easy bruising. - Bleeding more than the usual amount from mucous membranes, wounds, and other tissues. - Anemia (weakness, feeling tired, or looking pale). Diagnostic Tests See the General Information section for a description of tests and procedures used to diagnose kaposiform hemangioendothelioma. If a physical exam and MRI clearly show the tumor is a kaposiform hemangioendothelioma or a tufted angioma, a biopsy may not be needed. A biopsy is not always done because serious bleeding can occur. Treatment Treatment of kaposiform hemangioendotheliomas and tufted angiomas depends on the child's symptoms. Infection, delay in treatment, and surgery can cause bleeding that is life-threatening. Kaposiform hemangioendotheliomas and tufted angiomas are best treated by a vascular anomaly specialist. Treatment and supportive care to manage bleeding may include the following: - Steroid therapy which may be followed by chemotherapy. - Non-steroidal anti-inflammatory drugs (NSAID), such as aspirin. - Immunotherapy (interferon). - Antifibrinolytic therapy to improve blood clotting. - Chemotherapy with one or more anticancer drugs. - Beta-blocker therapy (propranolol). - Surgery (excision) to remove the tumor, with or without embolization. - Targeted therapy (sirolimus). - A clinical trial of targeted therapy (sirolimus) and steroid therapy. Even with treatment, these tumors do not fully go away and can come back. Long-term effects include chronic pain, heart failure, bone problems, and lymphedema (the build up of lymph fluid in tissues). Intermediate Tumors that Rarely Spread Retiform Hemangioendothelioma Retiform hemangioendotheliomas are slow growing, flat tumors that occur in young adults and sometimes children. These tumors usually occur on or under the skin of the arms, legs, and trunk. These tumors often come back after treatment, but they usually do not spread to other parts of the body. Diagnostic Tests See the General Information section for a description of tests and procedures used to diagnose retiform hemangioendothelioma. Treatment Treatment of retiform hemangioendotheliomas may include the following: - Surgery (excision) to remove the tumor. Follow up will include monitoring to see if the tumor comes back. - Radiation therapy and chemotherapy when surgery cannot be done or when the tumor has come back. Papillary Intralymphatic Angioendothelioma Papillary intralymphatic angioendotheliomas are also called Dabska tumors. These tumors form in or under the skin anywhere on the body. The tumors contain channels that look like lymph vessels. Lymph nodes are sometimes affected. Signs Papillary intralymphatic angioendotheliomas may appear as firm, raised, purplish bumps, which may be small or large. These tumors grow slowly over time. Diagnostic Tests See the General Information section for a description of tests and procedures used to diagnose papillary intralymphatic angioendothelioma. Treatment Treatment of papillary intralymphatic angioendotheliomas may include the following: - Surgery (excision) to remove the tumor. Composite Hemangioendothelioma Composite hemangioendotheliomas have features of both benign and malignant vascular tumors. These tumors usually occur on or under the skin on the arms or legs. They may also occur on the head, neck, or chest. Composite hemangioendotheliomas are not likely to metastasize (spread) but they may come back in the same place. When the tumors metastasize, they usually spread to nearby lymph nodes. Diagnostic Tests See the General Information section for a description of tests and procedures used to diagnose composite hemangioendothelioma and find out whether the tumor has spread. Treatment Treatment of composite hemangioendotheliomas may include the following: - Surgery to remove the tumor. - Radiation therapy and chemotherapy for tumors that have spread. Kaposi Sarcoma Kaposi sarcoma is a cancer that causes lesions to grow in the skin; the mucous membranes lining the mouth, nose, and throat; lymph nodes; or other organs. It is caused by the Kaposi sarcoma herpes virus (KSHV). In the United States, it usually occurs in people who have a weak immune system caused by AIDS or by drugs used in organ transplants. It is very rare in children and can be caused by HIV infection and rare immune system disorders. Signs Signs in children may include the following: - Lesions in the skin, mouth, or throat. Skin lesions are red, purple, or brown and change from flat, to raised, to scaly areas called plaques, to nodules. - Swollen lymph nodes. Diagnostic Tests See the General Information section for a description of tests and procedures used to diagnose Kaposi sarcoma. Treatment Treatment of Kaposi sarcoma may include the following: - Chemotherapy. See the PDQ summary on Kaposi Sarcoma Treatment for information about Kaposi sarcoma in adults. Malignant Tumors Epithelioid Hemangioendothelioma Epithelioid hemangioendotheliomas can occur in children, but are most common in adults between 30 and 50 years of age. They usually occur in the liver, lung, or in bone. They may be either fast growing or slow growing. In about a third of cases, the tumor spreads to other parts of the body very quickly. Signs and Symptoms Signs and symptoms depend on where the tumor is: - On the skin, the tumors can be raised and rounded or flat, red-brown patches that feel warm. - In the lung, there may be no early symptoms. Signs and symptoms that occur may include: - Chest pain. - Spitting up blood. - Anemia (weakness, feeling tired, or looking pale). - Trouble breathing (from scarred lung tissue). - In bone, the tumors can cause breaks. Diagnostic Tests Epithelioid hemangioendotheliomas in the liver are found with CT scans and MRI scans. See the General Information section for a description of these tests and procedures used to diagnose epithelioid hemangioendothelioma and find out whether the tumor has spread. X-rays may also be done. Treatment Treatment of slow-growing epithelioid hemangioendotheliomas includes the following: - Observation. Treatment of fast-growing epithelioid hemangioendotheliomas may include the following: - Surgery to remove the tumor when possible. - Immunotherapy (interferon) and targeted therapy (thalidomide, sorafenib, pazopanib, sirolimus) for tumors that are likely to spread. - Chemotherapy. - Total hepatectomy and liver transplant when the tumor is in the liver. Angiosarcoma of the Soft Tissue Angiosarcomas are fast-growing tumors that form in blood vessels or lymph vessels in any part of the body, usually in soft tissue. Most angiosarcomas are in or near the skin. Those in deeper soft tissue can form in the liver, spleen, and lung. These tumors are very rare in children. Children sometimes have more than one tumor in the skin and/or liver. Risk Factors Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get the disease; not having risk factors doesnt mean that you will not get the disease. Talk with your child's doctor if you think your child may be at risk. Risk factors for angiosarcomas include the following: - Being exposed to radiation. - Chronic (long-term) lymphedema, a condition in which extra lymph fluid builds up in tissues and causes swelling. - Having a benign vascular tumor. A benign tumor, such as a hemangioma, may become an angiosarcoma but this rare. Signs Signs of angiosarcoma depend on where the tumor is and may include the following: - Red patches on the skin that bleed easily. - Purple tumors. Diagnostic Tests See the General Information section for a description of tests and procedures used to diagnose angiosarcoma and find out whether the tumor has spread. Treatment Treatment of angiosarcoma may include the following: - Surgery to completely remove the tumor. - A combination of surgery, chemotherapy, and radiation therapy for angiosarcomas that have spread. - Targeted therapy (bevacizumab) and chemotherapy for angiosarcomas that began as infantile hemangiomas. - A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy.",Childhood Vascular Tumors,0000003_6,CancerGov,https://www.cancer.gov/types/soft-tissue-sarcoma/patient/child-vascular-tumors-treatment-pdq,C0282607,T191,Disorders How to diagnose Childhood Vascular Tumors ?,0000003_6-2,exams and tests,"Tests are used to detect (find) and diagnose childhood vascular tumors. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps, lesions, or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. A biopsy is not always needed to diagnose a vascular tumor.",Childhood Vascular Tumors,0000003_6,CancerGov,https://www.cancer.gov/types/soft-tissue-sarcoma/patient/child-vascular-tumors-treatment-pdq,C0282607,T191,Disorders What are the treatments for Childhood Vascular Tumors ?,0000003_6-3,treatment,"Key Points - There are different types of treatment for childhood vascular tumors. - Children with childhood vascular tumors should have their treatment planned by a team of health care providers who are experts in treating cancer in children. - Some treatments cause side effects months or years after treatment has ended. - Eleven types of standard treatment are used: - Beta-blocker therapy - Surgery - Photocoagulation - Embolization - Chemotherapy - Sclerotherapy - Radiation therapy - Targeted therapy - Immunotherapy - Other drug therapy - Observation - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for childhood vascular tumors. Different types of treatment are available for children with vascular tumors. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Because vascular tumors in children are rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment. Children with childhood vascular tumors should have their treatment planned by a team of health care providers who are experts in treating cancer in children. Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other pediatric health care providers who are experts in treating children with cancer and who specialize in certain areas of medicine. These may include the following specialists: - Pediatric vascular anomaly specialist (expert in treating children with vascular tumors). - Pediatric surgeon. - Orthopedic surgeon. - Radiation oncologist. - Pediatric nurse specialist. - Rehabilitation specialist. - Psychologist. - Social worker. Some treatments cause side effects months or years after treatment has ended. Some treatments, such as chemotherapy and radiation therapy, cause side effects that continue or appear months or years after treatment has ended. These are called late effects. Late effects of treatment may include: - Physical problems. - Changes in mood, feelings, thinking, learning, or memory. - Second cancers (new types of cancer). Some late effects may be treated or controlled. It is important to talk with your child's doctors about the possible late effects caused by some treatments. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information). Eleven types of standard treatment are used: Beta-blocker therapy Beta-blockers are drugs that decrease blood pressure and heart rate. When used in patients with vascular tumors, beta-blockers may help shrink the tumors. Beta-blocker therapy may be given by vein (IV), by mouth, or placed on the skin (topical). The way the beta-blocker therapy is given depends on the type of vascular tumor and where the tumor first formed. The beta-blocker propranolol is usually the first treatment for hemangiomas. Infants treated with IV propranolol may need to have their treatment started in a hospital. Propranolol is also used to treat benign vascular tumor of liver and kaposiform hemangioendothelioma. Other beta-blockers used to treat vascular tumors include atenolol, nadolol, and timolol. Infantile hemangioma may also be treated with propranolol and steroid therapy or propranolol and topical beta-blocker therapy. See the drug information summary on Propranolol Hydrochloride for more information. Surgery The following types of surgery may be used to remove many types of vascular tumors: - Excision: Surgery to remove the entire tumor and some of the healthy tissue around it. - Laser surgery: A surgical procedure that uses a laser beam (a narrow beam of intense light) as a knife to make bloodless cuts in tissue or to remove a skin lesion such as a tumor. Surgery with a pulsed dye laser may be used for some hemangiomas. This type of laser uses a beam of light that targets blood vessels in the skin. The light is changed into heat and the blood vessels are destroyed without damaging nearby skin. - Total hepatectomy and liver transplant: A surgical procedure to remove the entire liver followed by a transplant of a healthy liver from a donor. - Curettage: A procedure in which abnormal tissue is removed using a small, spoon-shaped instrument called a curette. The type of surgery used depends on the type of vascular tumor and where the tumor formed in the body. For malignant tumors, even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the tumor will come back, is called adjuvant therapy. Photocoagulation Photocoagulation is the use of an intense beam of light, such as a laser, to seal off blood vessels or destroy tissue. It is used to treat pyogenic granuloma. Embolization Embolization is a procedure that uses particles, such as tiny gelatin sponges or beads, to block blood vessels in the liver. It may be used to treat some benign vascular tumors of the liver and kaposiform hemangioendothelioma. Chemotherapy Chemotherapy is a treatment that uses drugs to stop the growth of tumor cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach tumor cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect tumor cells in those areas (regional chemotherapy). Chemotherapy for hemangiomas may also be given topically (applied to the skin in a cream or lotion). The way the chemotherapy is given depends on the type of the vascular tumor being treated. Sclerotherapy Sclerotherapy is a treatment used to destroy the blood vessel with the tumor. A liquid is injected into the blood vessel, causing it to scar and break down. Over time, the destroyed blood vessel is absorbed into normal tissue. The blood flows through nearby healthy veins instead. Sclerotherapy is used in the treatment of epithelioid hemangioma. Radiation therapy Radiation therapy is a treatment that uses high-energy x-rays or other types of radiation to kill tumor cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the tumor. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the tumor. The way the radiation therapy is given depends on the type of the vascular tumor being treated. External radiation is used to treat some vascular tumors. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to attack specific tumor cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. Angiogenesis inhibitors are a type of targeted therapy. - Angiogenesis inhibitors are drugs that stop cells from dividing and prevent the growth of new blood vessels that tumors need to grow. The targeted therapy drugs thalidomide, sorafenib, pazopanib, sirolimus, and bevacizumab are angiogenesis inhibitors used to treat childhood vascular tumors. Immunotherapy Immunotherapy is a treatment that uses the patients immune system to fight disease. Substances made by the body or made in a laboratory are used to boost, direct, or restore the bodys natural defenses against disease. Interferon is a type of immunotherapy used to treat childhood vascular tumors. It interferes with the division of tumor cells and can slow tumor growth. It is used in the treatment of juvenile nasopharyngeal angiofibroma, kaposiform hemangioendothelioma, and epithelioid hemangioendothelioma. Other drug therapy Other drugs used to treat childhood vascular tumors or manage their effects include the following: - Steroid therapy: Steroids are hormones made naturally in the body. They can also be made in a laboratory and used as drugs. Steroid drugs help shrink some vascular tumors. - Non-steroidal anti-inflammatory drugs (NSAIDs): NSAIDs are commonly used to decrease fever, swelling, pain, and redness. Examples of NSAIDs are aspirin, ibuprofen, and naproxen. In the treatment of vascular tumors, NSAIDs can increase the flow of blood through the tumors and decrease the chance that an unwanted blood clot will form. - Antifibrinolytic therapy: These drugs help the blood clot in patients who have Kasabach-Merritt syndrome. Fibrin is the main protein in a blood clot that helps stop bleeding and heal wounds. Some vascular tumors cause fibrin to break down and the patient's blood does not clot normally, causing uncontrolled bleeding. Antifibrinolytics help prevent the breakdown of fibrin. Observation Observation is closely monitoring a patients condition without giving any treatment until signs or symptoms appear or change. New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the research process. Clinical trials are done to find out if new treatments are safe and effective or better than the standard treatment. Many of today's standard treatments are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way disease will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose tumors have not gotten better. There are also clinical trials that test new ways to stop tumors from recurring (coming back) or reduce the side effects of treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the vascular tumor may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the tumor has recurred (come back). These tests are sometimes called follow-up tests or check-ups.",Childhood Vascular Tumors,0000003_6,CancerGov,https://www.cancer.gov/types/soft-tissue-sarcoma/patient/child-vascular-tumors-treatment-pdq,C0282607,T191,Disorders what research (or clinical trials) is being done for Childhood Vascular Tumors ?,0000003_6-4,research,"New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the research process. Clinical trials are done to find out if new treatments are safe and effective or better than the standard treatment. Many of today's standard treatments are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way disease will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose tumors have not gotten better. There are also clinical trials that test new ways to stop tumors from recurring (coming back) or reduce the side effects of treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Childhood Vascular Tumors,0000003_6,CancerGov,https://www.cancer.gov/types/soft-tissue-sarcoma/patient/child-vascular-tumors-treatment-pdq,C0282607,T191,Disorders What is (are) Adult Hodgkin Lymphoma ?,0000004_1-1,information,"Key Points - Adult Hodgkin lymphoma is a disease in which malignant (cancer) cells form in the lymph system. - There are two main types of Hodgkin lymphoma: classical and nodular lymphocyte-predominant. - Age, gender, and Epstein-Barr infection can affect the risk of adult Hodgkin lymphoma. - Signs of adult Hodgkin lymphoma include swollen lymph nodes, fever, night sweats, and weight loss. - Tests that examine the lymph nodes are used to detect (find) and diagnose adult Hodgkin lymphoma. - Certain factors affect prognosis (chance of recovery) and treatment options. Adult Hodgkin lymphoma is a disease in which malignant (cancer) cells form in the lymph system. Adult Hodgkin lymphoma is a type of cancer that develops in the lymph system, part of the body's immune system. The immune system protects the body from foreign substances, infection, and diseases. The lymph system is made up of the following: - Lymph: Colorless, watery fluid that carries white blood cells called lymphocytes through the lymph system. Lymphocytes protect the body against infections and the growth of tumors. - Lymph vessels: A network of thin tubes that collect lymph from different parts of the body and return it to the bloodstream. - Lymph nodes: Small, bean-shaped structures that filter lymph and store white blood cells that help fight infection and disease. Lymph nodes are located along the network of lymph vessels found throughout the body. Clusters of lymph nodes are found in the neck, underarm, abdomen, pelvis, and groin. - Spleen: An organ that makes lymphocytes, filters the blood, stores blood cells, and destroys old blood cells. It is located on the left side of the abdomen near the stomach. - Thymus: An organ in which lymphocytes grow and multiply. The thymus is in the chest behind the breastbone. - Tonsils: Two small masses of lymph tissue at the back of the throat. The tonsils make lymphocytes. - Bone marrow: The soft, spongy tissue in the center of large bones. Bone marrow makes white blood cells, red blood cells, and platelets. Lymph tissue is also found in other parts of the body such as the stomach, thyroid gland, brain, and skin. Cancer can spread to the liver and lungs. Lymphomas are divided into two general types: Hodgkin lymphoma and non-Hodgkin lymphoma. This summary is about the treatment of adult Hodgkin lymphoma. (See the PDQ summary on Adult Non-Hodgkin Lymphoma Treatment for more information.) Hodgkin lymphoma can occur in both adults and children. Treatment for adults is different than treatment for children. Hodgkin lymphoma may also occur in patients who have acquired immunodeficiency syndrome (AIDS); these patients require special treatment. See the following PDQ summaries for more information: - Childhood Hodgkin Lymphoma Treatment - AIDS-Related Lymphoma Treatment Hodgkin lymphoma in pregnant women is the same as the disease in nonpregnant women of childbearing age. However, treatment is different for pregnant women. This summary includes information about treating Hodgkin lymphoma during pregnancy. There are two main types of Hodgkin lymphoma: classical and nodular lymphocyte-predominant. Most Hodgkin lymphomas are the classical type. The classical type is broken down into the following four subtypes: - Nodular sclerosing Hodgkin lymphoma. - Mixed cellularity Hodgkin lymphoma. - Lymphocyte depletion Hodgkin lymphoma. - Lymphocyte-rich classical Hodgkin lymphoma.",Adult Hodgkin Lymphoma,0000004_1,CancerGov,https://www.cancer.gov/types/lymphoma/patient/adult-hodgkin-treatment-pdq,C0220597,T191,Disorders Who is at risk for Adult Hodgkin Lymphoma? ?,0000004_1-2,susceptibility,"Age, gender, and Epstein-Barr infection can affect the risk of adult Hodgkin lymphoma. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for adult Hodgkin lymphoma include the following: - Being in young or late adulthood. - Being male. - Being infected with the Epstein-Barr virus. - Having a first-degree relative (parent, brother, or sister) with Hodgkin lymphoma. Pregnancy is not a risk factor for Hodgkin lymphoma.",Adult Hodgkin Lymphoma,0000004_1,CancerGov,https://www.cancer.gov/types/lymphoma/patient/adult-hodgkin-treatment-pdq,C0220597,T191,Disorders What are the symptoms of Adult Hodgkin Lymphoma ?,0000004_1-3,symptoms,"Signs of adult Hodgkin lymphoma include swollen lymph nodes, fever, night sweats, and weight loss. These and other signs and symptoms may be caused by adult Hodgkin lymphoma or by other conditions. Check with your doctor if any of the following do not go away: - Painless, swollen lymph nodes in the neck, underarm, or groin. - Fever for no known reason. - Drenching night sweats. - Weight loss for no known reason. - Itchy skin. - Feeling very tired.",Adult Hodgkin Lymphoma,0000004_1,CancerGov,https://www.cancer.gov/types/lymphoma/patient/adult-hodgkin-treatment-pdq,C0220597,T191,Disorders How to diagnose Adult Hodgkin Lymphoma ?,0000004_1-4,exams and tests,"Tests that examine the lymph nodes are used to detect (find) and diagnose adult Hodgkin lymphoma. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient's past illnesses and treatments will also be taken. - Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following: - The number of red blood cells, white blood cells, and platelets. - The amount of hemoglobin (the protein that carries oxygen) in the red blood cells. - The portion of the sample made up of red blood cells. - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. - Sedimentation rate : A procedure in which a sample of blood is drawn and checked for the rate at which the red blood cells settle to the bottom of the test tube. The sedimentation rate is a measure of how much inflammation is in the body. A higher than normal sedimentation rate may be a sign of lymphoma or another condition. Also called erythrocyte sedimentation rate, sed rate, or ESR. - Lymph node biopsy : The removal of all or part of a lymph node. One of the following types of biopsies may be done: - Excisional biopsy : The removal of an entire lymph node. - Incisional biopsy : The removal of part of a lymph node. - Core biopsy : The removal of part of a lymph node using a wide needle. A pathologist views the tissue under a microscope to look for cancer cells, especially Reed-Sternberg cells. Reed-Sternberg cells are common in classical Hodgkin lymphoma. The following test may be done on tissue that was removed: - Immunophenotyping : A laboratory test used to identify cells, based on the types of antigens or markers on the surface of the cell. This test is used to diagnose the specific type of lymphoma by comparing the cancer cells to normal cells of the immune system.",Adult Hodgkin Lymphoma,0000004_1,CancerGov,https://www.cancer.gov/types/lymphoma/patient/adult-hodgkin-treatment-pdq,C0220597,T191,Disorders What is the outlook for Adult Hodgkin Lymphoma ?,0000004_1-5,outlook,"Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - The patient's signs and symptoms. - The stage of the cancer. - The type of Hodgkin lymphoma. - Blood test results. - The patient's age, gender, and general health. - Whether the cancer is recurrent or progressive. For Hodgkin lymphoma during pregnancy, treatment options also depend on: - The wishes of the patient. - The age of the fetus. Adult Hodgkin lymphoma can usually be cured if found and treated early.",Adult Hodgkin Lymphoma,0000004_1,CancerGov,https://www.cancer.gov/types/lymphoma/patient/adult-hodgkin-treatment-pdq,C0220597,T191,Disorders what research (or clinical trials) is being done for Adult Hodgkin Lymphoma ?,0000004_1-6,research,"New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Chemotherapy and radiation therapy with stem cell transplant High-dose chemotherapy and radiation therapy with stem cell transplant is a way of giving high doses of chemotherapy and radiation therapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After therapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. The use of lower-dose chemotherapy and radiation therapy with stem cell transplant is also being studied. Monoclonal antibody therapy Monoclonal antibody therapy is a cancer treatment that uses antibodies made in the laboratory, from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Adult Hodgkin Lymphoma,0000004_1,CancerGov,https://www.cancer.gov/types/lymphoma/patient/adult-hodgkin-treatment-pdq,C0220597,T191,Disorders What are the stages of Adult Hodgkin Lymphoma ?,0000004_1-7,stages,"Key Points - After adult Hodgkin lymphoma has been diagnosed, tests are done to find out if cancer cells have spread within the lymph system or to other parts of the body. - There are three ways that cancer spreads in the body. - Stages of adult Hodgkin lymphoma may include A, B, E, and S. - The following stages are used for adult Hodgkin lymphoma: - Stage I - Stage II - Stage III - Stage IV - Adult Hodgkin lymphoma may be grouped for treatment as follows: - Early Favorable - Early Unfavorable - Advanced After adult Hodgkin lymphoma has been diagnosed, tests are done to find out if cancer cells have spread within the lymph system or to other parts of the body. The process used to find out if cancer has spread within the lymph system or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following tests and procedures may be used in the staging process: - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. For adult Hodgkin lymphoma, CT scans of the neck, chest, abdomen, and pelvis are taken. - PET-CT scan : A procedure that combines the pictures from a positron emission tomography (PET) scan and a computed tomography (CT) scan. The PET and CT scans are done at the same time on the same machine. The pictures from both scans are combined to make a more detailed picture than either test would make by itself. A PET scan is a procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. - Bone marrow aspiration and biopsy : The removal of bone marrow, blood, and a small piece of bone by inserting a hollow needle into the hipbone or breastbone. A pathologist views the bone marrow, blood, and bone under a microscope to look for signs of cancer. For pregnant women with Hodgkin lymphoma, staging tests that protect the fetus from the harms of radiation are used. These include: - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Stages of adult Hodgkin lymphoma may include A, B, E, and S. Adult Hodgkin lymphoma may be described as follows: - A: The patient does not have B symptoms (fever, weight loss, or night sweats). - B: The patient has B symptoms. - E: Cancer is found in an organ or tissue that is not part of the lymph system but which may be next to an involved area of the lymph system. - S: Cancer is found in the spleen. The following stages are used for adult Hodgkin lymphoma: Stage I Stage I is divided into stage I and stage IE. - Stage I: Cancer is found in one of the following places in the lymph system: - One or more lymph nodes in one lymph node group. - Waldeyer's ring. - Thymus. - Spleen. - Stage IE: Cancer is found outside the lymph system in one organ or area. Stage II Stage II is divided into stage II and stage IIE. - Stage II: Cancer is found in two or more lymph node groups either above or below the diaphragm (the thin muscle below the lungs that helps breathing and separates the chest from the abdomen). - Stage IIE: Cancer is found in one or more lymph node groups either above or below the diaphragm and outside the lymph nodes in a nearby organ or area. Stage III Stage III is divided into stage III, stage IIIE, stage IIIS, and stage IIIE,S. - Stage III: Cancer is found in lymph node groups above and below the diaphragm (the thin muscle below the lungs that helps breathing and separates the chest from the abdomen). - Stage IIIE: Cancer is found in lymph node groups above and below the diaphragm and outside the lymph nodes in a nearby organ or area. - Stage IIIS: Cancer is found in lymph node groups above and below the diaphragm, and in the spleen. - Stage IIIE,S: Cancer is found in lymph node groups above and below the diaphragm, outside the lymph nodes in a nearby organ or area, and in the spleen. Stage IV In stage IV, the cancer: - is found outside the lymph nodes throughout one or more organs, and may be in lymph nodes near those organs; or - is found outside the lymph nodes in one organ and has spread to areas far away from that organ; or - is found in the lung, liver, bone marrow, or cerebrospinal fluid (CSF). The cancer has not spread to the lung, liver, bone marrow, or CSF from nearby areas. Adult Hodgkin lymphoma may be grouped for treatment as follows: Early Favorable Early favorable adult Hodgkin lymphoma is stage I or stage II, without risk factors. Early Unfavorable Early unfavorable adult Hodgkin lymphoma is stage I or stage II with one or more of the following risk factors: - A tumor in the chest that is larger than 1/3 of the width of the chest or at least 10 centimeters. - Cancer in an organ other than the lymph nodes. - A high sedimentation rate (in a sample of blood, the red blood cells settle to the bottom of the test tube more quickly than normal). - Three or more lymph nodes with cancer. - Symptoms such as fever, weight loss, or night sweats. Advanced Advanced Hodgkin lymphoma includes some or all of the following risk factors: - Being male. - Being aged 45 years or older. - Having stage IV disease. - Having a low blood albumin (protein) level (below 4). - Having a low hemoglobin level (below 10.5). - Having a high white blood cell count (15,000 or higher). - Having a low lymphocyte count (below 600 or less than 8% of the white blood cell count).",Adult Hodgkin Lymphoma,0000004_1,CancerGov,https://www.cancer.gov/types/lymphoma/patient/adult-hodgkin-treatment-pdq,C0220597,T191,Disorders What are the treatments for Adult Hodgkin Lymphoma ?,0000004_1-8,treatment,"Key Points - There are different types of treatment for patients with adult Hodgkin lymphoma. - Patients with Hodgkin lymphoma should have their treatment planned by a team of health care providers with expertise in treating lymphomas. - Patients may develop late effects that appear months or years after their treatment for Hodgkin lymphoma. - Three types of standard treatment are used: - Chemotherapy - Radiation therapy - Surgery - For pregnant patients with Hodgkin lymphoma, treatment options also include: - Watchful waiting - Steroid therapy - New types of treatment are being tested in clinical trials. - Chemotherapy and radiation therapy with stem cell transplant - Monoclonal antibody therapy - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with adult Hodgkin lymphoma. Different types of treatment are available for patients with adult Hodgkin lymphoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. For pregnant women with Hodgkin lymphoma, treatment is carefully chosen to protect the fetus. Treatment decisions are based on the mothers wishes, the stage of the Hodgkin lymphoma, and the age of the fetus. The treatment plan may change as the signs and symptoms, cancer, and pregnancy change. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team. Patients with Hodgkin lymphoma should have their treatment planned by a team of health care providers with expertise in treating lymphomas. Treatment will be overseen by a medical oncologist, a doctor who specializes in treating cancer. The medical oncologist may refer you to other health care providers who have experience and expertise in treating adult Hodgkin lymphoma and who specialize in certain areas of medicine. These may include the following specialists: - Neurosurgeon. - Neurologist. - Rehabilitation specialist. - Radiation oncologist. - Endocrinologist. - Hematologist. - Other oncology specialists. Patients may develop late effects that appear months or years after their treatment for Hodgkin lymphoma. Treatment with chemotherapy and/or radiation therapy for Hodgkin lymphoma may increase the risk of second cancers and other health problems for many months or years after treatment. These late effects depend on the type of treatment and the patient's age when treated, and may include: - Acute myelogenous leukemia. - Cancer of the breast, bone, cervix, gastrointestinal tract, head and neck, lung, soft tissue, and thyroid. - Heart, lung, and thyroid disease. - Avascular necrosis of bone (death of bone cells caused by lack of blood flow). - Herpes zoster (shingles) or severe infection. - Depression and fatigue. - Infertility. - Hypogonadism (low levels of testosterone and estrogen). Regular follow-up by doctors who are expert in finding and treating late effects is important for the long-term health of patients treated for Hodgkin lymphoma. Three types of standard treatment are used: Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. Combination chemotherapy is treatment with more than one anticancer drug. When a pregnant woman is treated with chemotherapy for Hodgkin lymphoma, it isn't possible to protect the fetus from being exposed to the chemotherapy. Some chemotherapy regimens may cause birth defects if given in the first trimester. Vinblastine is an anticancer drug that has not been linked with birth defects when given in the second half of pregnancy. See Drugs Approved for Hodgkin Lymphoma for more information. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External radiation therapy is used to treat adult Hodgkin lymphoma. For a pregnant woman with Hodgkin lymphoma, radiation therapy should be postponed until after delivery, if possible, to avoid any risk to the fetus. If immediate treatment is needed, the woman may decide to continue the pregnancy and receive radiation therapy. However, lead used to shield the fetus may not protect it from scattered radiation that could possibly cause cancer in the future. Surgery Laparotomy is a procedure in which an incision (cut) is made in the wall of the abdomen to check the inside of the abdomen for signs of disease. The size of the incision depends on the reason the laparotomy is being done. Sometimes organs are removed or tissue samples are taken and checked under a microscope for signs of disease. If cancer is found, the tissue or organ is removed during the laparotomy. For pregnant patients with Hodgkin lymphoma, treatment options also include: Watchful waiting Watchful waiting is closely monitoring a patients condition without giving any treatment unless signs or symptoms appear or change. Delivery may be induced when the fetus is 32 to 36 weeks old, so that the mother can begin treatment. Steroid therapy Steroids are hormones made naturally in the body by the adrenal glands and by reproductive organs. Some types of steroids are made in a laboratory. Certain steroid drugs have been found to help chemotherapy work better and help stop the growth of cancer cells. Steroids can also help the lungs of the fetus develop faster than normal. This is important when delivery is induced early. See Drugs Approved for Hodgkin Lymphoma for more information. New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Chemotherapy and radiation therapy with stem cell transplant High-dose chemotherapy and radiation therapy with stem cell transplant is a way of giving high doses of chemotherapy and radiation therapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After therapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. The use of lower-dose chemotherapy and radiation therapy with stem cell transplant is also being studied. Monoclonal antibody therapy Monoclonal antibody therapy is a cancer treatment that uses antibodies made in the laboratory, from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Adult Hodgkin Lymphoma Early Favorable Hodgkin Lymphoma Treatment of early favorable Hodgkin lymphoma may include the following: - Combination chemotherapy. - Combination chemotherapy with radiation therapy to parts of the body with cancer. - Radiation therapy alone to areas of the body with cancer or to the mantle field (neck, chest, armpits). Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I adult Hodgkin lymphoma and stage II adult Hodgkin lymphoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Early Unfavorable Hodgkin Lymphoma Treatment of early unfavorable Hodgkin lymphoma may include the following: - Combination chemotherapy. - Combination chemotherapy with radiation therapy to parts of the body with cancer. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I adult Hodgkin lymphoma and stage II adult Hodgkin lymphoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Advanced Hodgkin Lymphoma Treatment of advanced Hodgkin lymphoma may include the following: - Combination chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III adult Hodgkin lymphoma and stage IV adult Hodgkin lymphoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Recurrent Adult Hodgkin Lymphoma Treatment of recurrent Hodgkin lymphoma may include the following: - Combination chemotherapy. - Combination chemotherapy followed by high-dose chemotherapy and stem cell transplant with or without radiation therapy. - Combination chemotherapy with radiation therapy to parts of the body with cancer in patients older than 60 years. - Radiation therapy with or without chemotherapy. - Chemotherapy as palliative therapy to relieve symptoms and improve quality of life. - A clinical trial of high-dose chemotherapy and stem cell transplant. - A clinical trial of lower-dose chemotherapy and radiation therapy followed by stem cell transplant. - A clinical trial of a monoclonal antibody. - A clinical trial of chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent adult Hodgkin lymphoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Treatment Options for Hodgkin Lymphoma During Pregnancy Hodgkin Lymphoma During the First Trimester of Pregnancy When Hodgkin lymphoma is diagnosed in the first trimester of pregnancy, it does not necessarily mean that the woman will be advised to end the pregnancy. Each woman's treatment will depend on the stage of the lymphoma, how fast it is growing, and her wishes. For women who choose to continue the pregnancy, treatment of Hodgkin lymphoma during the first trimester of pregnancy may include the following: - Watchful waiting when the cancer is above the diaphragm and is slow-growing. Delivery may be induced when the fetus is 32 to 36 weeks old so the mother can begin treatment. - Radiation therapy above the diaphragm. (A lead shield is used to protect the fetus from the radiation as much as possible.) - Systemic chemotherapy using one or more drugs. Hodgkin Lymphoma During the Second Half of Pregnancy When Hodgkin lymphoma is diagnosed in the second half of pregnancy, most women can delay treatment until after the baby is born. Treatment of Hodgkin lymphoma during the second half of pregnancy may include the following: - Watchful waiting, with plans to induce delivery when the fetus is 32 to 36 weeks old. - Systemic chemotherapy using one or more drugs. - Steroid therapy. - Radiation therapy to relieve breathing problems caused by a large tumor in the chest.",Adult Hodgkin Lymphoma,0000004_1,CancerGov,https://www.cancer.gov/types/lymphoma/patient/adult-hodgkin-treatment-pdq,C0220597,T191,Disorders What is (are) Adult Non-Hodgkin Lymphoma ?,0000004_2-1,information,"Key Points - Adult non-Hodgkin lymphoma is a disease in which malignant (cancer) cells form in the lymph system. - The major types of lymphoma are Hodgkin lymphoma and non-Hodgkin lymphoma. - Non-Hodgkin lymphoma can be indolent or aggressive. - Age, gender, and a weakened immune system can affect the risk of adult non-Hodgkin lymphoma. - Signs and symptoms of adult non-Hodgkin lymphoma include swelling in the lymph nodes, fever, night sweats, weight loss, and fatigue. - Tests that examine the body and lymph system are used to help detect (find) and diagnose adult non-Hodgkin lymphoma. - Certain factors affect prognosis (chance of recovery) and treatment options. Adult non-Hodgkin lymphoma is a disease in which malignant (cancer) cells form in the lymph system. Non-Hodgkin lymphoma is a type of cancer that forms in the lymph system, which is part of the body's immune system. The immune system protects the body from foreign substances, infection, and diseases. The lymph system is made up of the following: - Lymph: Colorless, watery fluid that carries white blood cells called lymphocytes through the lymph system. Lymphocytes protect the body against infection and the growth of tumors. There are three types of lymphocytes: - B lymphocytes that make antibodies to help fight infection. Also called B cells. Most types of non-Hodgkin lymphoma begin in B lymphocytes. - T lymphocytes that help B lymphocytes make the antibodies that help fight infection. Also called T cells. - Natural killer cells that attack cancer cells and viruses. Also called NK cells. Non-Hodgkin lymphoma can begin in B lymphocytes, T lymphocytes, or natural killer cells. Lymphocytes can also be found in the blood and also collect in the lymph nodes, spleen, and thymus. - Lymph vessels: A network of thin tubes that collect lymph from different parts of the body and return it to the bloodstream. - Lymph nodes: Small, bean-shaped structures that filter lymph and store white blood cells that help fight infection and disease. Lymph nodes are located along the network of lymph vessels found throughout the body. Clusters of lymph nodes are found in the neck, underarm, abdomen, pelvis, and groin. - Spleen: An organ that makes lymphocytes, filters the blood, stores blood cells, and destroys old blood cells. It is on the left side of the abdomen near the stomach. - Thymus: An organ in which lymphocytes grow and multiply. The thymus is in the chest behind the breastbone. - Tonsils: Two small masses of lymph tissue at the back of the throat. The tonsils make lymphocytes. - Bone marrow: The soft, spongy tissue in the center of large bones. Bone marrow makes white blood cells, red blood cells, and platelets. Lymph tissue is also found in other parts of the body such as the stomach, thyroid gland, brain, and skin. Cancer can spread to the liver and lungs. Non-Hodgkin lymphoma during pregnancy is rare. Non-Hodgkin lymphoma in pregnant women is the same as the disease in nonpregnant women of childbearing age. However, treatment is different for pregnant women. This summary includes information on the treatment of non-Hodgkin lymphoma during pregnancy (see the Treatment Options for Non-Hodgkin Lymphoma During Pregnancy section for more information). Non-Hodgkin lymphoma can occur in both adults and children. Treatment for adults is different than treatment for children. (See the PDQ summary on Childhood Non-Hodgkin Lymphoma Treatment for more information.) The major types of lymphoma are Hodgkin lymphoma and non-Hodgkin lymphoma. Lymphomas are divided into two general types: Hodgkin lymphoma and non-Hodgkin lymphoma. This summary is about the treatment of adult non-Hodgkin lymphoma. For information about certain types of lymphoma, see the following PDQ summaries: - Adult Acute Lymphoblastic Leukemia Treatment (lymphoblastic lymphoma) - Adult Hodgkin Lymphoma Treatment - AIDS-Related Lymphoma Treatment - Chronic Lymphocytic Leukemia Treatment (small lymphocytic lymphoma) - Mycosis Fungoides and the Szary Syndrome Treatment (cutaneous T-cell lymphoma) - Primary CNS Lymphoma Treatment Non-Hodgkin lymphoma can be indolent or aggressive. Non-Hodgkin lymphoma grows and spreads at different rates and can be indolent or aggressive. Indolent lymphoma tends to grow and spread slowly, and has few signs and symptoms. Aggressive lymphoma grows and spreads quickly, and has signs and symptoms that can be severe. The treatments for indolent and aggressive lymphoma are different. This summary is about the following types of non-Hodgkin lymphoma: Indolent non-Hodgkin lymphomas - Follicular lymphoma. Follicular lymphoma is the most common type of indolent non-Hodgkin lymphoma. It is a very slow-growing type of non-Hodgkin lymphoma that begins in B lymphocytes. It affects the lymph nodes and may spread to the bone marrow or spleen. Most patients with follicular lymphoma are age 50 years and older when they are diagnosed. Follicular lymphoma may go away without treatment. The patient is closely watched for signs or symptoms that the disease has come back. Treatment is needed if signs or symptoms occur after the cancer disappeared or after initial cancer treatment. Sometimes follicular lymphoma can become a more aggressive type of lymphoma, such as diffuse large B-cell lymphoma. - Lymphoplasmacytic lymphoma. In most cases of lymphoplasmacytic lymphoma, B lymphocytes that are turning into plasma cells make large amounts of a protein called monoclonal immunoglobulin M (IgM) antibody. High levels of IgM antibody in the blood cause the blood plasma to thicken. This may cause signs or symptoms such as trouble seeing or hearing, heart problems, shortness of breath, headache, dizziness, and numbness or tingling of the hands and feet. Sometimes there are no signs or symptoms of lymphoplasmacytic lymphoma. It may be found when a blood test is done for another reason. Lymphoplasmacytic lymphoma often spreads to the bone marrow, lymph nodes, and spleen. It is also called Waldenstrm macroglobulinemia. - Marginal zone lymphoma. This type of non-Hodgkin lymphoma begins in B lymphocytes in a part of lymph tissue called the marginal zone. There are five different types of marginal zone lymphoma. They are grouped by the type of tissue where the lymphoma formed: - Nodal marginal zone lymphoma. Nodal marginal zone lymphoma forms in lymph nodes. This type of non-Hodgkin lymphoma is rare. It is also called monocytoid B-cell lymphoma. - Gastric mucosa-associated lymphoid tissue (MALT) lymphoma. Gastric MALT lymphoma usually begins in the stomach. This type of marginal zone lymphoma forms in cells in the mucosa that help make antibodies. Patients with gastric MALT lymphoma may also have Helicobacter gastritis or an autoimmune disease, such as Hashimoto thyroiditis or Sjgren syndrome. - Extragastric MALT lymphoma. Extragastric MALT lymphoma begins outside of the stomach in almost every part of the body including other parts of the gastrointestinal tract, salivary glands, thyroid, lung, skin, and around the eye. This type of marginal zone lymphoma forms in cells in the mucosa that help make antibodies. Extragastric MALT lymphoma may come back many years after treatment. - Mediterranean abdominal lymphoma. This is a type of MALT lymphoma that occurs in young adults in eastern Mediterranean countries. It often forms in the abdomen and patients may also be infected with bacteria called Campylobacter jejuni. This type of lymphoma is also called immunoproliferative small intestinal disease. - Splenic marginal zone lymphoma. This type of marginal zone lymphoma begins in the spleen and may spread to the peripheral blood and bone marrow. The most common sign of this type of splenic marginal zone lymphoma is a spleen that is larger than normal. - Primary cutaneous anaplastic large cell lymphoma. This type of non-Hodgkin lymphoma is in the skin only. It can be a benign (not cancer) nodule that may go away on its own or it can spread to many places on the skin and need treatment. Aggressive non-Hodgkin lymphomas - Diffuse large B-cell lymphoma. Diffuse large B-cell lymphoma is the most common type of non-Hodgkin lymphoma. It grows quickly in the lymph nodes and often the spleen, liver, bone marrow, or other organs are also affected. Signs and symptoms of diffuse large B-cell lymphoma may include fever, recurring night sweats, and weight loss. These are also called B symptoms. Primary mediastinal large B-cell lymphoma is a type of diffuse large B-cell lymphoma. - Primary mediastinal large B-cell lymphoma. This type of non-Hodgkin lymphoma is marked by the overgrowth of fibrous (scar-like) lymph tissue. A tumor most often forms behind the breastbone. It may press on the airways and cause coughing and trouble breathing. Most patients with primary mediastinal large B-cell lymphoma are women who are age 30 to 40 years. - Follicular large cell lymphoma, stage III. Follicular large cell lymphoma, stage III, is a very rare type of non-Hodgkin lymphoma. It is more like diffuse large B-cell lymphoma than other types of follicular lymphoma. - Anaplastic large cell lymphoma. Anaplastic large cell lymphoma is a type of non-Hodgkin lymphoma that usually begins in T lymphocytes. The cancer cells also have a marker called CD30 on the surface of the cell. There are two types of anaplastic large cell lymphoma: - Cutaneous anaplastic large cell lymphoma. This type of anaplastic large cell lymphoma mostly affects the skin, but other parts of the body may also be affected. Signs of cutaneous anaplastic large cell lymphoma include one or more bumps or ulcers on the skin. - Systemic anaplastic large cell lymphoma. This type of anaplastic large cell lymphoma begins in the lymph nodes and may affect other parts of the body. Patients may have a lot of anaplastic lymphoma kinase (ALK) protein inside the lymphoma cells. These patients have a better prognosis than patients who do not have extra ALK protein. Systemic anaplastic large cell lymphoma is more common in children than adults. (See the PDQ summary on Childhood Non-Hodgkin Lymphoma Treatment for more information.) - Extranodal NK -/T-cell lymphoma. Extranodal NK-/T-cell lymphoma usually begins in the area around the nose. It may also affect the paranasal sinus (hollow spaces in the bones around the nose), roof of the mouth, trachea, skin, stomach, and intestines. Most cases of extranodal NK-/T-cell lymphoma have Epstein-Barr virus in the tumor cells. Sometimes hemophagocytic syndrome occurs (a serious condition in which there are too many active histiocytes and T cells that cause severe inflammation in the body). Treatment to suppress the immune system is needed. This type of non-Hodgkin lymphoma is not common in the United States. - Lymphomatoid granulomatosis. Lymphomatoid granulomatosis mostly affects the lungs. It may also affect the paranasal sinuses (hollow spaces in the bones around the nose), skin, kidneys, and central nervous system. In lymphomatoid granulomatosis, cancer invades the blood vessels and kills tissue. Because the cancer may spread to the brain, intrathecal chemotherapy or radiation therapy to the brain is given. - Angioimmunoblastic T-cell lymphoma. This type of non-Hodgkin lymphoma begins in T cells. Swollen lymph nodes are a common sign. Other signs may include a skin rash, fever, weight loss, or night sweats. There may also be high levels of gamma globulin (antibodies) in the blood. Patients may also have opportunistic infections because their immune systems are weakened. - Peripheral T-cell lymphoma. Peripheral T-cell lymphoma begins in mature T lymphocytes. This type of T lymphocyte matures in the thymus gland and travels to other lymphatic sites in the body such as the lymph nodes, bone marrow, and spleen. There are three subtypes of peripheral T-cell lymphoma: - Hepatosplenic T-cell lymphoma. This is an uncommon type of peripheral T-cell lymphoma that occurs mostly in young men. It begins in the liver and spleen and the cancer cells also have a T-cell receptor called gamma/delta on the surface of the cell. - Subcutaneous panniculitis-like T-cell lymphoma. Subcutaneous panniculitis-like T-cell lymphoma begins in the skin or mucosa. It may occur with hemophagocytic syndrome (a serious condition in which there are too many active histiocytes and T cells that cause severe inflammation in the body). Treatment to suppress the immune system is needed. - Enteropathy-type intestinal T-cell lymphoma. This type of peripheral T-cell lymphoma occurs in the small bowel of patients with untreated celiac disease (an immune response to gluten that causes malnutrition). Patients who are diagnosed with celiac disease in childhood and stay on a gluten-free diet rarely develop enteropathy-type intestinal T-cell lymphoma. - Intravascular large B-cell lymphoma. This type of non-Hodgkin lymphoma affects blood vessels, especially the small blood vessels in the brain, kidney, lung, and skin. Signs and symptoms of intravascular large B-cell lymphoma are caused by blocked blood vessels. It is also called intravascular lymphomatosis. - Burkitt lymphoma. Burkitt lymphoma is a type of B-cell non-Hodgkin lymphoma that grows and spreads very quickly. It may affect the jaw, bones of the face, bowel, kidneys, ovaries, or other organs. There are three main types of Burkitt lymphoma (endemic, sporadic, and immunodeficiency related). Endemic Burkitt lymphoma commonly occurs in Africa and is linked to the Epstein-Barr virus, and sporadic Burkitt lymphoma occurs throughout the world. Immunodeficiency-related Burkitt lymphoma is most often seen in patients who have AIDS. Burkitt lymphoma may spread to the brain and spinal cord and treatment to prevent its spread may be given. Burkitt lymphoma occurs most often in children and young adults (See the PDQ summary on Childhood Non-Hodgkin Lymphoma Treatment for more information.) Burkitt lymphoma is also called diffuse small noncleaved-cell lymphoma. - Lymphoblastic lymphoma. Lymphoblastic lymphoma may begin in T cells or B cells, but it usually begins in T cells. In this type of non-Hodgkin lymphoma, there are too many lymphoblasts (immature white blood cells) in the lymph nodes and the thymus gland. These lymphoblasts may spread to other places in the body, such as the bone marrow, brain, and spinal cord. Lymphoblastic lymphoma is most common in teenagers and young adults. It is a lot like acute lymphoblastic leukemia (lymphoblasts are mostly found in the bone marrow and blood). (See the PDQ summary on Adult Acute Lymphoblastic Leukemia Treatment for more information.) - Adult T-cell leukemia/lymphoma. Adult T-cell leukemia/lymphoma is caused by the human T-cell leukemia virus type 1 (HTLV-1). Signs include bone and skin lesions, high blood calcium levels, and lymph nodes, spleen, and liver that are larger than normal. - Mantle cell lymphoma. Mantle cell lymphoma is a type of B-cell non-Hodgkin lymphoma that usually occurs in middle-aged or older adults. It begins in the lymph nodes and spreads to the spleen, bone marrow, blood, and sometimes the esophagus, stomach, and intestines. Patients with mantle cell lymphoma have too much of a protein called cyclin-D1 or a certain gene change in the lymphoma cells. In some patients who do not have signs or symptoms of lymphoma delaying the start of treatment does not affect the prognosis. - Posttransplantation lymphoproliferative disorder. This disease occurs in patients who have had a heart, lung, liver, kidney, or pancreas transplant and need lifelong immunosuppressive therapy. Most posttransplant lymphoproliferative disorders affect the B cells and have Epstein-Barr virus in the cells. Lymphoproliferative disorders are often treated like cancer. - True histiocytic lymphoma. This is a rare, very aggressive type of lymphoma. It is not known whether it begins in B cells or T cells. It does not respond well to treatment with standard chemotherapy. - Primary effusion lymphoma. Primary effusion lymphoma begins in B cells that are found in an area where there is a large build-up of fluid, such as the areas between the lining of the lung and chest wall (pleural effusion), the sac around the heart and the heart (pericardial effusion), or in the abdominal cavity. There is usually no tumor that can be seen. This type of lymphoma often occurs in patients who have AIDS. - Plasmablastic lymphoma. Plasmablastic lymphoma is a type of large B-cell non-Hodgkin lymphoma that is very aggressive. It is most often seen in patients with HIV infection.",Adult Non-Hodgkin Lymphoma,0000004_2,CancerGov,https://www.cancer.gov/types/lymphoma/patient/adult-nhl-treatment-pdq,C0019829,T191,Disorders what research (or clinical trials) is being done for Adult Non-Hodgkin Lymphoma ?,0000004_2-2,research,"New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Vaccine therapy Vaccine therapy is a type of biologic therapy. Biologic therapy is a treatment that uses the patients immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the bodys natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy. Vaccine therapy can also be a type of targeted therapy. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Adult Non-Hodgkin Lymphoma,0000004_2,CancerGov,https://www.cancer.gov/types/lymphoma/patient/adult-nhl-treatment-pdq,C0019829,T191,Disorders Who is at risk for Adult Non-Hodgkin Lymphoma? ?,0000004_2-3,susceptibility,"Age, gender, and a weakened immune system can affect the risk of adult non-Hodgkin lymphoma. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesnt mean that you will not get cancer. Talk with your doctor if you think you may be at risk. These and other risk factors may increase the risk of certain types of adult non-Hodgkin lymphoma: - Being older, male, or white. - Having one of the following medical conditions: - An inherited immune disorder (such as hypogammaglobulinemia or Wiskott-Aldrich syndrome). - An autoimmune disease (such as rheumatoid arthritis, psoriasis, or Sjgren syndrome). - HIV/AIDS. - Human T-lymphotrophic virus type I or Epstein-Barr virus infection. - Helicobacter pylori infection. - Taking immunosuppressant drugs after an organ transplant.",Adult Non-Hodgkin Lymphoma,0000004_2,CancerGov,https://www.cancer.gov/types/lymphoma/patient/adult-nhl-treatment-pdq,C0019829,T191,Disorders What are the symptoms of Adult Non-Hodgkin Lymphoma ?,0000004_2-4,symptoms,"Signs and symptoms of adult non-Hodgkin lymphoma include swelling in the lymph nodes, fever, night sweats, weight loss, and fatigue. These signs and symptoms may be caused by adult non-Hodgkin lymphoma or by other conditions. Check with your doctor if you have any of the following: - Swelling in the lymph nodes in the neck, underarm, groin, or stomach. - Fever for no known reason. - Recurring night sweats. - Feeling very tired. - Weight loss for no known reason. - Skin rash or itchy skin. - Pain in the chest, abdomen, or bones for no known reason. When fever, night sweats, and weight loss occur together, this group of symptoms is called B symptoms. Other signs and symptoms of adult non-Hodgkin lymphoma may occur and depend on the following: - Where the cancer forms in the body. - The size of the tumor. - How fast the tumor grows.",Adult Non-Hodgkin Lymphoma,0000004_2,CancerGov,https://www.cancer.gov/types/lymphoma/patient/adult-nhl-treatment-pdq,C0019829,T191,Disorders How to diagnose Adult Non-Hodgkin Lymphoma ?,0000004_2-5,exams and tests,"Tests that examine the body and lymph system are used to help detect (find) and diagnose adult non-Hodgkin lymphoma. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Flow cytometry : A laboratory test that measures the number of cells in a sample, the percentage of live cells in a sample, and certain characteristics of cells, such as size, shape, and the presence of tumor markers on the cell surface. The cells are stained with a light-sensitive dye, placed in a fluid, and passed in a stream before a laser or other type of light. The measurements are based on how the light-sensitive dye reacts to the light. This test is used to diagnose lymphoplasmacytic lymphoma. - Bone marrow aspiration and biopsy : The removal of bone marrow and a small piece of bone by inserting a needle into the hipbone or breastbone. A pathologist views the bone marrow and bone under a microscope to look for signs of cancer. - Lymph node biopsy: The removal of all or part of a lymph node. A pathologist views the tissue under a microscope to look for cancer cells. One of the following types of biopsies may be done: - Excisional biopsy : The removal of an entire lymph node. - Incisional biopsy : The removal of part of a lymph node. - Core biopsy : The removal of part of a lymph node using a wide needle. - Fine-needle aspiration (FNA) biopsy: The removal of tissue or fluid using a thin needle. - Laparoscopy : A surgical procedure to look at the organs inside the abdomen to check for signs of disease. Small incisions (cuts) are made in the wall of the abdomen and a laparoscope (a thin, lighted tube) is inserted into one of the incisions. Other instruments may be inserted through the same or other incisions to take tissue samples to be checked under a microscope for signs of disease. - Laparotomy : A surgical procedure in which an incision (cut) is made in the wall of the abdomen to check the inside of the abdomen for signs of disease. Tissue samples are taken and checked under a microscope for signs of disease. If cancer is found, the following tests may be done to study the cancer cells: - Immunohistochemistry : A test that uses antibodies to check for certain antigens in a sample of tissue. The antibody is usually linked to a radioactive substance or a dye that causes the tissue to light up under a microscope. This type of test may be used to tell the difference between different types of cancer. - Cytogenetic analysis : A laboratory test in which cells in a sample of tissue are viewed under a microscope to look for certain changes in the chromosomes. - FISH (fluorescence in situ hybridization): A laboratory test used to look at genes or chromosomes in cells and tissues. Pieces of DNA that contain a fluorescent dye are made in the laboratory and added to cells or tissues on a glass slide. When these pieces of DNA attach to certain genes or areas of chromosomes on the slide, they light up when viewed under a microscope with a special light. This type of test is used to look for certain genetic markers. - Immunophenotyping : A process used to identify cells, based on the types of antigens or markers on the surface of the cell. This process is used to diagnose specific types of leukemia and lymphoma by comparing the cancer cells to normal cells of the immune system. Other tests and procedures may be done depending on the signs and symptoms seen and where the cancer forms in the body.",Adult Non-Hodgkin Lymphoma,0000004_2,CancerGov,https://www.cancer.gov/types/lymphoma/patient/adult-nhl-treatment-pdq,C0019829,T191,Disorders What is the outlook for Adult Non-Hodgkin Lymphoma ?,0000004_2-6,outlook,"Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - The stage of the cancer. - The type of non-Hodgkin lymphoma. - The amount of lactate dehydrogenase (LDH) in the blood. - Whether there are certain changes in the genes. - The patients age and general health. - Whether the lymphoma has just been diagnosed or has recurred (come back). For non-Hodgkin lymphoma during pregnancy, the treatment options also depend on: - The wishes of the patient. - Which trimester of pregnancy the patient is in. - Whether the baby can be delivered early. Some types of non-Hodgkin lymphoma spread more quickly than others do. Most non-Hodgkin lymphomas that occur during pregnancy are aggressive. Delaying treatment of aggressive lymphoma until after the baby is born may lessen the mother's chance of survival. Immediate treatment is often recommended, even during pregnancy.",Adult Non-Hodgkin Lymphoma,0000004_2,CancerGov,https://www.cancer.gov/types/lymphoma/patient/adult-nhl-treatment-pdq,C0019829,T191,Disorders What are the stages of Adult Non-Hodgkin Lymphoma ?,0000004_2-7,stages,"Key Points - After adult non-Hodgkin lymphoma has been diagnosed, tests are done to find out if cancer cells have spread within the lymph system or to other parts of the body. - There are three ways that cancer spreads in the body. - Stages of adult non-Hodgkin lymphoma may include E and S. - The following stages are used for adult non-Hodgkin lymphoma: - Stage I - Stage II - Stage III - Stage IV - Adult non-Hodgkin lymphomas may be grouped for treatment according to whether the cancer is indolent or aggressive and whether affected lymph nodes are next to each other in the body. After adult non-Hodgkin lymphoma has been diagnosed, tests are done to find out if cancer cells have spread within the lymph system or to other parts of the body. The process used to find out the type of cancer and if cancer cells have spread within the lymph system or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage of the disease in order to plan treatment. The results of the tests and procedures done to diagnose non-Hodgkin lymphoma are used to help make decisions about treatment. The following tests and procedures may also be used in the staging process: - Complete blood count (CBC) with differential : A procedure in which a sample of blood is drawn and checked for the following: - The number of red blood cells and platelets. - The number and type of white blood cells. - The amount of hemoglobin (the protein that carries oxygen) in the red blood cells. - The portion of the blood sample made up of red blood cells. - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the lung, lymph nodes, and liver, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Bone marrow aspiration and biopsy : The removal of bone marrow and a small piece of bone by inserting a needle into the hipbone or breastbone. A pathologist views the bone marrow and bone under a microscope to look for signs of cancer. - Lumbar puncture : A procedure used to collect cerebrospinal fluid (CSF) from the spinal column. This is done by placing a needle between two bones in the spine and into the CSF around the spinal cord and removing a sample of the fluid. The sample of CSF is checked under a microscope for signs that the cancer has spread to the brain and spinal cord. This procedure is also called an LP or spinal tap. For pregnant women with non-Hodgkin lymphoma, staging tests and procedures that protect the baby from the harms of radiation are used. These tests and procedures include MRI, bone marrow aspiration and biopsy, lumbar puncture, and ultrasound. An ultrasound exam is a procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Stages of adult non-Hodgkin lymphoma may include E and S. Adult non-Hodgkin lymphoma may be described as follows: - E: ""E"" stands for extranodal and means the cancer is found in an area or organ other than the lymph nodes or has spread to tissues beyond, but near, the major lymphatic areas. - S: ""S"" stands for spleen and means the cancer is found in the spleen. The following stages are used for adult non-Hodgkin lymphoma: Stage I Stage I adult non-Hodgkin lymphoma is divided into stage I and stage IE. - Stage I: Cancer is found in one lymphatic area (lymph node group, tonsils and nearby tissue, thymus, or spleen). - Stage IE: Cancer is found in one organ or area outside the lymph nodes. Stage II Stage II adult non-Hodgkin lymphoma is divided into stage II and stage IIE. - Stage II: Cancer is found in two or more lymph node groups either above or below the diaphragm (the thin muscle below the lungs that helps breathing and separates the chest from the abdomen). - Stage IIE: Cancer is found in one or more lymph node groups either above or below the diaphragm. Cancer is also found outside the lymph nodes in one organ or area on the same side of the diaphragm as the affected lymph nodes. Stage III Stage III adult non-Hodgkin lymphoma is divided into stage III, stage IIIE, stage IIIS, and stage IIIE+S. - Stage III: Cancer is found in lymph node groups above and below the diaphragm (the thin muscle below the lungs that helps breathing and separates the chest from the abdomen). - Stage IIIE: Cancer is found in lymph node groups above and below the diaphragm and outside the lymph nodes in a nearby organ or area. - Stage IIIS: Cancer is found in lymph node groups above and below the diaphragm, and in the spleen. - Stage IIIE+S: Cancer is found in lymph node groups above and below the diaphragm, outside the lymph nodes in a nearby organ or area, and in the spleen. Stage IV In stage IV adult non-Hodgkin lymphoma, the cancer: - is found throughout one or more organs that are not part of a lymphatic area (lymph node group, tonsils and nearby tissue, thymus, or spleen), and may be in lymph nodes near those organs; or - is found in one organ that is not part of a lymphatic area and has spread to organs or lymph nodes far away from that organ; or - is found in the liver, bone marrow, cerebrospinal fluid (CSF), or lungs (other than cancer that has spread to the lungs from nearby areas). Adult non-Hodgkin lymphomas may be grouped for treatment according to whether the cancer is indolent or aggressive and whether affected lymph nodes are next to each other in the body. See the General Information section for more information on the types of indolent (slow-growing) and aggressive (fast-growing) non-Hodgkin lymphoma. Non-Hodgkin lymphoma can also be described as contiguous or noncontiguous: - Contiguous lymphomas: Lymphomas in which the lymph nodes with cancer are next to each other. - Noncontiguous lymphomas: Lymphomas in which the lymph nodes with cancer are not next to each other, but are on the same side of the diaphragm.",Adult Non-Hodgkin Lymphoma,0000004_2,CancerGov,https://www.cancer.gov/types/lymphoma/patient/adult-nhl-treatment-pdq,C0019829,T191,Disorders What are the treatments for Adult Non-Hodgkin Lymphoma ?,0000004_2-8,treatment,"Key Points - There are different types of treatment for patients with non-Hodgkin lymphoma. - Patients with non-Hodgkin lymphoma should have their treatment planned by a team of health care providers who are experts in treating lymphomas. - Patients may develop late effects that appear months or years after their treatment for non-Hodgkin lymphoma. - Nine types of standard treatment are used: - Radiation therapy - Chemotherapy - Immunotherapy - Targeted therapy - Plasmapheresis - Watchful waiting - Antibiotic therapy - Surgery - Stem cell transplant - New types of treatment are being tested in clinical trials. - Vaccine therapy - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with non-Hodgkin lymphoma. Different types of treatment are available for patients with non-Hodgkin lymphoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. For pregnant women with non-Hodgkin lymphoma, treatment is carefully chosen to protect the baby. Treatment decisions are based on the mothers wishes, the stage of the non-Hodgkin lymphoma, and the age of the baby. The treatment plan may change as the signs and symptoms, cancer, and pregnancy change. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team. Patients with non-Hodgkin lymphoma should have their treatment planned by a team of health care providers who are experts in treating lymphomas. Treatment will be overseen by a medical oncologist, a doctor who specializes in treating cancer, or a hematologist, a doctor who specializes in treating blood cancers. The medical oncologist may refer you to other health care providers who have experience and are experts in treating adult non-Hodgkin lymphoma and who specialize in certain areas of medicine. These may include the following specialists: - Neurosurgeon. - Neurologist. - Radiation oncologist. - Endocrinologist. - Rehabilitation specialist. - Other oncology specialists. Patients may develop late effects that appear months or years after their treatment for non-Hodgkin lymphoma. Side effects from cancer treatment that begin during or after treatment and continue for months or years are called late effects. Treatment with chemotherapy, radiation therapy, or stem cell transplant for non-Hodgkin lymphoma may increase the risk of late effects. Late effects of cancer treatment may include the following: - Heart problems. - Infertility (inability to have children). - Loss of bone density. - Neuropathy (nerve damage that causes numbness or trouble walking). - A second cancer, such as: - Lung cancer. - Brain cancer. - Kidney cancer. - Bladder cancer. - Melanoma. - Hodgkin lymphoma. - Myelodysplastic syndrome. - Acute myeloid leukemia. Some late effects may be treated or controlled. It is important to talk with your doctor about the effects cancer treatment can have on you. Regular follow-up to check for late effects is important. Nine types of standard treatment are used: Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. Total-body irradiation is a type of external radiation therapy that is given to the entire body. It may be given before a stem cell transplant. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External radiation therapy is used to treat adult non-Hodgkin lymphoma, and may also be used as palliative therapy to relieve symptoms and improve quality of life. For pregnant women with non-Hodgkin lymphoma, radiation therapy should be given after delivery, if possible, to avoid any risk to the baby. If treatment is needed right away, pregnant women may decide to continue the pregnancy and receive radiation therapy. However, lead used to shield the baby may not protect it from scattered radiation that could possibly cause cancer in the future. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid (intrathecal chemotherapy), an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is treatment using two or more anticancer drugs. Steroid drugs may be added, to lessen inflammation and lower the body's immune response. The way the chemotherapy is given depends on the type and stage of the cancer being treated. Intrathecal chemotherapy may also be used in the treatment of lymphoma that first forms in the testicles or sinuses (hollow areas) around the nose, diffuse large B-cell lymphoma, Burkitt lymphoma, lymphoblastic lymphoma, and some aggressive T-cell lymphomas. It is given to lessen the chance that lymphoma cells will spread to the brain and spinal cord. This is called CNS prophylaxis. In pregnant women, the baby is exposed to chemotherapy when the mother is treated, and some anticancer drugs cause birth defects. Because anticancer drugs are passed to the baby through the mother, both must be watched closely when chemotherapy is given. See Drugs Approved for Non-Hodgkin Lymphoma for more information. Immunotherapy Immunotherapy is a treatment that uses the patients immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the bodys natural defenses against cancer. Immunomodulators are a type of immunotherapy. Lenalidomide is an immunomodulator used to treat adult non-Hodgkin lymphoma. See Drugs Approved for Non-Hodgkin Lymphoma for more information. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Monoclonal antibody therapy, proteasome inhibitor therapy, and kinase inhibitor therapy are types of targeted therapy used to treat adult non-Hodgkin lymphoma. Monoclonal antibody therapy is a cancer treatment that uses antibodies made in the laboratory from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Rituximab is a monoclonal antibody used to treat many types of non-Hodgkin lymphoma. Monoclonal antibodies that have been joined to radioactive material are called radiolabeled monoclonal antibodies. Yttrium Y 90-ibritumomab tiuxetan is an example of a radiolabeled monoclonal antibody. Monoclonal antibodies are given by infusion. Proteasome inhibitor therapy blocks the action of proteasomes in cancer cells and may prevent the growth of tumors. Kinase inhibitor therapy, such as idelalisib, blocks certain proteins, which may help keep lymphoma cells from growing and may kill them. It is used to treat indolent lymphoma. Ibrutinib, a type of Bruton's tyrosine kinase inhibitor therapy, is used to treat lymphoplasmacytic lymphoma and mantle cell lymphoma. See Drugs Approved for Non-Hodgkin Lymphoma for more information. Plasmapheresis If the blood becomes thick with extra antibody proteins and affects circulation, plasmapheresis is done to remove extra plasma and antibody proteins from the blood. In this procedure, blood is removed from the patient and sent through a machine that separates the plasma (the liquid part of the blood) from the blood cells. The patient's plasma contains the unneeded antibodies and is not returned to the patient. The normal blood cells are returned to the bloodstream along with donated plasma or a plasma replacement. Plasmapheresis does not keep new antibodies from forming. Watchful waiting Watchful waiting is closely monitoring a patients condition without giving any treatment until signs or symptoms appear or change. Antibiotic therapy Antibiotic therapy is a treatment that uses drugs to treat infections and cancer caused by bacteria and other microorganisms. See Drugs Approved for Non-Hodgkin Lymphoma for more information. Surgery Surgery may be used to remove the lymphoma in certain patients with indolent or aggressive non-Hodgkin lymphoma. The type of surgery used depends on where the lymphoma formed in the body: - Local excision for certain patients with mucosa-associated lymphoid tissue (MALT) lymphoma, PTLD, and small bowel T-cell lymphoma. - Splenectomy for patients with marginal zone lymphoma of the spleen. Patients who have a heart, lung, liver, kidney, or pancreas transplant usually need to take drugs to suppress their immune system for the rest of their lives. Long-term immunosuppression after an organ transplant can cause a certain type of non-Hodgkin lymphoma called post-transplant lymphoproliferative disorder (PLTD). Small bowel surgery is often needed to diagnose celiac disease in adults who develop a type of T-cell lymphoma. Stem cell transplant Stem cell transplant is a method of giving high doses of chemotherapy and/or total-body irradiation and then replacing blood-forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient (autologous transplant) or a donor (allogeneic transplant) and are frozen and stored. After the chemotherapy and/or radiation therapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the bodys blood cells. New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Vaccine therapy Vaccine therapy is a type of biologic therapy. Biologic therapy is a treatment that uses the patients immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the bodys natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy. Vaccine therapy can also be a type of targeted therapy. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Non-Hodgkin Lymphoma During Pregnancy Indolent Non-Hodgkin Lymphoma During Pregnancy Women who have indolent (slow-growing) non-Hodgkin lymphoma during pregnancy may be treated with watchful waiting until after they give birth. (See the Treatment Options for Indolent Non-Hodgkin Lymphoma section for more information.) Aggressive Non-Hodgkin Lymphoma During Pregnancy Treatment of aggressive non-Hodgkin lymphoma during pregnancy may include the following: - Treatment given right away based on the type of non-Hodgkin lymphoma to increase the mother's chance of survival. Treatment may include combination chemotherapy and rituximab. - Early delivery of the baby followed by treatment based on the type of non-Hodgkin lymphoma. - If in the first trimester of pregnancy, medical oncologists may advise ending the pregnancy so that treatment may begin. Treatment depends on the type of non-Hodgkin lymphoma.",Adult Non-Hodgkin Lymphoma,0000004_2,CancerGov,https://www.cancer.gov/types/lymphoma/patient/adult-nhl-treatment-pdq,C0019829,T191,Disorders What is (are) AIDS-Related Lymphoma ?,0000004_3-1,information,"Key Points - AIDS-related lymphoma is a disease in which malignant (cancer) cells form in the lymph system of patients who have acquired immunodeficiency syndrome (AIDS). - There are many different types of lymphoma. - Signs of AIDS-related lymphoma include weight loss, fever, and night sweats. - Tests that examine the lymph system and other parts of the body are used to help detect (find) and diagnose AIDS-related lymphoma. - Certain factors affect prognosis (chance of recovery) and treatment options. AIDS-related lymphoma is a disease in which malignant (cancer) cells form in the lymph system of patients who have acquired immunodeficiency syndrome (AIDS). AIDS is caused by the human immunodeficiency virus (HIV), which attacks and weakens the body's immune system. The immune system is then unable to fight infection and disease. People with HIV disease have an increased risk of infection and lymphoma or other types of cancer. A person with HIV disease who develops certain types of infections or cancer is then diagnosed with AIDS. Sometimes, people are diagnosed with AIDS and AIDS-related lymphoma at the same time. For information about AIDS and its treatment, please see the AIDSinfo website. AIDS-related lymphoma is a type of cancer that affects the lymph system, which is part of the body's immune system. The immune system protects the body from foreign substances, infection, and diseases. The lymph system is made up of the following: - Lymph: Colorless, watery fluid that carries white blood cells called lymphocytes through the lymph system. Lymphocytes protect the body against infections and the growth of tumors. - Lymph vessels: A network of thin tubes that collect lymph from different parts of the body and return it to the bloodstream. - Lymph nodes: Small, bean-shaped structures that filter lymph and store white blood cells that help fight infection and disease. Lymph nodes are located along the network of lymph vessels found throughout the body. Clusters of lymph nodes are found in the neck, underarm, abdomen, pelvis, and groin. - Spleen: An organ that makes lymphocytes, filters the blood, stores blood cells, and destroys old blood cells. The spleen is on the left side of the abdomen near the stomach. - Thymus: An organ in which lymphocytes grow and multiply. The thymus is in the chest behind the breastbone. - Tonsils: Two small masses of lymph tissue at the back of the throat. The tonsils make lymphocytes. - Bone marrow: The soft, spongy tissue in the center of large bones. Bone marrow makes white blood cells, red blood cells, and platelets. Lymph tissue is also found in other parts of the body such as the brain, stomach, thyroid gland, and skin. Sometimes AIDS-related lymphoma occurs outside the lymph nodes in the bone marrow, liver, meninges (thin membranes that cover the brain) and gastrointestinal tract. Less often, it may occur in the anus, heart, bile duct, gingiva, and muscles. There are many different types of lymphoma. Lymphomas are divided into two general types: - Hodgkin lymphoma. - Non-Hodgkin lymphoma. Both Hodgkin lymphoma and non-Hodgkin lymphoma may occur in patients with AIDS, but non-Hodgkin lymphoma is more common. When a person with AIDS has non-Hodgkin lymphoma, it is called AIDS-related lymphoma. When AIDS-related lymphoma occurs in the central nervous system (CNS), it is called AIDS-related primary CNS lymphoma. Non-Hodgkin lymphomas are grouped by the way their cells look under a microscope. They may be indolent (slow-growing) or aggressive (fast-growing). AIDS-related lymphomas are aggressive. There are two main types of AIDS-related non-Hodgkin lymphoma: - Diffuse large B-cell lymphoma (including B-cell immunoblastic lymphoma). - Burkitt or Burkitt-like lymphoma. For more information about lymphoma or AIDS-related cancers, see the following PDQ summaries: - Adult Non-Hodgkin Lymphoma Treatment - Childhood Non-Hodgkin Lymphoma Treatment - Primary CNS Lymphoma Treatment - Kaposi Sarcoma Treatment",AIDS-Related Lymphoma,0000004_3,CancerGov,https://www.cancer.gov/types/lymphoma/patient/aids-related-treatment-pdq,C0445223,T191,Disorders What are the symptoms of AIDS-Related Lymphoma ?,0000004_3-2,symptoms,"Signs of AIDS-related lymphoma include weight loss, fever, and night sweats. These and other signs and symptoms may be caused by AIDS-related lymphoma or by other conditions. Check with your doctor if you have any of the following: - Weight loss or fever for no known reason. - Night sweats. - Painless, swollen lymph nodes in the neck, chest, underarm, or groin. - A feeling of fullness below the ribs.",AIDS-Related Lymphoma,0000004_3,CancerGov,https://www.cancer.gov/types/lymphoma/patient/aids-related-treatment-pdq,C0445223,T191,Disorders How to diagnose AIDS-Related Lymphoma ?,0000004_3-3,exams and tests,"Tests that examine the lymph system and other parts of the body are used to help detect (find) and diagnose AIDS-related lymphoma. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following: - The number of red blood cells, white blood cells, and platelets. - The amount of hemoglobin (the protein that carries oxygen) in the red blood cells. - The portion of the sample made up of red blood cells. - HIV test : A test to measure the level of HIV antibodies in a sample of blood. Antibodies are made by the body when it is invaded by a foreign substance. A high level of HIV antibodies may mean the body has been infected with HIV. - Lymph node biopsy : The removal of all or part of a lymph node. A pathologist views the tissue under a microscope to look for cancer cells. One of the following types of biopsies may be done: - Excisional biopsy : The removal of an entire lymph node. - Incisional biopsy : The removal of part of a lymph node. - Core biopsy : The removal of tissue from a lymph node using a wide needle. - Fine-needle aspiration (FNA) biopsy : The removal of tissue from a lymph node using a thin needle. - Bone marrow aspiration and biopsy : The removal of bone marrow and a small piece of bone by inserting a hollow needle into the hipbone or breastbone. A pathologist views the bone marrow and bone under a microscope to look for signs of cancer. - Chest x-ray : An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.",AIDS-Related Lymphoma,0000004_3,CancerGov,https://www.cancer.gov/types/lymphoma/patient/aids-related-treatment-pdq,C0445223,T191,Disorders What is the outlook for AIDS-Related Lymphoma ?,0000004_3-4,outlook,Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - The stage of the cancer. - The age of the patient. - The number of CD4 lymphocytes (a type of white blood cell) in the blood. - The number of places in the body lymphoma is found outside the lymph system. - Whether the patient has a history of intravenous (IV) drug use. - The patient's ability to carry out regular daily activities.,AIDS-Related Lymphoma,0000004_3,CancerGov,https://www.cancer.gov/types/lymphoma/patient/aids-related-treatment-pdq,C0445223,T191,Disorders What are the stages of AIDS-Related Lymphoma ?,0000004_3-5,stages,"Key Points - After AIDS-related lymphoma has been diagnosed, tests are done to find out if cancer cells have spread within the lymph system or to other parts of the body. - There are three ways that cancer spreads in the body. - Stages of AIDS-related lymphoma may include E and S. - The following stages are used for AIDS-related lymphoma: - Stage I - Stage II - Stage III - Stage IV - For treatment, AIDS-related lymphomas are grouped based on where they started in the body, as follows: - Peripheral/systemic lymphoma - Primary CNS lymphoma After AIDS-related lymphoma has been diagnosed, tests are done to find out if cancer cells have spread within the lymph system or to other parts of the body. The process used to find out if cancer cells have spread within the lymph system or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment, but AIDS-related lymphoma is usually advanced when it is diagnosed. The following tests and procedures may be used in the staging process: - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. The blood sample will be checked for the level of LDH (lactate dehydrogenase). - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the lung, lymph nodes, and liver, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. - MRI (magnetic resonance imaging) with gadolinium : A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. A substance called gadolinium is injected into the patient through a vein. The gadolinium collects around the cancer cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Lumbar puncture : A procedure used to collect cerebrospinal fluid (CSF) from the spinal column. This is done by placing a needle between two bones in the spine and into the CSF around the spinal cord and removing a sample of the fluid. The sample of CSF is checked under a microscope for signs that the cancer has spread to the brain and spinal cord. The sample may also be checked for Epstein-Barr virus. This procedure is also called an LP or spinal tap. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Stages of AIDS-related lymphoma may include E and S. AIDS-related lymphoma may be described as follows: - E: ""E"" stands for extranodal and means the cancer is found in an area or organ other than the lymph nodes or has spread to tissues beyond, but near, the major lymphatic areas. - S: ""S"" stands for spleen and means the cancer is found in the spleen. The following stages are used for AIDS-related lymphoma: Stage I Stage I AIDS-related lymphoma is divided into stage I and stage IE. - Stage I: Cancer is found in one lymphatic area (lymph node group, tonsils and nearby tissue, thymus, or spleen). - Stage IE: Cancer is found in one organ or area outside the lymph nodes. Stage II Stage II AIDS-related lymphoma is divided into stage II and stage IIE. - Stage II: Cancer is found in two or more lymph node groups either above or below the diaphragm (the thin muscle below the lungs that helps breathing and separates the chest from the abdomen). - Stage IIE: Cancer is found in one or more lymph node groups either above or below the diaphragm. Cancer is also found outside the lymph nodes in one organ or area on the same side of the diaphragm as the affected lymph nodes. Stage III Stage III AIDS-related lymphoma is divided into stage III, stage IIIE, stage IIIS, and stage IIIE+S. - Stage III: Cancer is found in lymph node groups above and below the diaphragm (the thin muscle below the lungs that helps breathing and separates the chest from the abdomen). - Stage IIIE: Cancer is found in lymph node groups above and below the diaphragm and outside the lymph nodes in a nearby organ or area. - Stage IIIS: Cancer is found in lymph node groups above and below the diaphragm, and in the spleen. - Stage IIIE+S: Cancer is found in lymph node groups above and below the diaphragm, outside the lymph nodes in a nearby organ or area, and in the spleen. Stage IV In stage IV AIDS-related lymphoma, the cancer: - is found throughout one or more organs that are not part of a lymphatic area (lymph node group, tonsils and nearby tissue, thymus, or spleen) and may be in lymph nodes near those organs; or - is found in one organ that is not part of a lymphatic area and has spread to organs or lymph nodes far away from that organ; or - is found in the liver, bone marrow, cerebrospinal fluid (CSF), or lungs (other than cancer that has spread to the lungs from nearby areas). Patients who are infected with the Epstein-Barr virus or whose AIDS-related lymphoma affects the bone marrow have an increased risk of the cancer spreading to the central nervous system (CNS). For treatment, AIDS-related lymphomas are grouped based on where they started in the body, as follows: Peripheral/systemic lymphoma Lymphoma that starts in the lymph system or elsewhere in the body, other than the brain, is called peripheral/systemic lymphoma. It may spread throughout the body, including to the brain or bone marrow. It is often diagnosed in an advanced stage. Primary CNS lymphoma Primary CNS lymphoma starts in the central nervous system (brain and spinal cord). It is linked to the Epstein-Barr virus. Lymphoma that starts somewhere else in the body and spreads to the central nervous system is not primary CNS lymphoma.",AIDS-Related Lymphoma,0000004_3,CancerGov,https://www.cancer.gov/types/lymphoma/patient/aids-related-treatment-pdq,C0445223,T191,Disorders What are the treatments for AIDS-Related Lymphoma ?,0000004_3-6,treatment,"Key Points - There are different types of treatment for patients with AIDS-related lymphoma. - Treatment of AIDS-related lymphoma combines treatment of the lymphoma with treatment for AIDS. - Four types of standard treatment are used: - Chemotherapy - Radiation therapy - High-dose chemotherapy with stem cell transplant - Targeted therapy - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with AIDS-related lymphoma. Different types of treatment are available for patients with AIDS-related lymphoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Treatment of AIDS-related lymphoma combines treatment of the lymphoma with treatment for AIDS. Patients with AIDS have weakened immune systems and treatment can cause the immune system to become even weaker. For this reason, treating patients who have AIDS-related lymphoma is difficult and some patients may be treated with lower doses of drugs than lymphoma patients who do not have AIDS. Combined antiretroviral therapy (cART) is used to lessen the damage to the immune system caused by HIV. Treatment with combined antiretroviral therapy may allow some patients with AIDS-related lymphoma to safely receive anticancer drugs in standard or higher doses. In these patients, treatment may work as well as it does in lymphoma patients who do not have AIDS. Medicine to prevent and treat infections, which can be serious, is also used. For more information about AIDS and its treatment, please see the AIDSinfo website. Four types of standard treatment are used: Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid (intrathecal chemotherapy), an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is treatment using more than one anticancer drug. The way the chemotherapy is given depends on where the cancer has formed. Intrathecal chemotherapy may be used in patients who are more likely to have lymphoma in the central nervous system (CNS). Chemotherapy is used in the treatment of AIDS-related peripheral/systemic lymphoma. It is not yet known whether it is best to give combined antiretroviral therapy at the same time as chemotherapy or after chemotherapy ends. Colony-stimulating factors are sometimes given together with chemotherapy. This helps lessen the side effects chemotherapy may have on the bone marrow. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on where the cancer has formed. External radiation therapy is used to treat AIDS-related primary CNS lymphoma. High-dose chemotherapy with stem cell transplant High-dose chemotherapy with stem cell transplant is a way of giving high doses of chemotherapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Monoclonal antibody therapy is a type of targeted therapy. Monoclonal antibody therapy is a cancer treatment that uses antibodies made in the laboratory from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. These may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Rituximab is used in the treatment of AIDS-related peripheral/systemic lymphoma. New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for AIDS-Related Lymphoma AIDS-Related Peripheral/Systemic Lymphoma Treatment of AIDS-related peripheral/systemic lymphoma may include the following: - Combination chemotherapy with or without targeted therapy. - High-dose chemotherapy and stem cell transplant, for lymphoma that has not responded to treatment or has come back. - Intrathecal chemotherapy for lymphoma that is likely to spread to the central nervous system (CNS). Check the list of NCI-supported cancer clinical trials that are now accepting patients with AIDS-related peripheral/systemic lymphoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. AIDS-Related Primary Central Nervous System Lymphoma Treatment of AIDS-related primary central nervous system lymphoma may include the following: - External radiation therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with AIDS-related primary CNS lymphoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",AIDS-Related Lymphoma,0000004_3,CancerGov,https://www.cancer.gov/types/lymphoma/patient/aids-related-treatment-pdq,C0445223,T191,Disorders What is (are) Mycosis Fungoides and the Szary Syndrome ?,0000004_4-1,information,"Key Points - Mycosis fungoides and the Szary syndrome are diseases in which lymphocytes (a type of white blood cell) become malignant (cancerous) and affect the skin. - Mycosis fungoides and the Szary syndrome are types of cutaneous T-cell lymphoma. - A sign of mycosis fungoides is a red rash on the skin. - In the Szary syndrome, cancerous T-cells are found in the blood. - Tests that examine the skin and blood are used to detect (find) and diagnose mycosis fungoides and the Szary syndrome. - Certain factors affect prognosis (chance of recovery) and treatment options. Mycosis fungoides and the Szary syndrome are diseases in which lymphocytes (a type of white blood cell) become malignant (cancerous) and affect the skin. Normally, the bone marrow makes blood stem cells (immature cells) that become mature blood stem cells over time. A blood stem cell may become a myeloid stem cell or a lymphoid stem cell. A myeloid stem cell becomes a red blood cell, white blood cell, or platelet. A lymphoid stem cell becomes a lymphoblast and then one of three types of lymphocytes (white blood cells): - B-cell lymphocytes that make antibodies to help fight infection. - T-cell lymphocytes that help B-lymphocytes make the antibodies that help fight infection. - Natural killer cells that attack cancer cells and viruses. In mycosis fungoides, T-cell lymphocytes become cancerous and affect the skin. In the Szary syndrome, cancerous T-cell lymphocytes affect the skin and are in the blood. Mycosis fungoides and the Szary syndrome are types of cutaneous T-cell lymphoma. Mycosis fungoides and the Szary syndrome are the two most common types of cutaneous T-cell lymphoma (a type of non-Hodgkin lymphoma). For information about other types of skin cancer or non-Hodgkin lymphoma, see the following PDQ summaries: - Adult Non-Hodgkin Lymphoma Treatment - Skin Cancer Treatment - Melanoma Treatment - Kaposi Sarcoma Treatment In the Szary syndrome, cancerous T-cells are found in the blood. Also, skin all over the body is reddened, itchy, peeling, and painful. There may also be patches, plaques, or tumors on the skin. It is not known if the Szary syndrome is an advanced form of mycosis fungoides or a separate disease.",Mycosis Fungoides and the Szary Syndrome,0000004_4,CancerGov,https://www.cancer.gov/types/lymphoma/patient/mycosis-fungoides-treatment-pdq,C0039082,T047,Disorders What are the symptoms of Mycosis Fungoides and the Szary Syndrome ?,0000004_4-2,symptoms,"A sign of mycosis fungoides is a red rash on the skin. Mycosis fungoides may go through the following phases: - Premycotic phase: A scaly, red rash in areas of the body that usually are not exposed to the sun. This rash does not cause symptoms and may last for months or years. It is hard to diagnose the rash as mycosis fungoides during this phase. - Patch phase: Thin, reddened, eczema -like rash. - Plaque phase: Small raised bumps (papules) or hardened lesions on the skin, which may be reddened. - Tumor phase: Tumors form on the skin. These tumors may develop ulcers and the skin may get infected. Check with your doctor if you have any of these signs.",Mycosis Fungoides and the Szary Syndrome,0000004_4,CancerGov,https://www.cancer.gov/types/lymphoma/patient/mycosis-fungoides-treatment-pdq,C0039082,T047,Disorders How to diagnose Mycosis Fungoides and the Szary Syndrome ?,0000004_4-3,exams and tests,"Tests that examine the skin and blood are used to detect (find) and diagnose mycosis fungoides and the Szary syndrome. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps, the number and type of skin lesions, or anything else that seems unusual. Pictures of the skin and a history of the patients health habits and past illnesses and treatments will also be taken. - Complete blood count with differential : A procedure in which a sample of blood is drawn and checked for the following: - The number of red blood cells and platelets. - The number and type of white blood cells. - The amount of hemoglobin (the protein that carries oxygen) in the red blood cells. - The portion of the blood sample made up of red blood cells. - Peripheral blood smear : A procedure in which a sample of blood is viewed under a microscope to count different circulating blood cells (red blood cells, white blood cells, platelets, etc.) and see whether the cells look normal. - Skin biopsy : The removal of cells or tissues so they can be viewed under a microscope to check for signs of cancer. The doctor may remove a growth from the skin, which will be examined by a pathologist. More than one skin biopsy may be needed to diagnose mycosis fungoides. - Immunophenotyping : A process used to identify cells, based on the types of antigens or markers on the surface of the cell. This process may include special staining of the blood cells. It is used to diagnose specific types of leukemia and lymphoma by comparing the cancer cells to normal cells of the immune system. - T-cell receptor (TCR) gene rearrangement test: A laboratory test in which cells in a sample of tissue are checked to see if there is a certain change in the genes. This gene change can lead to too many of one kind of T-cells (white blood cells that fight infection) to be made. - Flow cytometry : A laboratory test that measures the number of cells in a sample of blood, the percentage of live cells in a sample, and certain characteristics of cells, such as size, shape, and the presence of tumor markers on the cell surface. The cells are stained with a light-sensitive dye, placed in a fluid, and passed in a stream before a laser or other type of light. The measurements are based on how the light-sensitive dye reacts to the light.",Mycosis Fungoides and the Szary Syndrome,0000004_4,CancerGov,https://www.cancer.gov/types/lymphoma/patient/mycosis-fungoides-treatment-pdq,C0039082,T047,Disorders What is the outlook for Mycosis Fungoides and the Szary Syndrome ?,0000004_4-4,outlook,"Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - The stage of the cancer. - The type of lesion (patches, plaques, or tumors). Mycosis fungoides and the Szary syndrome are hard to cure. Treatment is usually palliative, to relieve symptoms and improve the quality of life. Patients with early stage disease may live many years.",Mycosis Fungoides and the Szary Syndrome,0000004_4,CancerGov,https://www.cancer.gov/types/lymphoma/patient/mycosis-fungoides-treatment-pdq,C0039082,T047,Disorders what research (or clinical trials) is being done for Mycosis Fungoides and the Szary Syndrome ?,0000004_4-5,research,"Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Mycosis Fungoides and the Szary Syndrome,0000004_4,CancerGov,https://www.cancer.gov/types/lymphoma/patient/mycosis-fungoides-treatment-pdq,C0039082,T047,Disorders What are the stages of Mycosis Fungoides and the Szary Syndrome ?,0000004_4-6,stages,"Key Points - After mycosis fungoides and the Szary syndrome have been diagnosed, tests are done to find out if cancer cells have spread from the skin to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - The following stages are used for mycosis fungoides and the Szary syndrome: - Stage I Mycosis Fungoides - Stage II Mycosis Fungoides - Stage III Mycosis Fungoides - Stage IV Mycosis Fungoides - Stage IV Szary Syndrome After mycosis fungoides and the Szary syndrome have been diagnosed, tests are done to find out if cancer cells have spread from the skin to other parts of the body. The process used to find out if cancer has spread from the skin to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following procedures may be used in the staging process: - Chest x-ray : An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the lymph nodes, chest, abdomen, and pelvis, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. - Lymph node biopsy : The removal of all or part of a lymph node. A pathologist views the tissue under a microscope to look for cancer cells. - Bone marrow aspiration and biopsy : The removal of bone marrow and a small piece of bone by inserting a hollow needle into the hipbone or breastbone. A pathologist views the bone marrow and bone under a microscope to look for signs of cancer. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if mycosis fungoides spreads to the liver, the cancer cells in the liver are actually mycosis fungoides cells. The disease is metastatic mycosis fungoides, not liver cancer. The following stages are used for mycosis fungoides and the Szary syndrome: Stage I Mycosis Fungoides Stage I is divided into stage IA and stage IB as follows: - Stage IA: Less than 10% of the skin surface is covered with patches, papules, and/or plaques. - Stage IB: Ten percent or more of the skin surface is covered with patches, papules, and/or plaques. There may be abnormal lymphocytes in the blood but they are not cancerous. Stage II Mycosis Fungoides Stage II is divided into stage IIA and stage IIB as follows: - Stage IIA: Any amount of the skin surface is covered with patches, papules, and/or plaques. Lymph nodes are enlarged but cancer has not spread to them. - Stage IIB: One or more tumors that are 1 centimeter or larger are found on the skin. Lymph nodes may be enlarged but cancer has not spread to them. There may be abnormal lymphocytes in the blood but they are not cancerous. Stage III Mycosis Fungoides In stage III, nearly all of the skin is reddened and may have patches, papules, plaques, or tumors. Lymph nodes may be enlarged but cancer has not spread to them. There may be abnormal lymphocytes in the blood but they are not cancerous. Stage IV Mycosis Fungoides Stage IV is divided into stage IVA and stage IVB as follows: - Stage IVA: Most of the skin is reddened and any amount of the skin surface is covered with patches, papules, plaques, or tumors, and either: - cancer has spread to lymph nodes and there may be cancerous lymphocytes in the blood; or - there are cancerous lymphocytes in the blood and lymph nodes may be enlarged, but cancer has not spread to them. - Stage IVB: Most of the skin is reddened and any amount of the skin surface is covered with patches, papules, plaques, or tumors. Cancer has spread to other organs in the body. Lymph nodes may be enlarged and cancer may have spread to them. There may be cancerous lymphocytes in the blood. Stage IV Szary Syndrome In stage IV: - Most of the skin is reddened and covered with patches, papules, plaques, or tumors; and - There is a high level of cancerous lymphocytes in the blood; and - Lymph nodes may be enlarged and cancer may have spread to them.",Mycosis Fungoides and the Szary Syndrome,0000004_4,CancerGov,https://www.cancer.gov/types/lymphoma/patient/mycosis-fungoides-treatment-pdq,C0039082,T047,Disorders What are the treatments for Mycosis Fungoides and the Szary Syndrome ?,0000004_4-7,treatment,"Key Points - There are different types of treatment for patients with mycosis fungoides and the Szary syndrome cancer. - Six types of standard treatment are used: - Photodynamic therapy - Radiation therapy - Chemotherapy - Other drug therapy - Biologic therapy - Targeted therapy - New types of treatment are being tested in clinical trials. - High-dose chemotherapy and radiation therapy with stem cell transplant - Treatment for mycosis fungoides and the Szary syndrome may cause side effects. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with mycosis fungoides and the Szary syndrome cancer. Different types of treatment are available for patients with mycosis fungoides and the Szary syndrome. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Six types of standard treatment are used: Photodynamic therapy Photodynamic therapy is a cancer treatment that uses a drug and a certain type of laser light to kill cancer cells. A drug that is not active until it is exposed to light is injected into a vein. The drug collects more in cancer cells than in normal cells. For skin cancer, laser light is shined onto the skin and the drug becomes active and kills the cancer cells. Photodynamic therapy causes little damage to healthy tissue. Patients undergoing photodynamic therapy will need to limit the amount of time spent in sunlight. In one type of photodynamic therapy, called psoralen and ultraviolet A (PUVA) therapy, the patient receives a drug called psoralen and then ultraviolet radiation is directed to the skin. In another type of photodynamic therapy, called extracorporeal photochemotherapy, the patient is given drugs and then some blood cells are taken from the body, put under a special ultraviolet A light, and put back into the body. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External radiation therapy is used to treat mycosis fungoides and the Szary syndrome, and may also be used as palliative therapy to relieve symptoms and improve quality of life. Sometimes, total skin electron beam (TSEB) radiation therapy is used to treat mycosis fungoides and the Szary syndrome. This is a type of external radiation treatment in which a radiation therapy machine aims electrons (tiny, invisible particles) at the skin covering the whole body. Ultraviolet B (UVB) radiation therapy uses a special lamp or laser that directs UVB radiation at the skin. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Sometimes the chemotherapy is topical (put on the skin in a cream, lotion, or ointment). The way the chemotherapy is given depends on the type and stage of the cancer being treated. See Drugs Approved for Non-Hodgkin Lymphoma for more information. (Mycosis fungoides and the Szary syndrome are types of non-Hodgkin lymphoma.) Other drug therapy Topical corticosteroids are used to relieve red, swollen, and inflamed skin. They are a type of steroid. Topical corticosteroids may be in a cream, lotion, or ointment. Retinoids, such as bexarotene, are drugs related to vitamin A that can slow the growth of certain types of cancer cells. The retinoids may be taken by mouth or put on the skin. See Drugs Approved for Non-Hodgkin Lymphoma for more information. (Mycosis fungoides and the Szary syndrome are types of non-Hodgkin lymphoma.) Biologic therapy Biologic therapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy. Interferon is a type of biologic therapy used to treat mycosis fungoides and the Szary syndrome. It interferes with the division of cancer cells and can slow tumor growth. See Drugs Approved for Non-Hodgkin Lymphoma for more information. (Mycosis fungoides and the Szary syndrome are types of non-Hodgkin lymphoma.) Targeted therapy Targeted therapy is a treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Monoclonal antibody therapy and other types of targeted therapies are used to treat mycosis fungoides and the Szary syndrome. Alemtuzumab is a monoclonal antibody used to treat mycosis fungoides and the Szary syndrome. Monoclonal antibody therapy uses antibodies made in the laboratory, from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Other types of monoclonal antibody therapy are being studied in clinical trials to treat mycosis fungoides and the Szary syndrome. Vorinostat and romidepsin are two of the histone deacetylase (HDAC) inhibitors used to treat mycosis fungoides and the Szary syndrome. HDAC inhibitors cause a chemical change that stops tumor cells from dividing. Pralatrexate is a dihydrofolate reductase (DHFR) inhibitor used to treat mycosis fungoides and the Szary syndrome. It builds up in cancer cells and stops them from using folate, a nutrient needed for cells to divide. Pralatrexate may slow the growth of tumors and kill cancer cells. See Drugs Approved for Non-Hodgkin Lymphoma for more information. (Mycosis fungoides and the Szary syndrome are types of non-Hodgkin lymphoma.) New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. High-dose chemotherapy and radiation therapy with stem cell transplant This treatment is a method of giving high doses of chemotherapy and radiation therapy and replacing blood-forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the bone marrow or blood of the patient or a donor and are frozen and stored. After therapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. Treatment for mycosis fungoides and the Szary syndrome may cause side effects. For information about side effects caused by treatment for cancer, see our Side Effects page. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options by Stage Stage I Mycosis Fungoides Treatment of stage I mycosis fungoides may include the following: - PUVA therapy. Biologic therapy may also be given. - Radiation therapy. In some cases, radiation therapy is given to skin lesions, as palliative therapy to reduce tumor size or relieve symptoms and improve quality of life. - Topical corticosteroid therapy. - Retinoid therapy. - Topical or systemic chemotherapy. - Biologic therapy. Topical chemotherapy may also be given. - Targeted therapy. - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I mycosis fungoides/Sezary syndrome. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage II Mycosis Fungoides Treatment of stage II mycosis fungoides is palliative (to relieve symptoms and improve the quality of life) and may include the following: - PUVA therapy. Biologic therapy may also be given. - Radiation therapy. - Topical corticosteroid therapy. - Retinoid therapy. - Topical or systemic chemotherapy. - Biologic therapy. Topical chemotherapy may also be given. - Targeted therapy. - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage II mycosis fungoides/Sezary syndrome. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage III Mycosis Fungoides Treatment of stage III mycosis fungoides is palliative (to relieve symptoms and improve the quality of life) and may include the following: - PUVA therapy. Systemic chemotherapy or biologic therapy may also be given. - Extracorporeal photochemotherapy. - Radiation therapy. - Topical corticosteroid therapy. - Retinoid therapy. - Systemic chemotherapy with one or more drugs. Topical chemotherapy or radiation therapy may also be given. - Topical chemotherapy. - Biologic therapy. Topical chemotherapy may also be given. - Targeted therapy. - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III mycosis fungoides/Sezary syndrome. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage IV Mycosis Fungoides and the Szary Syndrome Treatment of stage IV mycosis fungoides and stage IV Szary syndrome is palliative (to relieve symptoms and improve the quality of life) and may include the following: - PUVA therapy. Systemic chemotherapy or biologic therapy may also be given. - Extracorporeal photochemotherapy. Radiation therapy may also be given. - Radiation therapy. - Topical corticosteroid therapy. - Retinoid therapy. - Systemic chemotherapy with one or more drugs, or topical chemotherapy. - Biologic therapy. Topical chemotherapy may also be given. - Targeted therapy. - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage IV mycosis fungoides/Sezary syndrome. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Mycosis Fungoides and the Szary Syndrome,0000004_4,CancerGov,https://www.cancer.gov/types/lymphoma/patient/mycosis-fungoides-treatment-pdq,C0039082,T047,Disorders What is (are) Primary CNS Lymphoma ?,0000004_5-1,information,"Key Points - Primary central nervous system (CNS) lymphoma is a disease in which malignant (cancer) cells form in the lymph tissue of the brain and/or spinal cord. - Having a weakened immune system may increase the risk of developing primary CNS lymphoma. - Tests that examine the eyes, brain, and spinal cord are used to detect (find) and diagnose primary CNS lymphoma. - Certain factors affect prognosis (chance of recovery) and treatment options. Primary central nervous system (CNS) lymphoma is a disease in which malignant (cancer) cells form in the lymph tissue of the brain and/or spinal cord. Lymphoma is a disease in which malignant (cancer) cells form in the lymph system. The lymph system is part of the immune system and is made up of the lymph, lymph vessels, lymph nodes, spleen, thymus, tonsils, and bone marrow. Lymphocytes (carried in the lymph) travel in and out of the central nervous system (CNS). It is thought that some of these lymphocytes become malignant and cause lymphoma to form in the CNS. Primary CNS lymphoma can start in the brain, spinal cord, or meninges (the layers that form the outer covering of the brain). Because the eye is so close to the brain, primary CNS lymphoma can also start in the eye (called ocular lymphoma).",Primary CNS Lymphoma,0000004_5,CancerGov,https://www.cancer.gov/types/lymphoma/patient/primary-cns-lymphoma-treatment-pdq,C0280803,T191,Disorders Who is at risk for Primary CNS Lymphoma? ?,0000004_5-2,susceptibility,"Having a weakened immune system may increase the risk of developing primary CNS lymphoma. Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesnt mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Primary CNS lymphoma may occur in patients who have acquired immunodeficiency syndrome (AIDS) or other disorders of the immune system or who have had a kidney transplant. For more information about lymphoma in patients with AIDS, see the PDQ summary on AIDS-Related Lymphoma Treatment.",Primary CNS Lymphoma,0000004_5,CancerGov,https://www.cancer.gov/types/lymphoma/patient/primary-cns-lymphoma-treatment-pdq,C0280803,T191,Disorders How to diagnose Primary CNS Lymphoma ?,0000004_5-3,exams and tests,"Tests that examine the eyes, brain, and spinal cord are used to detect (find) and diagnose primary CNS lymphoma. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Neurological exam : A series of questions and tests to check the brain, spinal cord, and nerve function. The exam checks a persons mental status, coordination, ability to walk normally, and how well the muscles, senses, and reflexes work. This may also be called a neuro exam or a neurologic exam. - Slit-lamp eye exam : An exam that uses a special microscope with a bright, narrow slit of light to check the outside and inside of the eye. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the brain and spinal cord. A substance called gadolinium is injected into the patient through a vein. The gadolinium collects around the cancer cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI). - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. - Lumbar puncture : A procedure used to collect cerebrospinal fluid (CSF) from the spinal column. This is done by placing a needle between two bones in the spine and into the CSF around the spinal cord and removing a sample of the fluid. The sample of CSF is checked under a microscope for signs of tumor cells. The sample may also be checked for the amounts of protein and glucose. A higher than normal amount of protein or lower than normal amount of glucose may be a sign of a tumor. This procedure is also called an LP or spinal tap. - Stereotactic biopsy : A biopsy procedure that uses a computer and a 3-dimensional (3-D) scanning device to find a tumor site and guide the removal of tissue so it can be viewed under a microscope to check for signs of cancer. The following tests may be done on the samples of tissue that are removed: - Flow cytometry : A laboratory test that measures the number of cells in a sample, the percentage of live cells in a sample, and certain characteristics of cells, such as size, shape, and the presence of tumor markers on the cell surface. The cells are stained with a light-sensitive dye, placed in a fluid, and passed in a stream before a laser or other type of light. The measurements are based on how the light-sensitive dye reacts to the light. - Immunohistochemistry : A test that uses antibodies to check for certain antigens in a sample of tissue. The antibody is usually linked to a radioactive substance or a dye that causes the tissue to light up under a microscope. This type of test may be used to tell the difference between different types of cancer. - Cytogenetic analysis: A laboratory test in which cells in a sample of tissue are viewed under a microscope to look for certain changes in the chromosomes. Other tests, such as fluorescence in situ hybridization (FISH), may also be done to look for certain changes in the chromosomes. - Complete blood count (CBC) with differential : A procedure in which a sample of blood is drawn and checked for the following: - The number of red blood cells and platelets. - The number and type of white blood cells. - The amount of hemoglobin (the protein that carries oxygen) in the red blood cells. - The portion of the blood sample made up of red blood cells. - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease.",Primary CNS Lymphoma,0000004_5,CancerGov,https://www.cancer.gov/types/lymphoma/patient/primary-cns-lymphoma-treatment-pdq,C0280803,T191,Disorders What is the outlook for Primary CNS Lymphoma ?,0000004_5-4,outlook,"Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) depends on the following: - The patient's age and general health. - The level of certain substances in the blood and cerebrospinal fluid (CSF). - Where the tumor is in the central nervous system, eye, or both. - Whether the patient has AIDS. Treatment options depend on the following: - The stage of the cancer. - Where the tumor is in the central nervous system. - The patient's age and general health. - Whether the cancer has just been diagnosed or has recurred (come back). Treatment of primary CNS lymphoma works best when the tumor has not spread outside the cerebrum (the largest part of the brain) and the patient is younger than 60 years, able to carry out most daily activities, and does not have AIDS or other diseases that weaken the immune system.",Primary CNS Lymphoma,0000004_5,CancerGov,https://www.cancer.gov/types/lymphoma/patient/primary-cns-lymphoma-treatment-pdq,C0280803,T191,Disorders what research (or clinical trials) is being done for Primary CNS Lymphoma ?,0000004_5-6,research,"New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. High-dose chemotherapy with stem cell transplant High-dose chemotherapy with stem cell transplant is a method of giving high doses of chemotherapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to attack cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. Monoclonal antibody therapy is one type of targeted therapy being studied in the treatment of primary CNS lymphoma. Monoclonal antibody therapy is a cancer treatment that uses antibodies made in the laboratory from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Rituximab is a type of monoclonal antibody used to treat newly diagnosed primary CNS lymphoma in patients who do not have AIDS. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Primary CNS Lymphoma,0000004_5,CancerGov,https://www.cancer.gov/types/lymphoma/patient/primary-cns-lymphoma-treatment-pdq,C0280803,T191,Disorders What are the treatments for Primary CNS Lymphoma ?,0000004_5-7,treatment,"Key Points - There are different types of treatment for patients with primary CNS lymphoma. - Three standard treatments are used: - Radiation therapy - Chemotherapy - Steroid therapy - New types of treatment are being tested in clinical trials. - High-dose chemotherapy with stem cell transplant - Targeted therapy - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with primary CNS lymphoma. Different types of treatment are available for patients with primary central nervous system (CNS) lymphoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Surgery is not used to treat primary CNS lymphoma. Three standard treatments are used: Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. Because primary CNS lymphoma spreads throughout the brain, external radiation therapy is given to the whole brain. This is called whole brain radiation therapy. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on whether the patient has primary CNS lymphoma and AIDS. External radiation therapy is used to treat primary CNS lymphoma. High-dose radiation therapy to the brain can damage healthy tissue and cause disorders that can affect thinking, learning, problem solving, speech, reading, writing, and memory. Clinical trials have tested the use of chemotherapy alone or before radiation therapy to reduce the damage to healthy brain tissue that occurs with the use of radiation therapy. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid (intrathecal chemotherapy), an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on where the tumor is in the CNS or eye. Primary CNS lymphoma may be treated with systemic chemotherapy, intrathecal chemotherapy and/or intraventricular chemotherapy, in which anticancer drugs are placed into the ventricles (fluid -filled cavities) of the brain. If primary CNS lymphoma is found in the eye, anticancer drugs are injected directly into the vitreous humor (jelly-like substance) inside the eye. A network of blood vessels and tissue, called the blood-brain barrier, protects the brain from harmful substances. This barrier can also keep anticancer drugs from reaching the brain. In order to treat CNS lymphoma, certain drugs may be used to make openings between cells in the blood-brain barrier. This is called blood-brain barrier disruption. Anticancer drugs infused into the bloodstream may then reach the brain. Steroid therapy Steroids are hormones made naturally in the body. They can also be made in a laboratory and used as drugs. Glucocorticoids are steroid drugs that have an anticancer effect in lymphomas. New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. High-dose chemotherapy with stem cell transplant High-dose chemotherapy with stem cell transplant is a method of giving high doses of chemotherapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to attack cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. Monoclonal antibody therapy is one type of targeted therapy being studied in the treatment of primary CNS lymphoma. Monoclonal antibody therapy is a cancer treatment that uses antibodies made in the laboratory from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Rituximab is a type of monoclonal antibody used to treat newly diagnosed primary CNS lymphoma in patients who do not have AIDS. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Primary CNS Lymphoma Primary CNS Lymphoma Not Related to AIDS Treatment of primary central nervous system (CNS) lymphoma in patients who do not have AIDS may include the following: - Whole brain radiation therapy. - Chemotherapy. - Chemotherapy followed by radiation therapy. - Chemotherapy and targeted therapy (rituximab) followed by high-dose chemotherapy and stem cell transplant. - A clinical trial of high-dose chemotherapy with stem cell transplant. - A clinical trial of high-dose chemotherapy and targeted therapy (rituximab), with or without stem cell transplant or whole brain radiation therapy. Primary CNS Lymphoma Related to AIDS Treatment of primary central nervous system (CNS) lymphoma in patients who do have AIDS may include the following: - Whole brain radiation therapy. - Chemotherapy followed by radiation therapy. Treatment of primary CNS lymphoma is different in patients with AIDS because the treatment side effects may be more severe. (See the PDQ summary on AIDS-Related Lymphoma Treatment for more information). Primary Intraocular Lymphoma Treatment of primary intraocular lymphoma may include the following: - Chemotherapy (intraocular or systemic). - Whole brain radiation therapy. Recurrent Primary CNS Lymphoma Treatment of recurrent primary central nervous system (CNS) lymphoma may include the following: - Chemotherapy. - Radiation therapy (if not received in earlier treatment). - A clinical trial of a new drug or treatment schedule.",Primary CNS Lymphoma,0000004_5,CancerGov,https://www.cancer.gov/types/lymphoma/patient/primary-cns-lymphoma-treatment-pdq,C0280803,T191,Disorders What is (are) Childhood Hodgkin Lymphoma ?,0000004_6-1,information,"Key Points - Childhood Hodgkin lymphoma is a disease in which malignant (cancer) cells form in the lymph system. - There are two types of childhood Hodgkin lymphoma. - Epstein-Barr virus infection increases the risk of childhood Hodgkin lymphoma. - Signs of childhood Hodgkin lymphoma include swollen lymph nodes, fever, night sweats, and weight loss. - Tests that examine the lymph system are used to detect (find) and diagnose childhood Hodgkin lymphoma. - Certain factors affect prognosis (chance of recovery) and treatment options. Childhood Hodgkin lymphoma is a disease in which malignant (cancer) cells form in the lymph system. Childhood Hodgkin lymphoma is a type of cancer that develops in the lymph system, which is part of the body's immune system. The immune system protects the body from foreign substances, infection, and diseases. The lymph system is made up of the following: - Lymph: Colorless, watery fluid that carries white blood cells called lymphocytes through the lymph system. Lymphocytes protect the body against infections and the growth of tumors. - Lymph vessels: A network of thin tubes that collect lymph from different parts of the body and return it to the bloodstream. - Lymph nodes: Small, bean-shaped structures that filter lymph and store white blood cells that help fight infection and disease. Lymph nodes are located along the network of lymph vessels found throughout the body. Clusters of lymph nodes are found in the neck, underarm, abdomen, pelvis, and groin. - Spleen: An organ that makes lymphocytes, filters the blood, stores blood cells, and destroys old blood cells. The spleen is on the left side of the abdomen near the stomach. - Thymus: An organ in which lymphocytes grow and multiply. The thymus is in the chest behind the breastbone. - Tonsils: Two small masses of lymph tissue at the back of the throat. The tonsils make lymphocytes. - Bone marrow: The soft, spongy tissue in the center of large bones. Bone marrow makes white blood cells, red blood cells, and platelets. Lymph tissue is also found in other parts of the body such as the stomach, thyroid gland, brain, and skin. There are two general types of lymphoma: Hodgkin lymphoma and non-Hodgkin lymphoma. (See the PDQ summary on Childhood Non-Hodgkin Lymphoma Treatment for more information.) Hodgkin lymphoma often occurs in adolescents 15 to 19 years of age. The treatment for children and adolescents is different than treatment for adults. (See the PDQ summary on Adult Hodgkin Lymphoma Treatment for more information.) There are two types of childhood Hodgkin lymphoma. The two types of childhood Hodgkin lymphoma are: - Classical Hodgkin lymphoma. - Nodular lymphocyte-predominant Hodgkin lymphoma. Classical Hodgkin lymphoma is divided into four subtypes, based on how the cancer cells look under a microscope: - Lymphocyte-rich classical Hodgkin lymphoma. - Nodular sclerosis Hodgkin lymphoma. - Mixed cellularity Hodgkin lymphoma. - Lymphocyte-depleted Hodgkin lymphoma.",Childhood Hodgkin Lymphoma,0000004_6,CancerGov,https://www.cancer.gov/types/lymphoma/patient/child-hodgkin-treatment-pdq,C0220644,T191,Disorders Who is at risk for Childhood Hodgkin Lymphoma? ?,0000004_6-2,susceptibility,"Epstein-Barr virus infection increases the risk of childhood Hodgkin lymphoma. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesnt mean that you will not get cancer. Talk with your childs doctor if you think your child may be at risk. Risk factors for childhood Hodgkin lymphoma include the following: - Being infected with the Epstein-Barr virus. - Being infected with the human immunodeficiency virus (HIV). - Having certain diseases of the immune system. - Having a personal history of mononucleosis (""mono""). - Having a parent or sibling with a personal history of Hodgkin lymphoma. Being exposed to common infections in early childhood may decrease the risk of Hodgkin lymphoma in children because of the effect it has on the immune system.",Childhood Hodgkin Lymphoma,0000004_6,CancerGov,https://www.cancer.gov/types/lymphoma/patient/child-hodgkin-treatment-pdq,C0220644,T191,Disorders What are the symptoms of Childhood Hodgkin Lymphoma ?,0000004_6-3,symptoms,"Signs of childhood Hodgkin lymphoma include swollen lymph nodes, fever, night sweats, and weight loss. These and other signs and symptoms may be caused by childhood Hodgkin lymphoma or by other conditions. Check with your child's doctor if your child has any of the following: - Painless, swollen lymph nodes near the collarbone or in the neck, chest, underarm, or groin. - Fever for no known reason. - Weight loss for no known reason. - Night sweats. - Fatigue. - Anorexia. - Itchy skin. - Pain in the lymph nodes after drinking alcohol. Fever, weight loss, and night sweats are called B symptoms.",Childhood Hodgkin Lymphoma,0000004_6,CancerGov,https://www.cancer.gov/types/lymphoma/patient/child-hodgkin-treatment-pdq,C0220644,T191,Disorders How to diagnose Childhood Hodgkin Lymphoma ?,0000004_6-4,exams and tests,"Tests that examine the lymph system are used to detect (find) and diagnose childhood Hodgkin lymphoma. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient's health habits and past illnesses and treatments will also be taken. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the neck, chest, abdomen, or pelvis, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. Sometimes a PET scan and a CT scan are done at the same time. If there is any cancer, this increases the chance that it will be found. - Chest x-ray : An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. - Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following: - The number of red blood cells, white blood cells, and platelets. - The amount of hemoglobin (the protein that carries oxygen) in the red blood cells. - The portion of the blood sample made up of red blood cells. - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. - Sedimentation rate : A procedure in which a sample of blood is drawn and checked for the rate at which the red blood cells settle to the bottom of the test tube. The sedimentation rate is a measure of how much inflammation is in the body. A higher than normal sedimentation rate may be a sign of lymphoma. Also called erythrocyte sedimentation rate, sed rate, or ESR. - Lymph node biopsy : The removal of all or part of a lymph node. The lymph node may be removed during an image-guided CT scan or a thoracoscopy, mediastinoscopy, or laparoscopy. One of the following types of biopsies may be done: - Excisional biopsy : The removal of an entire lymph node. - Incisional biopsy : The removal of part of a lymph node. - Core biopsy : The removal of tissue from a lymph node using a wide needle. - Fine-needle aspiration (FNA) biopsy : The removal of tissue from a lymph node using a thin needle. A pathologist views the tissue under a microscope to look for cancer cells, especially Reed-Sternberg cells. Reed-Sternberg cells are common in classical Hodgkin lymphoma. The following test may be done on tissue that was removed: - Immunophenotyping : A laboratory test used to identify cells, based on the types of antigens or markers on the surface of the cell. This test is used to diagnose the specific type of lymphoma by comparing the cancer cells to normal cells of the immune system.",Childhood Hodgkin Lymphoma,0000004_6,CancerGov,https://www.cancer.gov/types/lymphoma/patient/child-hodgkin-treatment-pdq,C0220644,T191,Disorders What is the outlook for Childhood Hodgkin Lymphoma ?,0000004_6-5,outlook,Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - The stage of the cancer. - The size of the tumor. - Whether there are B symptoms at diagnosis. - The type of Hodgkin lymphoma. - Certain features of the cancer cells. - Whether there are too many white blood cells or too few red blood cells at the time of diagnosis. - How well the tumor responds to initial treatment with chemotherapy. - Whether the cancer is newly diagnosed or has recurred (come back). The treatment options also depend on: - The child's age and gender. - The risk of long-term side effects. Most children and adolescents with newly diagnosed Hodgkin lymphoma can be cured.,Childhood Hodgkin Lymphoma,0000004_6,CancerGov,https://www.cancer.gov/types/lymphoma/patient/child-hodgkin-treatment-pdq,C0220644,T191,Disorders What are the stages of Childhood Hodgkin Lymphoma ?,0000004_6-6,stages,"Key Points - After childhood Hodgkin lymphoma has been diagnosed, tests are done to find out if cancer cells have spread within the lymph system or to other parts of the body. - There are three ways that cancer spreads in the body. - Stages of childhood Hodgkin lymphoma may include A, B, E, and S. - The following stages are used for childhood Hodgkin lymphoma: - Stage I - Stage II - Stage III - Stage IV - Untreated Hodgkin lymphoma is divided into risk groups. After childhood Hodgkin lymphoma has been diagnosed, tests are done to find out if cancer cells have spread within the lymph system or to other parts of the body. The process used to find out if cancer has spread within the lymph system or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. Treatment is based on the stage and other factors that affect prognosis. The following tests and procedures may be used in the staging process: - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the neck, chest, abdomen, or pelvis, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. Sometimes a PET scan and a CT scan are done at the same time. If there is any cancer, this increases the chance that it will be found. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). An MRI of the abdomen and pelvis may be done. - Bone marrow aspiration and biopsy : The removal of bone marrow and a small piece of bone by inserting a hollow needle into the hipbone or breastbone. A pathologist views the bone marrow and bone under a microscope to look for abnormal cells. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Stages of childhood Hodgkin lymphoma may include A, B, E, and S. Childhood Hodgkin lymphoma may be described as follows: - A: The patient does not have B symptoms (fever, weight loss, or night sweats). - B: The patient has B symptoms. - E: Cancer is found in an organ or tissue that is not part of the lymph system but which may be next to an area of the lymph system affected by the cancer. - S: Cancer is found in the spleen. The following stages are used for childhood Hodgkin lymphoma: Stage I Stage I is divided into stage I and stage IE. - Stage I: Cancer is found in one of the following places in the lymph system: - One or more lymph nodes in one lymph node group. - Waldeyer's ring. - Thymus. - Spleen. - Stage IE: Cancer is found outside the lymph system in one organ or area. Stage II Stage II is divided into stage II and stage IIE. - Stage II: Cancer is found in two or more lymph node groups either above or below the diaphragm (the thin muscle below the lungs that helps breathing and separates the chest from the abdomen). - Stage IIE: Cancer is found in one or more lymph node groups either above or below the diaphragm and outside the lymph nodes in a nearby organ or area. Stage III Stage III is divided into stage III, stage IIIE, stage IIIS, and stage IIIE,S. - Stage III: Cancer is found in lymph node groups above and below the diaphragm (the thin muscle below the lungs that helps breathing and separates the chest from the abdomen). - Stage IIIE: Cancer is found in lymph node groups above and below the diaphragm and outside the lymph nodes in a nearby organ or area. - Stage IIIS: Cancer is found in lymph node groups above and below the diaphragm, and in the spleen. - Stage IIIE,S: Cancer is found in lymph node groups above and below the diaphragm, outside the lymph nodes in a nearby organ or area, and in the spleen. Stage IV In stage IV, the cancer: - is found outside the lymph nodes throughout one or more organs, and may be in lymph nodes near those organs; or - is found outside the lymph nodes in one organ and has spread to areas far away from that organ; or - is found in the lung, liver, bone marrow, or cerebrospinal fluid (CSF). The cancer has not spread to the lung, liver, bone marrow, or CSF from nearby areas. Untreated Hodgkin lymphoma is divided into risk groups. Untreated childhood Hodgkin lymphoma is divided into risk groups based on the stage, size of the tumor, and whether the patient has B symptoms (fever, weight loss, or night sweats). The risk group is used to plan treatment. - Low-risk childhood Hodgkin lymphoma. - Intermediate-risk childhood Hodgkin lymphoma. - High-risk childhood Hodgkin lymphoma.",Childhood Hodgkin Lymphoma,0000004_6,CancerGov,https://www.cancer.gov/types/lymphoma/patient/child-hodgkin-treatment-pdq,C0220644,T191,Disorders what research (or clinical trials) is being done for Childhood Hodgkin Lymphoma ?,0000004_6-7,research,"New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Proton beam radiation therapy Proton-beam therapy is a type of high-energy, external radiation therapy that uses streams of protons (small, positively-charged particles of matter) to make radiation. This type of radiation therapy may help lessen the damage to healthy tissue near the tumor. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Childhood Hodgkin Lymphoma,0000004_6,CancerGov,https://www.cancer.gov/types/lymphoma/patient/child-hodgkin-treatment-pdq,C0220644,T191,Disorders What are the treatments for Childhood Hodgkin Lymphoma ?,0000004_6-8,treatment,"Key Points - There are different types of treatment for children with Hodgkin lymphoma. - Children with Hodgkin lymphoma should have their treatment planned by a team of health care providers who are experts in treating childhood cancer. - Children and adolescents may have treatment-related side effects that appear months or years after treatment for Hodgkin lymphoma. - Five types of standard treatment are used: - Chemotherapy - Radiation therapy - Targeted therapy - Surgery - High-dose chemotherapy with stem cell transplant - New types of treatment are being tested in clinical trials. - Proton beam radiation therapy - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for children with Hodgkin lymphoma. Different types of treatment are available for children with Hodgkin lymphoma. Some treatments are standard and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment. Children with Hodgkin lymphoma should have their treatment planned by a team of health care providers who are experts in treating childhood cancer. Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other pediatric health care providers who are experts in treating children with Hodgkin lymphoma and who specialize in certain areas of medicine. These may include the following specialists: - Pediatrician. - Medical oncologist /hematologist. - Pediatric surgeon. - Radiation oncologist. - Endocrinologist. - Pediatric nurse specialist. - Rehabilitation specialist. - Psychologist. - Social worker. - Child-life specialist. The treatment of Hodgkin lymphoma in adolescents and young adults may be different than the treatment for children. Some adolescents and young adults are treated with an adult treatment regimen. Children and adolescents may have treatment-related side effects that appear months or years after treatment for Hodgkin lymphoma. Some cancer treatments cause side effects that continue or appear months or years after cancer treatment has ended. These are called late effects. Because late effects affect health and development, regular follow-up exams are important. Late effects of cancer treatment may include: - Physical problems that affect the following: - Development of sex and reproductive organs. - Fertility (ability to have children). - Bone and muscle growth and development. - Thyroid, heart, or lung function. - Teeth, gums, and salivary gland function. - Spleen function (increased risk of infection). - Changes in mood, feelings, thinking, learning, or memory. - Second cancers (new types of cancer). For female survivors of Hodgkin lymphoma, there is an increased risk of breast cancer. This risk depends on the amount of radiation therapy they received to the breast during treatment and the chemotherapy regimen used. The risk of breast cancer is decreased if these female survivors also received radiation therapy to the ovaries. It is suggested that female survivors who received radiation therapy to the breast have a mammogram once a year starting 8 years after treatment or at age 25 years, whichever is later. Female survivors of childhood Hodgkin lymphoma who have breast cancer have an increased risk of dying from the disease compared to patients with no history of Hodgkin lymphoma who have breast cancer. Some late effects may be treated or controlled. It is important to talk with your child's doctors about the possible late effects caused by some treatments. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information). Five types of standard treatment are used: Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is treatment using more than one anticancer drug. The way the chemotherapy is given depends on the risk group. For example, children with low-risk Hodgkin lymphoma receive fewer cycles of treatment, fewer anticancer drugs, and lower doses of anticancer drugs than children with high-risk lymphoma. See Drugs Approved for Hodgkin Lymphoma for more information. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. Certain ways of giving radiation therapy can help keep radiation from damaging nearby healthy tissue. These types of external radiation therapy include the following: - Conformal radiation therapy: Conformal radiation therapy is a type of external radiation therapy that uses a computer to make a 3-dimensional (3-D) picture of the tumor and shapes the radiation beams to fit the tumor. - Intensity-modulated radiation therapy (IMRT): IMRT is a type of 3-dimensional (3-D) radiation therapy that uses a computer to make pictures of the size and shape of the tumor. Thin beams of radiation of different intensities (strengths) are aimed at the tumor from many angles. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. Radiation therapy may be given, based on the childs risk group and chemotherapy regimen. External radiation therapy is used to treat childhood Hodgkin lymphoma. The radiation is given only to the lymph nodes or other areas with cancer. Internal radiation therapy is not used to treat Hodgkin lymphoma. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Monoclonal antibody therapy and proteasome inhibitor therapy are being used in the treatment of childhood Hodgkin lymphoma. Monoclonal antibody therapy is a cancer treatment that uses antibodies made in the laboratory from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. In children, rituximab may be used to treat refractory or recurrent Hodgkin lymphoma. Brentuximab, nivolumab, pembrolizumab, and atezolizumab are monoclonal antibodies being studied to treat children. Proteasome inhibitor therapy is a type of targeted therapy that blocks the action of proteasomes (proteins that remove other proteins the body no longer needs) in cancer cells and may prevent the growth of tumors. Bortezomib is a proteasome inhibitor used to treat refractory or recurrent childhood Hodgkin lymphoma. Surgery Surgery may be done to remove as much of the tumor as possible for localized nodular lymphocyte -predominant childhood Hodgkin lymphoma. High-dose chemotherapy with stem cell transplant High-dose chemotherapy with stem cell transplant is a way of giving high doses of chemotherapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. See Drugs Approved for Hodgkin Lymphoma for more information. New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Proton beam radiation therapy Proton-beam therapy is a type of high-energy, external radiation therapy that uses streams of protons (small, positively-charged particles of matter) to make radiation. This type of radiation therapy may help lessen the damage to healthy tissue near the tumor. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For patients who receive chemotherapy alone, a PET scan may be done 3 weeks or more after treatment ends. For patients who receive radiation therapy last, a PET scan should not be done until 8 to 12 weeks after treatment. Treatment Options for Children and Adolescents with Hodgkin Lymphoma Low-Risk Classical Childhood Hodgkin Lymphoma Treatment of low-risk classical childhood Hodgkin lymphoma may include the following: - Combination chemotherapy. - Radiation therapy may also be given to the areas with cancer. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I childhood Hodgkin lymphoma and stage II childhood Hodgkin lymphoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Intermediate-Risk Classical Childhood Hodgkin Lymphoma Treatment of intermediate-risk classical childhood Hodgkin lymphoma may include the following: - Combination chemotherapy. - Radiation therapy may also be given to the areas with cancer. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I childhood Hodgkin lymphoma, stage II childhood Hodgkin lymphoma, stage III childhood Hodgkin lymphoma and stage IV childhood Hodgkin lymphoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. High-Risk Classical Childhood Hodgkin Lymphoma Treatment of high-risk classical childhood Hodgkin lymphoma may include the following: - Higher dose combination chemotherapy. - Radiation therapy may also be given to the areas with cancer. - A clinical trial of targeted therapy and combination chemotherapy. Radiation therapy may also be given to the areas with cancer. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III childhood Hodgkin lymphoma and stage IV childhood Hodgkin lymphoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Nodular Lymphocyte-Predominant Childhood Hodgkin Lymphoma Treatment of nodular lymphocyte-predominant childhood Hodgkin lymphoma may include the following: - Surgery, if the tumor can be completely removed. - Chemotherapy with or without low-dose external radiation therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with childhood nodular lymphocyte predominant Hodgkin lymphoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website.",Childhood Hodgkin Lymphoma,0000004_6,CancerGov,https://www.cancer.gov/types/lymphoma/patient/child-hodgkin-treatment-pdq,C0220644,T191,Disorders What is (are) Childhood Non-Hodgkin Lymphoma ?,0000004_7-1,information,"Key Points - Childhood non-Hodgkin lymphoma is a disease in which malignant (cancer) cells form in the lymph system. - The main types of lymphoma are Hodgkin lymphoma and non-Hodgkin lymphoma. - There are three major types of childhood non-Hodgkin lymphoma. - Mature B-cell non-Hodgkin lymphoma - Lymphoblastic lymphoma - Anaplastic large cell lymphoma - Some types of non-Hodgkin lymphoma are rare in children. - Past treatment for cancer and having a weakened immune system affect the risk of having childhood non-Hodgkin lymphoma. - Signs of childhood non-Hodgkin lymphoma include breathing problems and swollen lymph nodes. - Tests that examine the body and lymph system are used to detect (find) and diagnose childhood non-Hodgkin lymphoma. - A biopsy is done to diagnose childhood non-Hodgkin lymphoma. - Certain factors affect prognosis (chance of recovery) and treatment options. Childhood non-Hodgkin lymphoma is a disease in which malignant (cancer) cells form in the lymph system. Childhood non-Hodgkin lymphoma is a type of cancer that forms in the lymph system, which is part of the body's immune system. The immune system protects the body from foreign substances, infection, and diseases. The lymph system is made up of the following: - Lymph: Colorless, watery fluid that carries white blood cells called lymphocytes through the lymph system. Lymphocytes protect the body against infections and the growth of tumors. There are three types of lymphocytes: - B lymphocytes that make antibodies to help fight infection. - T lymphocytes that help B lymphocytes make the antibodies that help fight infection. - Natural killer cells that attack cancer cells and viruses. - Lymph vessels: A network of thin tubes that collect lymph from different parts of the body and return it to the bloodstream. - Lymph nodes: Small, bean-shaped structures that filter lymph and store white blood cells that help fight infection and disease. Lymph nodes are located along the network of lymph vessels found throughout the body. Clusters of lymph nodes are found in the neck, underarm, abdomen, pelvis, and groin. - Spleen: An organ that makes lymphocytes, filters the blood, stores blood cells, and destroys old blood cells. The spleen is on the left side of the abdomen near the stomach. - Thymus: An organ in which lymphocytes grow and multiply. The thymus is in the chest behind the breastbone. - Tonsils: Two small masses of lymph tissue at the back of the throat. The tonsils make lymphocytes. - Bone marrow: The soft, spongy tissue in the center of large bones. Bone marrow makes white blood cells, red blood cells, and platelets. Non-Hodgkin lymphoma can begin in B lymphocytes, T lymphocytes, or natural killer cells. Lymphocytes can also be found in the blood and collect in the lymph nodes, spleen, and thymus. Lymph tissue is also found in other parts of the body such as the stomach, thyroid gland, brain, and skin. Non-Hodgkin lymphoma can occur in both adults and children. Treatment for children is different than treatment for adults. See the following PDQ summaries for information about treatment of non-Hodgkin lymphoma in adults: - Adult Non-Hodgkin Lymphoma - Primary CNS Lymphoma Treatment - Mycosis Fungoides and the Sezary Syndrome Treatment The main types of lymphoma are Hodgkin lymphoma and non-Hodgkin lymphoma. Lymphomas are divided into two general types: Hodgkin lymphoma and non-Hodgkin lymphoma. This summary is about the treatment of childhood non-Hodgkin lymphoma. See the PDQ summary on Childhood Hodgkin Lymphoma Treatment for information about childhood Hodgkin lymphoma. There are three major types of childhood non-Hodgkin lymphoma. The type of lymphoma is determined by how the cells look under a microscope. The three major types of childhood non-Hodgkin lymphoma are: Mature B-cell non-Hodgkin lymphoma Mature B-cell non-Hodgkin lymphomas include: - Burkitt and Burkitt-like lymphoma/leukemia: Burkitt lymphoma and Burkitt leukemia are different forms of the same disease. Burkitt lymphoma/leukemia is an aggressive (fast-growing) disorder of B lymphocytes that is most common in children and young adults. It may form in the abdomen, Waldeyer's ring, testicles, bone, bone marrow, skin, or central nervous system (CNS). Burkitt leukemia may start in the lymph nodes as Burkitt lymphoma and then spread to the blood and bone marrow, or it may start in the blood and bone marrow without forming in the lymph nodes first. Both Burkitt leukemia and Burkitt lymphoma have been linked to infection with the Epstein-Barr virus (EBV), although EBV infection is more likely to occur in patients in Africa than in the United States. Burkitt and Burkitt-like lymphoma/leukemia are diagnosed when a sample of tissue is checked and a certain change to the c-myc gene is found. - Diffuse large B-cell lymphoma: Diffuse large B-cell lymphoma is the most common type of non-Hodgkin lymphoma. It is a type of B-cell non-Hodgkin lymphoma that grows quickly in the lymph nodes. The spleen, liver, bone marrow, or other organs are also often affected. Diffuse large B-cell lymphoma occurs more often in adolescents than in children. - Primary mediastinal B-cell lymphoma: A type of lymphoma that develops from B cells in the mediastinum (the area behind the breastbone). It may spread to nearby organs including the lungs and the sac around the heart. It may also spread to lymph nodes and distant organs including the kidneys. In children and adolescents, primary mediastinal B-cell lymphoma occurs more often in older adolescents. Lymphoblastic lymphoma Lymphoblastic lymphoma is a type of lymphoma that mainly affects T-cell lymphocytes. It usually forms in the mediastinum (the area behind the breastbone). This causes trouble breathing, wheezing, trouble swallowing, or swelling of the head and neck. It may spread to lymph nodes, bone, bone marrow, skin, the CNS, abdominal organs, and other areas. Lymphoblastic lymphoma is a lot like acute lymphoblastic leukemia (ALL). Anaplastic large cell lymphoma Anaplastic large cell lymphoma is a type of lymphoma that mainly affects T-cell lymphocytes. It usually forms in the lymph nodes, skin, or bone, and sometimes forms in the gastrointestinal tract, lung, tissue that covers the lungs, and muscle. Patients with anaplastic large cell lymphoma have a receptor, called CD30, on the surface of their T cells. In many children, anaplastic large cell lymphoma is marked by changes in the ALK gene that makes a protein called anaplastic lymphoma kinase. A pathologist checks for these cell and gene changes to help diagnose anaplastic large cell lymphoma. Some types of non-Hodgkin lymphoma are rare in children. Some types of childhood non-Hodgkin lymphoma are less common. These include: - Pediatric-type follicular lymphoma : In children, follicular lymphoma occurs mainly in males. It is more likely to be found in one area and does not spread to other places in the body. It usually forms in the tonsils and lymph nodes in the neck, but may also form in the testicles, kidney, gastrointestinal tract, and salivary gland. - Marginal zone lymphoma : Marginal zone lymphoma is a type of lymphoma that tends to grow and spread slowly and is usually found at an early stage. It may be found in the lymph nodes or in areas outside the lymph nodes. Marginal zone lymphoma found outside the lymph nodes in children is called mucosa-associated lymphoid tissue (MALT) lymphoma and may be linked to Helicobacter pylori infection of the gastrointestinal tract and Chlamydophila psittaci infection of the conjunctival membrane which lines the eye. - Primary central nervous system (CNS) lymphoma : Primary CNS lymphoma is extremely rare in children. - Peripheral T-cell lymphoma : Peripheral T-cell lymphoma is an aggressive (fast-growing) non-Hodgkin lymphoma that begins in mature T lymphocytes. The T lymphocytes mature in the thymus gland and travel to other parts of the lymph system, such as the lymph nodes, bone marrow, and spleen. - Cutaneous T-cell lymphoma : Cutaneous T-cell lymphoma begins in the skin and can cause the skin to thicken or form a tumor. It is very rare in children, but is more common in adolescents and young adults. There are different types of cutaneous T-cell lymphoma, such as cutaneous anaplastic large cell lymphoma, subcutaneous panniculitis-like T-cell lymphoma, gamma-delta T-cell lymphoma, and mycosis fungoides. Mycosis fungoides rarely occurs in children and adolescents. Past treatment for cancer and having a weakened immune system affect the risk of having childhood non-Hodgkin lymphoma. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesnt mean that you will not get cancer. Talk with your child's doctor if you think your child may be at risk. Possible risk factors for childhood non-Hodgkin lymphoma include the following: - Past treatment for cancer. - Being infected with the Epstein-Barr virus or human immunodeficiency virus (HIV). - Having a weakened immune system after a transplant or from medicines given after a transplant. - Having certain inherited diseases of the immune system. If lymphoma or lymphoproliferative disease is linked to a weakened immune system from certain inherited diseases, HIV infection, a transplant or medicines given after a transplant, the condition is called lymphoproliferative disease associated with immunodeficiency. The different types of lymphoproliferative disease associated with immunodeficiency include: - Lymphoproliferative disease associated with primary immunodeficiency. - HIV-associated non-Hodgkin lymphoma. - Post-transplant lymphoproliferative disease.",Childhood Non-Hodgkin Lymphoma,0000004_7,CancerGov,https://www.cancer.gov/types/lymphoma/patient/child-nhl-treatment-pdq,C0019829,T191,Disorders What are the symptoms of Childhood Non-Hodgkin Lymphoma ?,0000004_7-2,symptoms,"Signs of childhood non-Hodgkin lymphoma include breathing problems and swollen lymph nodes. These and other signs may be caused by childhood non-Hodgkin lymphoma or by other conditions. Check with a doctor if your child has any of the following: - Trouble breathing. - Wheezing. - Coughing. - High-pitched breathing sounds. - Swelling of the head, neck, upper body, or arms. - Trouble swallowing. - Painless swelling of the lymph nodes in the neck, underarm, stomach, or groin. - Painless lump or swelling in a testicle. - Fever for no known reason. - Weight loss for no known reason. - Night sweats.",Childhood Non-Hodgkin Lymphoma,0000004_7,CancerGov,https://www.cancer.gov/types/lymphoma/patient/child-nhl-treatment-pdq,C0019829,T191,Disorders How to diagnose Childhood Non-Hodgkin Lymphoma ?,0000004_7-3,exams and tests,"Tests that examine the body and lymph system are used to detect (find) and diagnose childhood non-Hodgkin lymphoma. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body, including electrolytes, uric acid, blood urea nitrogen (BUN), creatinine, and liver function values. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. - Liver function tests : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by the liver. A higher than normal amount of a substance can be a sign of cancer. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. Sometimes a PET scan and a CT scan are done at the same time. If there is any cancer, this increases the chance that it will be found. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Lumbar puncture : A procedure used to collect cerebrospinal fluid (CSF) from the spinal column. This is done by placing a needle between two bones in the spine and into the CSF around the spinal cord and removing a sample of the fluid. The sample of CSF is checked under a microscope for signs that the cancer has spread to the brain and spinal cord. This procedure is also called an LP or spinal tap. - Chest x-ray : An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. - Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later. - A biopsy is done to diagnose childhood non-Hodgkin lymphoma: Cells and tissues are removed during a biopsy so they can be viewed under a microscope by a pathologist to check for signs of cancer. Because treatment depends on the type of non-Hodgkin lymphoma, biopsy samples should be checked by a pathologist who has experience in diagnosing childhood non-Hodgkin lymphoma. One of the following types of biopsies may be done: - Excisional biopsy : The removal of an entire lymph node or lump of tissue. - Incisional biopsy : The removal of part of a lump, lymph node, or sample of tissue. - Core biopsy : The removal of tissue or part of a lymph node using a wide needle. - Fine-needle aspiration (FNA) biopsy : The removal of tissue or part of a lymph node using a thin needle. The procedure used to remove the sample of tissue depends on where the tumor is in the body: - Bone marrow aspiration and biopsy : The removal of bone marrow and a small piece of bone by inserting a hollow needle into the hipbone or breastbone. - Mediastinoscopy : A surgical procedure to look at the organs, tissues, and lymph nodes between the lungs for abnormal areas. An incision (cut) is made at the top of the breastbone and a mediastinoscope is inserted into the chest. A mediastinoscope is a thin, tube-like instrument with a light and a lens for viewing. It also has a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of cancer. - Anterior mediastinotomy : A surgical procedure to look at the organs and tissues between the lungs and between the breastbone and heart for abnormal areas. An incision (cut) is made next to the breastbone and a mediastinoscope is inserted into the chest. A mediastinoscope is a thin, tube-like instrument with a light and a lens for viewing. It also has a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of cancer. This is also called the Chamberlain procedure. - Thoracentesis : The removal of fluid from the space between the lining of the chest and the lung, using a needle. A pathologist views the fluid under a microscope to look for cancer cells. If cancer is found, the following tests may be done to study the cancer cells: - Immunohistochemistry : A laboratory test that uses antibodies to check for certain antigens in a sample of tissue. The antibody is usually linked to a radioactive substance or a dye that causes the tissue to light up under a microscope. This type of test may be used to tell the difference between different types of cancer. - Flow cytometry : A laboratory test that measures the number of cells in a sample, the percentage of live cells in a sample, and certain characteristics of cells, such as size, shape, and the presence of tumor markers on the cell surface. The cells are stained with a light-sensitive dye, placed in a fluid, and passed in a stream before a laser or other type of light. The measurements are based on how the light-sensitive dye reacts to the light. - Cytogenetic analysis : A laboratory test in which cells in a sample of tissue are viewed under a microscope to look for certain changes in the chromosomes. - FISH (fluorescence in situ hybridization): A laboratory test used to look at genes or chromosomes in cells and tissues. Pieces of DNA that contain a fluorescent dye are made in the laboratory and added to cells or tissues on a glass slide. When these pieces of DNA attach to certain genes or areas of chromosomes on the slide, they light up when viewed under a microscope with a special light. This type of test is used to find certain gene changes. - Immunophenotyping : A laboratory test used to identify cells, based on the types of antigens or markers on the surface of the cell. This test is used to diagnose specific types of lymphoma by comparing the cancer cells to normal cells of the immune system.",Childhood Non-Hodgkin Lymphoma,0000004_7,CancerGov,https://www.cancer.gov/types/lymphoma/patient/child-nhl-treatment-pdq,C0019829,T191,Disorders What is the outlook for Childhood Non-Hodgkin Lymphoma ?,0000004_7-4,outlook,Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on: - The type of lymphoma. - Where the tumor is in the body when the tumor is diagnosed. - The stage of the cancer. - Whether there are certain changes in the chromosomes. - The type of initial treatment. - Whether the lymphoma responded to initial treatment. - The patients age and general health.,Childhood Non-Hodgkin Lymphoma,0000004_7,CancerGov,https://www.cancer.gov/types/lymphoma/patient/child-nhl-treatment-pdq,C0019829,T191,Disorders what research (or clinical trials) is being done for Childhood Non-Hodgkin Lymphoma ?,0000004_7-5,research,"New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Childhood Non-Hodgkin Lymphoma,0000004_7,CancerGov,https://www.cancer.gov/types/lymphoma/patient/child-nhl-treatment-pdq,C0019829,T191,Disorders What are the stages of Childhood Non-Hodgkin Lymphoma ?,0000004_7-6,stages,"Key Points - After childhood non-Hodgkin lymphoma has been diagnosed, tests are done to find out if cancer cells have spread within the lymph system or to other parts of the body. - There are three ways that cancer spreads in the body. - The following stages are used for childhood non-Hodgkin lymphoma: - Stage I - Stage II - Stage III - Stage IV After childhood non-Hodgkin lymphoma has been diagnosed, tests are done to find out if cancer cells have spread within the lymph system or to other parts of the body. The process used to find out if cancer has spread within the lymph system or to other parts of the body is called staging. The results of tests and procedures used to diagnose non-Hodgkin lymphoma may also be used for staging. See the General Information section for a description of these tests and procedures. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following procedure also may be used to determine the stage: - Bone scan : A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones with cancer and is detected by a scanner. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. The following stages are used for childhood non-Hodgkin lymphoma: Stage I In stage I childhood non-Hodgkin lymphoma, cancer is found: - in one group of lymph nodes; or - in one area outside the lymph nodes. No cancer is found in the abdomen or mediastinum (area between the lungs). Stage II In stage II childhood non-Hodgkin lymphoma, cancer is found: - in one area outside the lymph nodes and in nearby lymph nodes; or - in two or more areas either above or below the diaphragm, and may have spread to nearby lymph nodes; or - to have started in the stomach or intestines and can be completely removed by surgery. Cancer may have spread to certain nearby lymph nodes. Stage III In stage III childhood non-Hodgkin lymphoma, cancer is found: - in at least one area above the diaphragm and in at least one area below the diaphragm; or - to have started in the chest; or - to have started in the abdomen and spread throughout the abdomen; or - in the area around the spine. Stage IV In stage IV childhood non-Hodgkin lymphoma, cancer is found in the bone marrow, brain, or cerebrospinal fluid. Cancer may also be found in other parts of the body.",Childhood Non-Hodgkin Lymphoma,0000004_7,CancerGov,https://www.cancer.gov/types/lymphoma/patient/child-nhl-treatment-pdq,C0019829,T191,Disorders What are the treatments for Childhood Non-Hodgkin Lymphoma ?,0000004_7-7,treatment,"Key Points - There are different types of treatment for children with non-Hodgkin lymphoma. - Children with non-Hodgkin lymphoma should have their treatment planned by a team of doctors who are experts in treating childhood cancer. - Some cancer treatments cause side effects months or years after treatment has ended. - Six types of standard treatment are used: - Chemotherapy - Radiation therapy - High-dose chemotherapy with stem cell transplant - Targeted therapy - Other drug therapy - Phototherapy - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for children with non-Hodgkin lymphoma. Different types of treatment are available for children with non-Hodgkin lymphoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Taking part in a clinical trial should be considered for all children with non-Hodgkin lymphoma. Some clinical trials are open only to patients who have not started treatment. Children with non-Hodgkin lymphoma should have their treatment planned by a team of doctors who are experts in treating childhood cancer. Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other health care providers who are experts in treating children with non-Hodgkin lymphoma and who specialize in certain areas of medicine. These may include the following specialists: - Pediatrician. - Radiation oncologist. - Pediatric hematologist. - Pediatric surgeon. - Pediatric nurse specialist. - Rehabilitation specialist. - Psychologist. - Social worker. Some cancer treatments cause side effects months or years after treatment has ended. Side effects from cancer treatment that begin during or after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include the following: - Physical problems. - Changes in mood, feelings, thinking, learning, or memory. - Second cancers (new types of cancer). Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information.) Six types of standard treatment are used: Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid (intrathecal chemotherapy), an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas. Combination chemotherapy is treatment using two or more anticancer drugs. The way the chemotherapy is given depends on the type and stage of the cancer being treated. Intrathecal chemotherapy may be used to treat childhood non-Hodgkin lymphoma that has spread, or may spread, to the brain. When used to lessen the chance cancer will spread to the brain, it is called CNS prophylaxis. Intrathecal chemotherapy is given in addition to chemotherapy by mouth or vein. Higher than usual doses of chemotherapy may also be used as CNS prophylaxis. See Drugs Approved for Non-Hodgkin Lymphoma for more information. Radiation therapy Radiation therapy is a cancer treatment that uses high energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type of non-Hodgkin lymphoma being treated. External radiation therapy may be used to treat childhood non-Hodgkin lymphoma that has spread, or may spread, to the brain and spinal cord. Internal radiation therapy is not used to treat non-Hodgkin lymphoma. High-dose chemotherapy with stem cell transplant This treatment is a way of giving high doses of chemotherapy and then replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the bone marrow or blood of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the bodys blood cells. See Drugs Approved for Non-Hodgkin Lymphoma for more information. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Monoclonal antibodies, tyrosine kinase inhibitors, and immunotoxins are three types of targeted therapy being used or studied in the treatment of childhood non-Hodgkin lymphoma. Monoclonal antibody therapy is a cancer treatment that uses antibodies made in the laboratory from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. - Rituximab is used to treat several types of childhood non-Hodgkin lymphoma. - Brentuximab vedotin is a monoclonal antibody combined with an anticancer drug that is used to treat anaplastic large cell lymphoma. A bispecific monoclonal antibody is made up of two different monoclonal antibodies that bind to two different substances and kills cancer cells. Bispecific monoclonal antibody therapy is used in the treatment of Burkitt and Burkitt-like lymphoma /leukemia and diffuse large B-cell lymphoma. Tyrosine kinase inhibitors (TKIs) block signals that tumors need to grow. Some TKIs also keep tumors from growing by preventing the growth of new blood vessels to the tumors. Other types of kinase inhibitors, such as crizotinib, are being studied for childhood non-Hodgkin lymphoma. Immunotoxins can bind to cancer cells and kill them. Denileukin diftitox is an immunotoxin used to treat cutaneous T-cell lymphoma. See Drugs Approved for Non-Hodgkin Lymphoma for more information. Other drug therapy Retinoids are drugs related to vitamin A. Retinoid therapy with bexarotene is used to treat several types of cutaneous T-cell lymphoma. Steroids are hormones made naturally in the body. They can also be made in a laboratory and used as drugs. Steroid therapy is used to treat cutaneous T-cell lymphoma. Phototherapy Phototherapy is a cancer treatment that uses a drug and a certain type of laser light to kill cancer cells. A drug that is not active until it is exposed to light is injected into a vein. The drug collects more in cancer cells than in normal cells. For skin cancer in the skin, laser light is shined onto the skin and the drug becomes active and kills the cancer cells. Phototherapy is used in the treatment of cutaneous T-cell lymphoma. New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Childhood Non-Hodgkin Lymphoma Burkitt and Burkitt-like lymphoma/leukemia Treatment options for newly diagnosed Burkitt and Burkitt-like lymphoma/leukemia Treatment options for newly diagnosed Burkitt and Burkitt-like lymphoma /leukemia may include: - Surgery to remove as much of the tumor as possible, followed by combination chemotherapy. - Combination chemotherapy. - Combination chemotherapy and targeted therapy (rituximab). Treatment options for recurrent Burkitt and Burkitt-like lymphoma/leukemia Treatment options for recurrent Burkitt and Burkitt-like non-Hodgkin lymphoma /leukemia may include: - Combination chemotherapy and targeted therapy (rituximab). - High-dose chemotherapy with stem cell transplant with the patient's own cells or cells from a donor. - Targeted therapy with a bispecific antibody. Check the list of NCI-supported cancer clinical trials that are now accepting patients with childhood Burkitt lymphoma, stage I childhood small noncleaved cell lymphoma, stage II childhood small noncleaved cell lymphoma, stage III childhood small noncleaved cell lymphoma, stage IV childhood small noncleaved cell lymphoma and recurrent childhood small noncleaved cell lymphoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Diffuse large B-cell lymphoma Treatment options for newly diagnosed diffuse large B-cell lymphoma Treatment options for newly diagnosed diffuse large B-cell lymphoma may include: - Surgery to remove as much of the tumor as possible, followed by combination chemotherapy. - Combination chemotherapy. - Combination chemotherapy and targeted therapy (rituximab). Treatment options for recurrent diffuse large B-cell lymphoma Treatment options for recurrent diffuse large B-cell lymphoma may include: - Combination chemotherapy and targeted therapy (rituximab). - High-dose chemotherapy with stem cell transplant with the patient's own cells or cells from a donor. - Targeted therapy with a bispecific antibody. Check the list of NCI-supported cancer clinical trials that are now accepting patients with childhood diffuse large cell lymphoma, stage I childhood large cell lymphoma, stage II childhood large cell lymphoma, stage III childhood large cell lymphoma, stage IV childhood large cell lymphoma and recurrent childhood large cell lymphoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Primary Mediastinal B-cell Lymphoma Treatment options for primary mediastinal B-cell lymphoma Treatment options for primary mediastinal B-cell lymphoma may include: - Combination chemotherapy and targeted therapy (rituximab). Lymphoblastic Lymphoma Treatment options for newly diagnosed lymphoblastic lymphoma Lymphoblastic lymphoma may be classified as the same disease as acute lymphoblastic leukemia (ALL). Treatment options for lymphoblastic lymphoma may include: - Combination chemotherapy. CNS prophylaxis with radiation therapy or chemotherapy is also given if cancer has spread to the brain and spinal cord. - A clinical trial of chemotherapy with different regimens for CNS prophylaxis. - A clinical trial of combination chemotherapy with or without targeted therapy (bortezomib). Treatment options for recurrent lymphoblastic lymphoma Treatment options for recurrent lymphoblastic lymphoma may include: - Combination chemotherapy. - High-dose chemotherapy with stem cell transplant with cells from a donor. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I childhood lymphoblastic lymphoma, stage II childhood lymphoblastic lymphoma, stage III childhood lymphoblastic lymphoma, stage IV childhood lymphoblastic lymphoma and recurrent childhood lymphoblastic lymphoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Anaplastic Large Cell Lymphoma Treatment options for newly diagnosed anaplastic large cell lymphoma Treatment options for anaplastic large cell lymphoma may include: - Surgery followed by combination chemotherapy. - Combination chemotherapy. - Intrathecal and systemic chemotherapy, for patients with cancer in the brain or spinal cord. - A clinical trial of targeted therapy (crizotinib or brentuximab) and combination chemotherapy. Treatment options for recurrent anaplastic large cell lymphoma Treatment options for recurrent anaplastic large cell lymphoma may include: - Chemotherapy with one or more drugs. - Stem cell transplant with the patient's own cells or cells from a donor. - A clinical trial of targeted therapy (crizotinib) in children with recurrent anaplastic large cell lymphoma and changes in the ALK gene. - A clinical trial of targeted therapy (crizotinib) and combination chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I childhood anaplastic large cell lymphoma, stage II childhood anaplastic large cell lymphoma, stage III childhood anaplastic large cell lymphoma, stage IV childhood anaplastic large cell lymphoma and recurrent childhood anaplastic large cell lymphoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Lymphoproliferative Disease Associated With Immunodeficiency in Children Treatment options for lymphoproliferative disease associated with primary immunodeficiency Treatment options for lymphoproliferative disease in children and adolescents with weakened immune systems may include: - Chemotherapy. - Stem cell transplant with cells from a donor. Treatment options for HIV-associated non-Hodgkin lymphoma Treatment with highly active antiretroviral therapy or HAART (a combination of antiretroviral drugs) lowers the risk of non-Hodgkin lymphoma in patients infected with the human immunodeficiency virus (HIV). Treatment options for HIV-related non-Hodgkin lymphoma (NHL) in children may include: - Chemotherapy. For treatment of recurrent disease, treatment options depend on the type of non-Hodgkin lymphoma. Treatment options for post-transplant lymphoproliferative disease Treatment options for post-transplant lymphoproliferative disease may include: - Surgery to remove the tumor. If possible, lower doses of immunosuppressive drugs after a stem cell or organ transplant may be given. - Targeted therapy (rituximab). - Chemotherapy with or without targeted therapy (rituximab). - A clinical trial of immunotherapy using donor lymphocytes or the patient's own T cells to target Epstein-Barr infection. Rare NHL Occurring in Children Treatment options for pediatric-type follicular lymphoma Treatment options for follicular lymphoma in children may include: - Surgery. - Combination chemotherapy. For children whose cancer has certain changes in the genes, treatment is similar to that given to adults with follicular lymphoma. See the Follicular Lymphoma section in the PDQ summary on Adult Non-Hodgkin Lymphoma for information. Treatment options for marginal zone lymphoma Treatment options for marginal zone lymphoma in children may include: - Surgery. - Radiation therapy. - Antibiotic therapy, for mucosa-associated lymphoid tissue (MALT) lymphoma. Treatment options for primary CNS lymphoma Treatment options for primary CNS lymphoma in children may include: - Chemotherapy. Treatment options for peripheral T-cell lymphoma Treatment options for peripheral T-cell lymphoma in children may include: - Chemotherapy. - Radiation therapy. - Stem cell transplant with the patient's own cells or cells from a donor. Treatment options for cutaneous T-cell lymphoma Treatment options for subcutaneous panniculitis-like cutaneous T-cell lymphoma in children may include: - Watchful waiting. - High-dose steroids. - Targeted therapy (denileukin diftitox). - Combination chemotherapy. - Retinoid therapy. - Stem cell transplant. Treatment options for cutaneous anaplastic large cell lymphoma may include: - Surgery, radiation therapy, or both. In children, treatment options for mycosis fungoides may include: - Steroids applied to the skin. - Retinoid therapy. - Radiation therapy. - Phototherapy (light therapy using ultraviolet B radiation).",Childhood Non-Hodgkin Lymphoma,0000004_7,CancerGov,https://www.cancer.gov/types/lymphoma/patient/child-nhl-treatment-pdq,C0019829,T191,Disorders What is (are) Anal Cancer ?,0000005_1-1,information,"Key Points - Anal cancer is a disease in which malignant (cancer) cells form in the tissues of the anus. - Squamous cell carcinoma is the most common type of anal cancer. - In the United States, the number of new cases of anal cancer has increased in recent years. Anal cancer is a disease in which malignant (cancer) cells form in the tissues of the anus. The anus is the end of the large intestine, below the rectum, through which stool (solid waste) leaves the body. The anus is formed partly from the outer skin layers of the body and partly from the intestine. Two ring-like muscles, called sphincter muscles, open and close the anal opening and let stool pass out of the body. The anal canal, the part of the anus between the rectum and the anal opening, is about 1-1 inches long. The skin around the outside of the anus is called the perianal area. Tumors in this area are skin tumors, not anal cancer. See the following PDQ summary for more information about anal cancer: - Anal Cancer Treatment Squamous cell carcinoma is the most common type of anal cancer. In the United States, the most common type of anal cancer is squamous cell carcinoma. Studies show that human papillomavirus (HPV) infection is the main cause of this type of anal cancer. Another type of anal cancer, called anal adenocarcinoma, is very rare and is not discussed in this summary. In the United States, the number of new cases of anal cancer has increased in recent years. From 2004 to 2013, new cases of anal cancer and deaths from anal cancer increased each year. The increase in new cases was slightly higher in women and the increase in deaths from anal cancer was slightly higher in men.",Anal Cancer,0000005_1,CancerGov,https://www.cancer.gov/types/anal/patient/anal-treatment-pdq,C0279637,T191,Disorders Who is at risk for Anal Cancer? ?,0000005_1-2,susceptibility,"Being infected with the human papillomavirus (HPV) increases the risk of developing anal cancer. Risk factors include the following: - Being infected with human papillomavirus (HPV). - Having many sexual partners. - Having receptive anal intercourse (anal sex). - Being older than 50 years. - Frequent anal redness, swelling, and soreness. - Having anal fistulas (abnormal openings). - Smoking cigarettes.",Anal Cancer,0000005_1,CancerGov,https://www.cancer.gov/types/anal/patient/anal-treatment-pdq,C0279637,T191,Disorders What are the symptoms of Anal Cancer ?,0000005_1-3,symptoms,Signs of anal cancer include bleeding from the anus or rectum or a lump near the anus. These and other signs and symptoms may be caused by anal cancer or by other conditions. Check with your doctor if you have any of the following: - Bleeding from the anus or rectum. - Pain or pressure in the area around the anus. - Itching or discharge from the anus. - A lump near the anus. - A change in bowel habits.,Anal Cancer,0000005_1,CancerGov,https://www.cancer.gov/types/anal/patient/anal-treatment-pdq,C0279637,T191,Disorders How to diagnose Anal Cancer ?,0000005_1-4,exams and tests,"Tests that examine the rectum and anus are used to detect (find) and diagnose anal cancer. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Digital rectal examination (DRE): An exam of the anus and rectum. The doctor or nurse inserts a lubricated, gloved finger into the lower part of the rectum to feel for lumps or anything else that seems unusual. - Anoscopy: An exam of the anus and lower rectum using a short, lighted tube called an anoscope. - Proctoscopy : An exam of the rectum using a short, lighted tube called a proctoscope. - Endo-anal or endorectal ultrasound : A procedure in which an ultrasound transducer (probe) is inserted into the anus or rectum and used to bounce high-energy sound waves (ultrasound) off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. - Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. If an abnormal area is seen during the anoscopy, a biopsy may be done at that time.",Anal Cancer,0000005_1,CancerGov,https://www.cancer.gov/types/anal/patient/anal-treatment-pdq,C0279637,T191,Disorders What is the outlook for Anal Cancer ?,0000005_1-5,outlook,Certain factors affect the prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) depends on the following: - The size of the tumor. - Where the tumor is in the anus. - Whether the cancer has spread to the lymph nodes. The treatment options depend on the following: - The stage of the cancer. - Where the tumor is in the anus. - Whether the patient has human immunodeficiency virus (HIV). - Whether cancer remains after initial treatment or has recurred.,Anal Cancer,0000005_1,CancerGov,https://www.cancer.gov/types/anal/patient/anal-treatment-pdq,C0279637,T191,Disorders What are the stages of Anal Cancer ?,0000005_1-6,stages,"Key Points - After anal cancer has been diagnosed, tests are done to find out if cancer cells have spread within the anus or to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - The following stages are used for anal cancer: - Stage 0 (Carcinoma in Situ) - Stage I - Stage II - Stage IIIA - Stage IIIB - Stage IV After anal cancer has been diagnosed, tests are done to find out if cancer cells have spread within the anus or to other parts of the body. The process used to find out if cancer has spread within the anus or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following tests may be used in the staging process: - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the abdomen or chest, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. For anal cancer, a CT scan of the pelvis and abdomen may be done. - Chest x-ray : An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if anal cancer spreads to the lung, the cancer cells in the lung are actually anal cancer cells. The disease is metastatic anal cancer, not lung cancer. The following stages are used for anal cancer: Stage 0 (Carcinoma in Situ) In stage 0, abnormal cells are found in the innermost lining of the anus. These abnormal cells may become cancer and spread into nearby normal tissue. Stage 0 is also called carcinoma in situ. Stage I In stage I, cancer has formed and the tumor is 2 centimeters or smaller. Stage II In stage II, the tumor is larger than 2 centimeters. Stage IIIA In stage IIIA, the tumor may be any size and has spread to either: - lymph nodes near the rectum; or - nearby organs, such as the vagina, urethra, and bladder. Stage IIIB In stage IIIB, the tumor may be any size and has spread: - to nearby organs and to lymph nodes near the rectum; or - to lymph nodes on one side of the pelvis and/or groin, and may have spread to nearby organs; or - to lymph nodes near the rectum and in the groin, and/or to lymph nodes on both sides of the pelvis and/or groin, and may have spread to nearby organs. Stage IV In stage IV, the tumor may be any size and cancer may have spread to lymph nodes or nearby organs and has spread to distant parts of the body.",Anal Cancer,0000005_1,CancerGov,https://www.cancer.gov/types/anal/patient/anal-treatment-pdq,C0279637,T191,Disorders what research (or clinical trials) is being done for Anal Cancer ?,0000005_1-7,research,"New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Radiosensitizers Radiosensitizers are drugs that make tumor cells more sensitive to radiation therapy. Combining radiation therapy with radiosensitizers may kill more tumor cells. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Anal Cancer,0000005_1,CancerGov,https://www.cancer.gov/types/anal/patient/anal-treatment-pdq,C0279637,T191,Disorders What are the treatments for Anal Cancer ?,0000005_1-8,treatment,"Key Points - There are different types of treatment for patients with anal cancer. - Three types of standard treatment are used: - Radiation therapy - Chemotherapy - Surgery - Having the human immunodeficiency virus can affect treatment of anal cancer. - New types of treatment are being tested in clinical trials. - Radiosensitizers - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with anal cancer. Different types of treatments are available for patients with anal cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Three types of standard treatment are used: Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External and internal radiation therapy are used to treat anal cancer. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. Surgery - Local resection: A surgical procedure in which the tumor is cut from the anus along with some of the healthy tissue around it. Local resection may be used if the cancer is small and has not spread. This procedure may save the sphincter muscles so the patient can still control bowel movements. Tumors that form in the lower part of the anus can often be removed with local resection. - Abdominoperineal resection: A surgical procedure in which the anus, the rectum, and part of the sigmoid colon are removed through an incision made in the abdomen. The doctor sews the end of the intestine to an opening, called a stoma, made in the surface of the abdomen so body waste can be collected in a disposable bag outside of the body. This is called a colostomy. Lymph nodes that contain cancer may also be removed during this operation. Having the human immunodeficiency virus can affect treatment of anal cancer. Cancer therapy can further damage the already weakened immune systems of patients who have the human immunodeficiency virus (HIV). For this reason, patients who have anal cancer and HIV are usually treated with lower doses of anticancer drugs and radiation than patients who do not have HIV. New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Radiosensitizers Radiosensitizers are drugs that make tumor cells more sensitive to radiation therapy. Combining radiation therapy with radiosensitizers may kill more tumor cells. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options by Stage Stage 0 (Carcinoma in Situ) Treatment of stage 0 is usually local resection. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage 0 anal cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage I Anal Cancer Treatment of stage I anal cancer may include the following: - Local resection. - External-beam radiation therapy with or without chemotherapy. If cancer remains after treatment, more chemotherapy and radiation therapy may be given to avoid the need for a permanent colostomy. - Internal radiation therapy. - Abdominoperineal resection, if cancer remains or comes back after treatment with radiation therapy and chemotherapy. - Internal radiation therapy for cancer that remains after treatment with external-beam radiation therapy. Patients who have had treatment that saves the sphincter muscles may receive follow-up exams every 3 months for the first 2 years, including rectal exams with endoscopy and biopsy, as needed. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I anal cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage II Anal Cancer Treatment of stage II anal cancer may include the following: - Local resection. - External-beam radiation therapy with chemotherapy. If cancer remains after treatment, more chemotherapy and radiation therapy may be given to avoid the need for a permanent colostomy. - Internal radiation therapy. - Abdominoperineal resection, if cancer remains or comes back after treatment with radiation therapy and chemotherapy. - A clinical trial of new treatment options. Patients who have had treatment that saves the sphincter muscles may receive follow-up exams every 3 months for the first 2 years, including rectal exams with endoscopy and biopsy, as needed. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage II anal cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage IIIA Anal Cancer Treatment of stage IIIA anal cancer may include the following: - External-beam radiation therapy with chemotherapy. If cancer remains after treatment, more chemotherapy and radiation therapy may be given to avoid the need for a permanent colostomy. - Internal radiation therapy. - Abdominoperineal resection, if cancer remains or comes back after treatment with chemotherapy and radiation therapy. - A clinical trial of new treatment options. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage IIIA anal cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage IIIB Anal Cancer Treatment of stage IIIB anal cancer may include the following: - External-beam radiation therapy with chemotherapy. - Local resection or abdominoperineal resection, if cancer remains or comes back after treatment with chemotherapy and radiation therapy. Lymph nodes may also be removed. - A clinical trial of new treatment options. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage IIIB anal cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage IV Anal Cancer Treatment of stage IV anal cancer may include the following: - Surgery as palliative therapy to relieve symptoms and improve the quality of life. - Radiation therapy as palliative therapy. - Chemotherapy with radiation therapy as palliative therapy. - A clinical trial of new treatment options. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage IV anal cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Anal Cancer,0000005_1,CancerGov,https://www.cancer.gov/types/anal/patient/anal-treatment-pdq,C0279637,T191,Disorders How to prevent Anal Cancer ?,0000005_2-1,prevention,"Key Points - Avoiding risk factors and increasing protective factors may help prevent cancer. - The following are risk factors for anal cancer: - Anal HPV infection - Certain medical conditions - History of cervical, vaginal, or vulvar cancer - HIV infection/AIDS - Immunosuppression - Certain sexual practices - Cigarette smoking - The following protective factor decreases the risk of anal cancer: - HPV vaccine - It is not clear if the following protective factor decreases the risk of anal cancer: - Condom use - Cancer prevention clinical trials are used to study ways to prevent cancer. - New ways to prevent anal cancer are being studied in clinical trials. Avoiding risk factors and increasing protective factors may help prevent cancer. Avoiding cancer risk factors may help prevent certain cancers. Risk factors include smoking, being overweight, and not getting enough exercise. Increasing protective factors such as quitting smoking and exercising may also help prevent some cancers. Talk to your doctor or other health care professional about how you might lower your risk of cancer. The following are risk factors for anal cancer: Anal HPV infection Being infected with human papillomavirus (HPV) is the main risk factor for anal cancer. Being infected with HPV can lead to squamous cell carcinoma of the anus, the most common type of anal cancer. About nine out of every ten cases of anal cancer are found in patients with anal HPV infection. Patients with healthy immune systems are usually able to fight HPV infections. Patients with weakened immune systems who are infected with HPV have a higher risk of anal cancer. Certain medical conditions History of cervical, vaginal, or vulvar cancer Cervical cancer, vaginal cancer, and vulvar cancer are related to HPV infection. Women who have had cervical, vaginal, or vulvar cancer have a higher risk of anal cancer. HIV infection/AIDS Being infected with human immunodeficiency virus (HIV) is a strong risk factor for anal cancer. HIV is the cause of acquired immunodeficiency syndrome (AIDS). HIV weakens the body's immune system and its ability to fight infection. HPV infection of the anus is common among patients who are HIV-positive. The risk of anal cancer is higher in men who are HIV-positive and have sex with men compared with men who are HIV-negative and have sex with men. Women who are HIV-positive also have an increased risk of anal cancer compared with women who are HIV-negative. Studies show that intravenous drug use or cigarette smoking may further increase the risk of anal cancer in patients who are HIV-positive. Immunosuppression Immunosuppression is a condition that weakens the body's immune system and its ability to fight infections and other diseases. Chronic (long-term) immunosuppression may increase the risk of anal cancer because it lowers the body's ability to fight HPV infection. Patients who have an organ transplant and receive immunosuppressive medicine to prevent organ rejection have an increased risk of anal cancer. Having an autoimmune disorder such as Crohn disease or psoriasis may increase the risk of anal cancer. It is not clear if the increased risk is due to the autoimmune condition, the treatment for the condition, or a combination of both. Certain sexual practices The following sexual practices increase the risk of anal cancer because they increase the chance of being infected with HPV: - Having receptive anal intercourse (anal sex). - Having many sexual partners. - Sex between men. Men and women who have a history of anal warts or other sexually transmitted diseases also have an increased risk of anal cancer. Cigarette smoking Studies show that cigarette smoking increases the risk of anal cancer. Studies also show that current smokers have a higher risk of anal cancer than smokers who have quit or people who have never smoked. The following protective factor decreases the risk of anal cancer: HPV vaccine The human papillomavirus (HPV) vaccine is used to prevent anal cancer, cervical cancer, vulvar cancer, and vaginal cancer caused by HPV. It is also used to prevent lesions caused by HPV that may become cancer in the future. Studies show that being vaccinated against HPV lowers the risk of anal cancer. The vaccine may work best when it is given before a person is exposed to HPV. It is not clear if the following protective factor decreases the risk of anal cancer: Condom use It is not known if the use of condoms protects against anal HPV infection. This is because not enough studies have been done to prove this. Cancer prevention clinical trials are used to study ways to prevent cancer. Cancer prevention clinical trials are used to study ways to lower the risk of developing certain types of cancer. Some cancer prevention trials are conducted with healthy people who have not had cancer but who have an increased risk for cancer. Other prevention trials are conducted with people who have had cancer and are trying to prevent another cancer of the same type or to lower their chance of developing a new type of cancer. Other trials are done with healthy volunteers who are not known to have any risk factors for cancer. The purpose of some cancer prevention clinical trials is to find out whether actions people take can prevent cancer. These may include eating fruits and vegetables, exercising, quitting smoking, or taking certain medicines, vitamins, minerals, or food supplements. New ways to prevent anal cancer are being studied in clinical trials. Clinical trials are taking place in many parts of the country. Information about clinical trials for anal cancer prevention can be found in the Clinical Trials section of the NCI Web site.",Anal Cancer,0000005_2,CancerGov,https://www.cancer.gov/types/anal/patient/anal-prevention-pdq,C0279637,T191,Disorders What is (are) Adult Central Nervous System Tumors ?,0000006_1-1,information,"Key Points - An adult central nervous system tumor is a disease in which abnormal cells form in the tissues of the brain and/or spinal cord. - A tumor that starts in another part of the body and spreads to the brain is called a metastatic brain tumor. - The brain controls many important body functions. - The spinal cord connects the brain to nerves in most parts of the body. - There are different types of brain and spinal cord tumors. - Astrocytic Tumors - Oligodendroglial Tumors - Mixed Gliomas - Ependymal Tumors - Medulloblastomas - Pineal Parenchymal Tumors - Meningeal Tumors - Germ Cell Tumors - Craniopharyngioma (Grade I) - Having certain genetic syndromes may increase the risk of a central nervous system tumor. - The cause of most adult brain and spinal cord tumors is not known. - The signs and symptoms of adult brain and spinal cord tumors are not the same in every person. - Tests that examine the brain and spinal cord are used to diagnose adult brain and spinal cord tumors. - A biopsy is also used to diagnose a brain tumor. - Sometimes a biopsy or surgery cannot be done. - Certain factors affect prognosis (chance of recovery) and treatment options. An adult central nervous system tumor is a disease in which abnormal cells form in the tissues of the brain and/or spinal cord. There are many types of brain and spinal cord tumors. The tumors are formed by the abnormal growth of cells and may begin in different parts of the brain or spinal cord. Together, the brain and spinal cord make up the central nervous system (CNS). The tumors may be either benign (not cancer) or malignant (cancer): - Benign brain and spinal cord tumors grow and press on nearby areas of the brain. They rarely spread into other tissues and may recur (come back). - Malignant brain and spinal cord tumors are likely to grow quickly and spread into other brain tissue. When a tumor grows into or presses on an area of the brain, it may stop that part of the brain from working the way it should. Both benign and malignant brain tumors cause signs and symptoms and need treatment. Brain and spinal cord tumors can occur in both adults and children. However, treatment for children may be different than treatment for adults. (See the PDQ summary on Childhood Brain and Spinal Cord Tumors Treatment Overview for more information on the treatment of children.) For information about lymphoma that begins in the brain, see the PDQ summary on Primary CNS Lymphoma Treatment. A tumor that starts in another part of the body and spreads to the brain is called a metastatic brain tumor. Tumors that start in the brain are called primary brain tumors. Primary brain tumors may spread to other parts of the brain or to the spine. They rarely spread to other parts of the body. Often, tumors found in the brain have started somewhere else in the body and spread to one or more parts of the brain. These are called metastatic brain tumors (or brain metastases). Metastatic brain tumors are more common than primary brain tumors. Up to half of metastatic brain tumors are from lung cancer. Other types of cancer that commonly spread to the brain include: - Melanoma. - Breast cancer. - Colon cancer. - Kidney cancer. - Nasopharyngeal cancer. - Cancer of unknown primary site. Cancer may spread to the leptomeninges (the two innermost membranes covering the brain and spinal cord). This is called leptomeningeal carcinomatosis. The most common cancers that spread to the leptomeninges include: - Breast cancer. - Lung cancer. - Leukemia. - Lymphoma. See the following for more information from PDQ about cancers that commonly spread to the brain or spinal cord: - Adult Hodgkin Lymphoma Treatment - Adult Non-Hodgkin Lymphoma Treatment - Breast Cancer Treatment - Carcinoma of Unknown Primary Treatment - Colon Cancer Treatment - Leukemia Home Page - Melanoma Treatment - Nasopharyngeal Cancer Treatment - Non-Small Cell Lung Cancer Treatment - Renal Cell Cancer Treatment - Small Cell Lung Cancer Treatment The brain controls many important body functions. The brain has three major parts: - The cerebrum is the largest part of the brain. It is at the top of the head. The cerebrum controls thinking, learning, problem solving, emotions, speech, reading, writing, and voluntary movement. - The cerebellum is in the lower back of the brain (near the middle of the back of the head). It controls movement, balance, and posture. - The brain stem connects the brain to the spinal cord. It is in the lowest part of the brain (just above the back of the neck). The brain stem controls breathing, heart rate, and the nerves and muscles used to see, hear, walk, talk, and eat. The spinal cord connects the brain to nerves in most parts of the body. The spinal cord is a column of nerve tissue that runs from the brain stem down the center of the back. It is covered by three thin layers of tissue called membranes. These membranes are surrounded by the vertebrae (back bones). Spinal cord nerves carry messages between the brain and the rest of the body, such as a message from the brain to cause muscles to move or a message from the skin to the brain to feel touch. There are different types of brain and spinal cord tumors. Brain and spinal cord tumors are named based on the type of cell they formed in and where the tumor first formed in the CNS. The grade of a tumor may be used to tell the difference between slow-growing and fast-growing types of the tumor. The World Health Organization (WHO) tumor grades are based on how abnormal the cancer cells look under a microscope and how quickly the tumor is likely to grow and spread. WHO Tumor Grading System - Grade I (low-grade) The tumor cells look more like normal cells under a microscope and grow and spread more slowly than grade II, III, and IV tumor cells. They rarely spread into nearby tissues. Grade I brain tumors may be cured if they are completely removed by surgery. - Grade II The tumor cells grow and spread more slowly than grade III and IV tumor cells. They may spread into nearby tissue and may recur (come back). Some tumors may become a higher-grade tumor. - Grade III The tumor cells look very different from normal cells under a microscope and grow more quickly than grade I and II tumor cells. They are likely to spread into nearby tissue. - Grade IV (high-grade) The tumor cells do not look like normal cells under a microscope and grow and spread very quickly. There may be areas of dead cells in the tumor. Grade IV tumors usually cannot be cured. The following types of primary tumors can form in the brain or spinal cord: Astrocytic Tumors An astrocytic tumor begins in star-shaped brain cells called astrocytes, which help keep nerve cells healthy. An astrocyte is a type of glial cell. Glial cells sometimes form tumors called gliomas. Astrocytic tumors include the following: - Brain stem glioma (usually high grade): A brain stem glioma forms in the brain stem, which is the part of the brain connected to the spinal cord. It is often a high-grade tumor, which spreads widely through the brain stem and is hard to cure. Brain stem gliomas are rare in adults. (See the PDQ summary on Childhood Brain Stem Glioma Treatment for more information.) - Pineal astrocytic tumor (any grade): A pineal astrocytic tumor forms in tissue around the pineal gland and may be any grade. The pineal gland is a tiny organ in the brain that makes melatonin, a hormone that helps control the sleeping and waking cycle. - Pilocytic astrocytoma (grade I): A pilocytic astrocytoma grows slowly in the brain or spinal cord. It may be in the form of a cyst and rarely spreads into nearby tissues. Pilocytic astrocytomas can often be cured. - Diffuse astrocytoma (grade II): A diffuse astrocytoma grows slowly, but often spreads into nearby tissues. The tumor cells look something like normal cells. In some cases, a diffuse astrocytoma can be cured. It is also called a low-grade diffuse astrocytoma. - Anaplastic astrocytoma (grade III): An anaplastic astrocytoma grows quickly and spreads into nearby tissues. The tumor cells look different from normal cells. This type of tumor usually cannot be cured. An anaplastic astrocytoma is also called a malignant astrocytoma or high-grade astrocytoma. - Glioblastoma (grade IV): A glioblastoma grows and spreads very quickly. The tumor cells look very different from normal cells. This type of tumor usually cannot be cured. It is also called glioblastoma multiforme. See the PDQ summary on Childhood Astrocytomas Treatment for more information about astrocytomas in children. Oligodendroglial Tumors An oligodendroglial tumor begins in brain cells called oligodendrocytes, which help keep nerve cells healthy. An oligodendrocyte is a type of glial cell. Oligodendrocytes sometimes form tumors called oligodendrogliomas. Grades of oligodendroglial tumors include the following: - Oligodendroglioma (grade II): An oligodendroglioma grows slowly, but often spreads into nearby tissues. The tumor cells look something like normal cells. In some cases, an oligodendroglioma can be cured. - Anaplastic oligodendroglioma (grade III): An anaplastic oligodendroglioma grows quickly and spreads into nearby tissues. The tumor cells look different from normal cells. This type of tumor usually cannot be cured. See the PDQ summary on Childhood Astrocytomas Treatment for more information about oligodendroglial tumors in children. Mixed Gliomas A mixed glioma is a brain tumor that has two types of tumor cells in it oligodendrocytes and astrocytes. This type of mixed tumor is called an oligoastrocytoma. - Oligoastrocytoma (grade II): An oligoastrocytoma is a slow-growing tumor. The tumor cells look something like normal cells. In some cases, an oligoastrocytoma can be cured. - Anaplastic oligoastrocytoma (grade III): An anaplastic oligoastrocytoma grows quickly and spreads into nearby tissues. The tumor cells look different from normal cells. This type of tumor has a worse prognosis than oligoastrocytoma (grade II). See the PDQ summary on Childhood Astrocytomas Treatment for more information about mixed gliomas in children. Ependymal Tumors An ependymal tumor usually begins in cells that line the fluid -filled spaces in the brain and around the spinal cord. An ependymal tumor may also be called an ependymoma. Grades of ependymomas include the following: - Ependymoma (grade I or II): A grade I or II ependymoma grows slowly and has cells that look something like normal cells. There are two types of grade I ependymoma myxopapillary ependymoma and subependymoma. A grade II ependymoma grows in a ventricle (fluid-filled space in the brain) and its connecting paths or in the spinal cord. In some cases, a grade I or II ependymoma can be cured. - Anaplastic ependymoma (grade III): An anaplastic ependymoma grows quickly and spreads into nearby tissues. The tumor cells look different from normal cells. This type of tumor usually has a worse prognosis than a grade I or II ependymoma. See the PDQ summary on Childhood Ependymoma Treatment for more information about ependymoma in children. Medulloblastomas A medulloblastoma is a type of embryonal tumor. Medulloblastomas are most common in children or young adults. See the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information about medulloblastomas in children. Pineal Parenchymal Tumors A pineal parenchymal tumor forms in parenchymal cells or pineocytes, which are the cells that make up most of the pineal gland. These tumors are different from pineal astrocytic tumors. Grades of pineal parenchymal tumors include the following: - Pineocytoma (grade II): A pineocytoma is a slow-growing pineal tumor. - Pineoblastoma (grade IV): A pineoblastoma is a rare tumor that is very likely to spread. See the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information about pineal parenchymal tumors in children. Meningeal Tumors A meningeal tumor, also called a meningioma, forms in the meninges (thin layers of tissue that cover the brain and spinal cord). It can form from different types of brain or spinal cord cells. Meningiomas are most common in adults. Types of meningeal tumors include the following: - Meningioma (grade I): A grade I meningioma is the most common type of meningeal tumor. A grade I meningioma is a slow-growing tumor. It forms most often in the dura mater. A grade I meningioma can be cured if it is completely removed by surgery. - Meningioma (grade II and III): This is a rare meningeal tumor. It grows quickly and is likely to spread within the brain and spinal cord. The prognosis is worse than a grade I meningioma because the tumor usually cannot be completely removed by surgery. A hemangiopericytoma is not a meningeal tumor but is treated like a grade II or III meningioma. A hemangiopericytoma usually forms in the dura mater. The prognosis is worse than a grade I meningioma because the tumor usually cannot be completely removed by surgery. Germ Cell Tumors A germ cell tumor forms in germ cells, which are the cells that develop into sperm in men or ova (eggs) in women. There are different types of germ cell tumors. These include germinomas, teratomas, embryonal yolk sac carcinomas, and choriocarcinomas. Germ cell tumors can be either benign or malignant. See the PDQ summary on Childhood Central Nervous System Germ Cell Tumors Treatment for more information about childhood germ cell tumors in the brain. Craniopharyngioma (Grade I) A craniopharyngioma is a rare tumor that usually forms in the center of the brain just above the pituitary gland (a pea-sized organ at the bottom of the brain that controls other glands). Craniopharyngiomas can form from different types of brain or spinal cord cells. See the PDQ summary on Childhood Craniopharyngioma Treatment for more information about craniopharyngioma in children.",Adult Central Nervous System Tumors,0000006_1,CancerGov,https://www.cancer.gov/types/brain/patient/adult-brain-treatment-pdq,C1377678,T191,Disorders Who is at risk for Adult Central Nervous System Tumors? ?,0000006_1-2,susceptibility,"Having certain genetic syndromes may increase the risk of a central nervous system tumor. Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesnt mean that you will not get cancer. Talk with your doctor if you think you may be at risk. There are few known risk factors for brain tumors. The following conditions may increase the risk of certain types of brain tumors: - Being exposed to vinyl chloride may increase the risk of glioma. - Infection with the Epstein-Barr virus, having AIDS (acquired immunodeficiency syndrome), or receiving an organ transplant may increase the risk of primary CNS lymphoma. (See the PDQ summary on Primary CNS Lymphoma for more information.) - Having certain genetic syndromes may increase the risk brain tumors: - Neurofibromatosis type 1 (NF1) or 2 (NF2). - von Hippel-Lindau disease. - Tuberous sclerosis. - Li-Fraumeni syndrome. - Turcot syndrome type 1 or 2. - Nevoid basal cell carcinoma syndrome.",Adult Central Nervous System Tumors,0000006_1,CancerGov,https://www.cancer.gov/types/brain/patient/adult-brain-treatment-pdq,C1377678,T191,Disorders What causes Adult Central Nervous System Tumors ?,0000006_1-3,causes,The cause of most adult brain and spinal cord tumors is not known.,Adult Central Nervous System Tumors,0000006_1,CancerGov,https://www.cancer.gov/types/brain/patient/adult-brain-treatment-pdq,C1377678,T191,Disorders What are the symptoms of Adult Central Nervous System Tumors ?,0000006_1-4,symptoms,"The signs and symptoms of adult brain and spinal cord tumors are not the same in every person. Signs and symptoms depend on the following: - Where the tumor forms in the brain or spinal cord. - What the affected part of the brain controls. - The size of the tumor. Signs and symptoms may be caused by CNS tumors or by other conditions, including cancer that has spread to the brain. Check with your doctor if you have any of the following: Brain Tumor Symptoms - Morning headache or headache that goes away after vomiting. - Seizures. - Vision, hearing, and speech problems. - Loss of appetite. - Frequent nausea and vomiting. - Changes in personality, mood, ability to focus, or behavior. - Loss of balance and trouble walking. - Weakness. - Unusual sleepiness or change in activity level. Spinal Cord Tumor Symptoms - Back pain or pain that spreads from the back towards the arms or legs. - A change in bowel habits or trouble urinating. - Weakness or numbness in the arms or legs. - Trouble walking.",Adult Central Nervous System Tumors,0000006_1,CancerGov,https://www.cancer.gov/types/brain/patient/adult-brain-treatment-pdq,C1377678,T191,Disorders How to diagnose Adult Central Nervous System Tumors ?,0000006_1-5,exams and tests,"Tests that examine the brain and spinal cord are used to diagnose adult brain and spinal cord tumors. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Neurological exam : A series of questions and tests to check the brain, spinal cord, and nerve function. The exam checks a persons mental status, coordination, and ability to walk normally, and how well the muscles, senses, and reflexes work. This may also be called a neuro exam or a neurologic exam. - Visual field exam: An exam to check a persons field of vision (the total area in which objects can be seen). This test measures both central vision (how much a person can see when looking straight ahead) and peripheral vision (how much a person can see in all other directions while staring straight ahead). Any loss of vision may be a sign of a tumor that has damaged or pressed on the parts of the brain that affect eyesight. - Tumor marker test : A procedure in which a sample of blood, urine, or tissue is checked to measure the amounts of certain substances made by organs, tissues, or tumor cells in the body. Certain substances are linked to specific types of cancer when found in increased levels in the body. These are called tumor markers. This test may be done to diagnose a germ cell tumor. - Gene testing : A laboratory test in which a sample of blood or tissue is tested for changes in a chromosome that has been linked with a certain type of brain tumor. This test may be done to diagnose an inherited syndrome. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - MRI (magnetic resonance imaging) with gadolinium : A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of the brain and spinal cord. A substance called gadolinium is injected into a vein. The gadolinium collects around the cancer cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI). MRI is often used to diagnose tumors in the spinal cord. Sometimes a procedure called magnetic resonance spectroscopy (MRS) is done during the MRI scan. An MRS is used to diagnose tumors, based on their chemical make-up. - SPECT scan (single photon emission computed tomography scan): A procedure that uses a special camera linked to a computer to make a 3-dimensional (3-D) picture of the brain. A very small amount of a radioactive substance is injected into a vein or inhaled through the nose. As the substance travels through the blood, the camera rotates around the head and takes pictures of the brain. Blood flow and metabolism are higher than normal in areas where cancer cells are growing. These areas will show up brighter in the picture. This procedure may be done just before or after a CT scan. SPECT is used to tell the difference between a primary tumor and a tumor that has spread to the brain from somewhere else in the body. - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the brain. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. PET is used to tell the difference between a primary tumor and a tumor that has spread to the brain from somewhere else in the body. A biopsy is also used to diagnose a brain tumor. If imaging tests show there may be a brain tumor, a biopsy is usually done. One of the following types of biopsies may be used: - Stereotactic biopsy : When imaging tests show there may be a tumor deep in the brain in a hard to reach place, a stereotactic brain biopsy may be done. This kind of biopsy uses a computer and a 3-dimensional (3-D) scanning device to find the tumor and guide the needle used to remove the tissue. A small incision is made in the scalp and a small hole is drilled through the skull. A biopsy needle is inserted through the hole to remove cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. - Open biopsy : When imaging tests show that there may be a tumor that can be removed by surgery, an open biopsy may be done. A part of the skull is removed in an operation called a craniotomy. A sample of brain tissue is removed and viewed under a microscope by a pathologist. If cancer cells are found, some or all of the tumor may be removed during the same surgery. Tests are done before surgery to find the areas around the tumor that are important for normal brain function. There are also ways to test brain function during surgery. The doctor will use the results of these tests to remove as much of the tumor as possible with the least damage to normal tissue in the brain. The pathologist checks the biopsy sample to find out the type and grade of brain tumor. The grade of the tumor is based on how the tumor cells look under a microscope and how quickly the tumor is likely to grow and spread. The following tests may be done on the tumor tissue that is removed: - Immunohistochemistry : A test that uses antibodies to check for certain antigens in a sample of tissue. The antibody is usually linked to a radioactive substance or a dye that causes the tissue to light up under a microscope. This type of test may be used to tell the difference between different types of cancer. - Light and electron microscopy : A laboratory test in which cells in a sample of tissue are viewed under regular and high-powered microscopes to look for certain changes in the cells. - Cytogenetic analysis : A laboratory test in which cells in a sample of tissue are viewed under a microscope to look for certain changes in the chromosomes. Sometimes a biopsy or surgery cannot be done. For some tumors, a biopsy or surgery cannot be done safely because of where the tumor formed in the brain or spinal cord. These tumors are diagnosed and treated based on the results of imaging tests and other procedures. Sometimes the results of imaging tests and other procedures show that the tumor is very likely to be benign and a biopsy is not done.",Adult Central Nervous System Tumors,0000006_1,CancerGov,https://www.cancer.gov/types/brain/patient/adult-brain-treatment-pdq,C1377678,T191,Disorders What is the outlook for Adult Central Nervous System Tumors ?,0000006_1-6,outlook,Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options for primary brain and spinal cord tumors depend on the following: - The type and grade of the tumor. - Where the tumor is in the brain or spinal cord. - Whether the tumor can be removed by surgery. - Whether cancer cells remain after surgery. - Whether there are certain changes in the chromosomes. - Whether the cancer has just been diagnosed or has recurred (come back). - The patient's general health. The prognosis and treatment options for metastatic brain and spinal cord tumors depend on the following: - Whether there are more than two tumors in the brain or spinal cord. - Where the tumor is in the brain or spinal cord. - How well the tumor responds to treatment. - Whether the primary tumor continues to grow or spread.,Adult Central Nervous System Tumors,0000006_1,CancerGov,https://www.cancer.gov/types/brain/patient/adult-brain-treatment-pdq,C1377678,T191,Disorders What are the stages of Adult Central Nervous System Tumors ?,0000006_1-7,stages,"Key Points - There is no standard staging system for adult brain and spinal cord tumors. - Imaging tests may be repeated after surgery to help plan more treatment. There is no standard staging system for adult brain and spinal cord tumors. The extent or spread of cancer is usually described as stages. There is no standard staging system for brain and spinal cord tumors. Brain tumors that begin in the brain may spread to other parts of the brain and spinal cord, but they rarely spread to other parts of the body. Treatment of primary brain and spinal cord tumors is based on the following: - The type of cell in which the tumor began. - Where the tumor formed in the brain or spinal cord. - The amount of cancer left after surgery. - The grade of the tumor. Treatment of tumors that have spread to the brain from other parts of the body is based on the number of tumors in the brain. Imaging tests may be repeated after surgery to help plan more treatment. Some of the tests and procedures used to diagnose a brain or spinal cord tumor may be repeated after treatment to find out how much tumor is left.",Adult Central Nervous System Tumors,0000006_1,CancerGov,https://www.cancer.gov/types/brain/patient/adult-brain-treatment-pdq,C1377678,T191,Disorders what research (or clinical trials) is being done for Adult Central Nervous System Tumors ?,0000006_1-8,research,"New types of treatment are being tested in clinical trials. This summary section refers to new treatments being studied in clinical trials, but it may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Proton beam radiation therapy Proton beam radiation therapy is a type of high-energy, external radiation therapy that uses streams of protons (small, positively-charged pieces of matter) to make radiation. This type of radiation kills tumor cells with little damage to nearby tissues. It is used to treat cancers of the head, neck, and spine and organs such as the brain, eye, lung, and prostate. Proton beam radiation is different from x-ray radiation. Biologic therapy Biologic therapy is a treatment that uses the patients immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the bodys natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy. Biologic therapy is being studied for the treatment of some types of brain tumors. Treatments may include the following: - Dendritic cell vaccine therapy. - Gene therapy. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Adult Central Nervous System Tumors,0000006_1,CancerGov,https://www.cancer.gov/types/brain/patient/adult-brain-treatment-pdq,C1377678,T191,Disorders What are the treatments for Adult Central Nervous System Tumors ?,0000006_1-9,treatment,"Key Points - There are different types of treatment for patients with adult brain and spinal cord tumors. - Five types of standard treatment are used: - Active surveillance - Surgery - Radiation therapy - Chemotherapy - Targeted therapy - Supportive care is given to lessen the problems caused by the disease or its treatment. - New types of treatment are being tested in clinical trials. - Proton beam radiation therapy - Biologic therapy - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with adult brain and spinal cord tumors. Different types of treatment are available for patients with adult brain and spinal cord tumors. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Five types of standard treatment are used: Active surveillance Active surveillance is closely watching a patients condition but not giving any treatment unless there are changes in test results that show the condition is getting worse. Active surveillance may be used to avoid or delay the need for treatments such as radiation therapy or surgery, which can cause side effects or other problems. During active surveillance, certain exams and tests are done on a regular schedule. Active surveillance may be used for very slow-growing tumors that do not cause symptoms. Surgery Surgery may be used to diagnose and treat adult brain and spinal cord tumors. Removing tumor tissue helps decrease pressure of the tumor on nearby parts of the brain. See the General Information section of this summary. Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. Certain ways of giving radiation therapy can help keep radiation from damaging nearby healthy tissue. These types of radiation therapy include the following: - Conformal radiation therapy: Conformal radiation therapy is a type of external radiation therapy that uses a computer to make a 3-dimensional (3-D) picture of the tumor and shapes the radiation beams to fit the tumor. - Intensity-modulated radiation therapy (IMRT): IMRT is a type of 3-dimensional (3-D) external radiation therapy that uses a computer to make pictures of the size and shape of the tumor. Thin beams of radiation of different intensities (strengths) are aimed at the tumor from many angles. - Stereotactic radiosurgery: Stereotactic radiosurgery is a type of external radiation therapy. A rigid head frame is attached to the skull to keep the head still during the radiation treatment. A machine aims a single large dose of radiation directly at the tumor. This procedure does not involve surgery. It is also called stereotaxic radiosurgery, radiosurgery, and radiation surgery. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and grade of tumor and where it is in the brain or spinal cord. External radiation therapy is used to treat adult central nervous system tumors. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is treatment using more than one anticancer drug. To treat brain tumors, a wafer that dissolves may be used to deliver an anticancer drug directly to the brain tumor site after the tumor has been removed by surgery. The way the chemotherapy is given depends on the type and grade of tumor and where it is in the brain. Anticancer drugs given by mouth or vein to treat brain and spinal cord tumors cannot cross the blood-brain barrier and enter the fluid that surrounds the brain and spinal cord. Instead, an anticancer drug is injected into the fluid-filled space to kill cancer cells there. This is called intrathecal chemotherapy. See Drugs Approved for Brain Tumors for more information. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Monoclonal antibody therapy is a type of targeted therapy that uses antibodies made in the laboratory from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Bevacizumab is a monoclonal antibody that binds to a protein called vascular endothelial growth factor (VEGF) and may prevent the growth of new blood vessels that tumors need to grow. Bevacizumab is used in the treatment of recurrent glioblastoma. Other types of targeted therapies are being studied for adult brain tumors, including tyrosine kinase inhibitors and new VEGF inhibitors. See Drugs Approved for Brain Tumors for more information. Supportive care is given to lessen the problems caused by the disease or its treatment. This therapy controls problems or side effects caused by the disease or its treatment and improves quality of life. For brain tumors, supportive care includes drugs to control seizures and fluid buildup or swelling in the brain. New types of treatment are being tested in clinical trials. This summary section refers to new treatments being studied in clinical trials, but it may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Proton beam radiation therapy Proton beam radiation therapy is a type of high-energy, external radiation therapy that uses streams of protons (small, positively-charged pieces of matter) to make radiation. This type of radiation kills tumor cells with little damage to nearby tissues. It is used to treat cancers of the head, neck, and spine and organs such as the brain, eye, lung, and prostate. Proton beam radiation is different from x-ray radiation. Biologic therapy Biologic therapy is a treatment that uses the patients immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the bodys natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy. Biologic therapy is being studied for the treatment of some types of brain tumors. Treatments may include the following: - Dendritic cell vaccine therapy. - Gene therapy. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. The following tests and procedures may be used to check whether a brain tumor has come back after treatment: - SPECT scan (single photon emission computed tomography scan): A procedure that uses a special camera linked to a computer to make a 3-dimensional (3-D) picture of the brain. A very small amount of a radioactive substance is injected into a vein or inhaled through the nose. As the substance travels through the blood, the camera rotates around the head and takes pictures of the brain. Blood flow and metabolism are higher than normal in areas where cancer cells are growing. These areas will show up brighter in the picture. This procedure may be done just before or after a CT scan. - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the brain. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. Treatment Options by Type of Primary Adult Brain Tumor Astrocytic Tumors Brain Stem Gliomas Treatment of brain stem gliomas may include the following: - Radiation therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with adult brain stem glioma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Pineal Astrocytic Tumors Treatment of pineal astrocytic tumors may include the following: - Surgery and radiation therapy. For high-grade tumors, chemotherapy may also be given. Check the list of NCI-supported cancer clinical trials that are now accepting patients with adult pineal gland astrocytoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Pilocytic Astrocytomas Treatment of pilocytic astrocytomas may include the following: - Surgery to remove the tumor. Radiation therapy may also be given if tumor remains after surgery. Check the list of NCI-supported cancer clinical trials that are now accepting patients with adult pilocytic astrocytoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Diffuse Astrocytomas Treatment of diffuse astrocytomas may include the following: - Surgery with or without radiation therapy. - Surgery followed by radiation therapy and chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with adult diffuse astrocytoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Anaplastic Astrocytomas Treatment of anaplastic astrocytomas may include the following: - Surgery and radiation therapy. Chemotherapy may also be given. - Surgery and chemotherapy. - A clinical trial of chemotherapy placed into the brain during surgery. - A clinical trial of a new treatment added to standard treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with adult anaplastic astrocytoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Glioblastomas Treatment of glioblastomas may include the following: - Surgery followed by radiation therapy and chemotherapy given at the same time, followed by chemotherapy alone. - Surgery followed by radiation therapy. - Chemotherapy placed into the brain during surgery. - Radiation therapy and chemotherapy given at the same time. - A clinical trial of a new treatment added to standard treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with adult glioblastoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Oligodendroglial Tumors Treatment of oligodendrogliomas may include the following: - Surgery with or without radiation therapy. Chemotherapy may be given after radiation therapy. Treatment of anaplastic oligodendroglioma may include the following: - Surgery followed by radiation therapy with or without chemotherapy. - A clinical trial of a new treatment added to standard treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with adult oligodendroglial tumors. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Mixed Gliomas Treatment of mixed gliomas may include the following: - Surgery and radiation therapy. Sometimes chemotherapy is also given. Check the list of NCI-supported cancer clinical trials that are now accepting patients with adult mixed glioma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Ependymal Tumors Treatment of grade I and grade II ependymomas may include the following: - Surgery to remove the tumor. Radiation therapy may also be given if tumor remains after surgery. Treatment of grade III anaplastic ependymoma may include the following: - Surgery and radiation therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with adult ependymal tumors. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Medulloblastomas Treatment of medulloblastomas may include the following: - Surgery and radiation therapy to the brain and spine. - A clinical trial of chemotherapy added to surgery and radiation therapy to the brain and spine Check the list of NCI-supported cancer clinical trials that are now accepting patients with adult medulloblastoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Pineal Parenchymal Tumors Treatment of pineal parenchymal tumors may include the following: - For pineocytomas, surgery and radiation therapy. - For pineoblastomas, surgery, radiation therapy, and chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with adult pineal parenchymal tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Meningeal Tumors Treatment of grade I meningiomas may include the following: - Active surveillance for tumors with no signs or symptoms. - Surgery to remove the tumor. Radiation therapy may also be given if tumor remains after surgery. - Stereotactic radiosurgery for tumors smaller than 3 centimeters. - Radiation therapy for tumors that cannot be removed by surgery. Treatment of grade II and III meningiomas and hemangiopericytoma s may include the following: - Surgery and radiation therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with adult meningeal tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Germ Cell Tumors There is no standard treatment for germ cell tumors (germinoma, embryonal carcinoma, choriocarcinoma, and teratoma). Treatment depends on what the tumor cells look like under a microscope, the tumor markers, where the tumor is in the brain, and whether it can be removed by surgery. Check the list of NCI-supported cancer clinical trials that are now accepting patients with adult central nervous system germ cell tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Craniopharyngiomas Treatment of craniopharyngiomas may include the following: - Surgery to completely remove the tumor. - Surgery to remove as much of the tumor as possible, followed by radiation therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with adult craniopharyngioma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Adult Central Nervous System Tumors,0000006_1,CancerGov,https://www.cancer.gov/types/brain/patient/adult-brain-treatment-pdq,C1377678,T191,Disorders What is (are) Childhood Brain and Spinal Cord Tumors ?,0000006_2-1,information,"Key Points - A childhood brain or spinal cord tumor is a disease in which abnormal cells form in the tissues of the brain or spinal cord. - The brain controls many important body functions. - The spinal cord connects the brain with nerves in most parts of the body. - Brain and spinal cord tumors are a common type of childhood cancer. - The cause of most childhood brain and spinal cord tumors is unknown. - The signs and symptoms of childhood brain and spinal cord tumors are not the same in every child. - Tests that examine the brain and spinal cord are used to detect (find) childhood brain and spinal cord tumors. - Most childhood brain tumors are diagnosed and removed in surgery. - Some childhood brain and spinal cord tumors are diagnosed by imaging tests. - Certain factors affect prognosis (chance of recovery). A childhood brain or spinal cord tumor is a disease in which abnormal cells form in the tissues of the brain or spinal cord. There are many types of childhood brain and spinal cord tumors. The tumors are formed by the abnormal growth of cells and may begin in different areas of the brain or spinal cord. The tumors may be benign (not cancer) or malignant (cancer). Benign brain tumors grow and press on nearby areas of the brain. They rarely spread into other tissues. Malignant brain tumors are likely to grow quickly and spread into other brain tissue. When a tumor grows into or presses on an area of the brain, it may stop that part of the brain from working the way it should. Both benign and malignant brain tumors can cause signs or symptoms and need treatment. Together, the brain and spinal cord make up the central nervous system (CNS). The brain controls many important body functions. The brain has three major parts: - The cerebrum is the largest part of the brain. It is at the top of the head. The cerebrum controls thinking, learning, problem solving, emotions, speech, reading, writing, and voluntary movement. - The cerebellum is in the lower back of the brain (near the middle of the back of the head). It controls movement, balance, and posture. - The brain stem connects the brain to the spinal cord. It is in the lowest part of the brain (just above the back of the neck). The brain stem controls breathing, heart rate, and the nerves and muscles used in seeing, hearing, walking, talking, and eating. The spinal cord connects the brain with nerves in most parts of the body. The spinal cord is a column of nerve tissue that runs from the brain stem down the center of the back. It is covered by three thin layers of tissue called membranes. These membranes are surrounded by the vertebrae (back bones). Spinal cord nerves carry messages between the brain and the rest of the body, such as a message from the brain to cause muscles to move or a message from the skin to the brain to feel touch. Brain and spinal cord tumors are a common type of childhood cancer. Although cancer is rare in children, brain and spinal cord tumors are the third most common type of childhood cancer, after leukemia and lymphoma. Brain tumors can occur in both children and adults. Treatment for children is usually different than treatment for adults. (See the PDQ summary on Adult Central Nervous System Tumors Treatment for more information about the treatment of adults.) This summary describes the treatment of primary brain and spinal cord tumors (tumors that begin in the brain and spinal cord). Treatment of metastatic brain and spinal cord tumors is not covered in this summary. Metastatic tumors are formed by cancer cells that begin in other parts of the body and spread to the brain or spinal cord.",Childhood Brain and Spinal Cord Tumors,0000006_2,CancerGov,https://www.cancer.gov/types/brain/patient/child-brain-treatment-pdq,C0037930,T191,Disorders What causes Childhood Brain and Spinal Cord Tumors ?,0000006_2-2,causes,The cause of most childhood brain and spinal cord tumors is unknown.,Childhood Brain and Spinal Cord Tumors,0000006_2,CancerGov,https://www.cancer.gov/types/brain/patient/child-brain-treatment-pdq,C0037930,T191,Disorders What are the symptoms of Childhood Brain and Spinal Cord Tumors ?,0000006_2-3,symptoms,"The signs and symptoms of childhood brain and spinal cord tumors are not the same in every child. Signs and symptoms depend on the following: - Where the tumor forms in the brain or spinal cord. - The size of the tumor. - How fast the tumor grows. - The child's age and development. Signs and symptoms may be caused by childhood brain and spinal cord tumors or by other conditions, including cancer that has spread to the brain. Check with your child's doctor if your child has any of the following: Brain Tumor Signs and Symptoms - Morning headache or headache that goes away after vomiting. - Frequent nausea and vomiting. - Vision, hearing, and speech problems. - Loss of balance and trouble walking. - Unusual sleepiness or change in activity level. - Unusual changes in personality or behavior. - Seizures. - Increase in the head size (in infants). Spinal Cord Tumor Signs and Symptoms - Back pain or pain that spreads from the back towards the arms or legs. - A change in bowel habits or trouble urinating. - Weakness in the legs. - Trouble walking. In addition to these signs and symptoms of brain and spinal cord tumors, some children are unable to reach certain growth and development milestones such as sitting up, walking, and talking in sentences.",Childhood Brain and Spinal Cord Tumors,0000006_2,CancerGov,https://www.cancer.gov/types/brain/patient/child-brain-treatment-pdq,C0037930,T191,Disorders How to diagnose Childhood Brain and Spinal Cord Tumors ?,0000006_2-4,exams and tests,"Tests that examine the brain and spinal cord are used to detect (find) childhood brain and spinal cord tumors. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Neurological exam : A series of questions and tests to check the brain, spinal cord, and nerve function. The exam checks a persons mental status, coordination, and ability to walk normally, and how well the muscles, senses, and reflexes work. This may also be called a neuro exam or a neurologic exam. - MRI (magnetic resonance imaging) with gadolinium : A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of the brain and spinal cord. A substance called gadolinium is injected into a vein. The gadolinium collects around the cancer cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Serum tumor marker test : A procedure in which a sample of blood is examined to measure the amounts of certain substances released into the blood by organs, tissues, or tumor cells in the body. Certain substances are linked to specific types of cancer when found in increased levels in the blood. These are called tumor markers. Most childhood brain tumors are diagnosed and removed in surgery. If doctors think there might be a brain tumor, a biopsy may be done to remove a sample of tissue. For tumors in the brain, the biopsy is done by removing part of the skull and using a needle to remove a sample of tissue. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are found, the doctor may remove as much tumor as safely possible during the same surgery. The pathologist checks the cancer cells to find out the type and grade of brain tumor. The grade of the tumor is based on how abnormal the cancer cells look under a microscope and how quickly the tumor is likely to grow and spread. The following test may be done on the sample of tissue that is removed: - Immunohistochemistry : A test that uses antibodies to check for certain antigens in a sample of tissue. The antibody is usually linked to a radioactive substance or a dye that causes the tissue to light up under a microscope. This type of test may be used to tell the difference between different types of cancer. Some childhood brain and spinal cord tumors are diagnosed by imaging tests. Sometimes a biopsy or surgery cannot be done safely because of where the tumor formed in the brain or spinal cord. These tumors are diagnosed based on the results of imaging tests and other procedures.",Childhood Brain and Spinal Cord Tumors,0000006_2,CancerGov,https://www.cancer.gov/types/brain/patient/child-brain-treatment-pdq,C0037930,T191,Disorders What is the outlook for Childhood Brain and Spinal Cord Tumors ?,0000006_2-5,outlook,Certain factors affect prognosis (chance of recovery). The prognosis (chance of recovery) depends on the following: - Whether there are any cancer cells left after surgery. - The type of tumor. - Where the tumor is in the body. - The child's age. - Whether the tumor has just been diagnosed or has recurred (come back).,Childhood Brain and Spinal Cord Tumors,0000006_2,CancerGov,https://www.cancer.gov/types/brain/patient/child-brain-treatment-pdq,C0037930,T191,Disorders what research (or clinical trials) is being done for Childhood Brain and Spinal Cord Tumors ?,0000006_2-7,research,"New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. High-dose chemotherapy with stem cell transplant High-dose chemotherapy with stem cell transplant is a way of giving high doses of chemotherapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the bodys blood cells. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials is available from the NCI website.",Childhood Brain and Spinal Cord Tumors,0000006_2,CancerGov,https://www.cancer.gov/types/brain/patient/child-brain-treatment-pdq,C0037930,T191,Disorders What are the treatments for Childhood Brain and Spinal Cord Tumors ?,0000006_2-8,treatment,"Key Points - There are different types of treatment for children with brain and spinal cord tumors. - Children with brain or spinal cord tumors should have their treatment planned by a team of health care providers who are experts in treating childhood brain and spinal cord tumors. - Childhood brain and spinal cord tumors may cause signs or symptoms that begin before the cancer is diagnosed and continue for months or years. - Some cancer treatments cause side effects months or years after treatment has ended. - Three types of standard treatment are used: - Surgery - Radiation therapy - Chemotherapy - New types of treatment are being tested in clinical trials. - High-dose chemotherapy with stem cell transplant - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for children with brain and spinal cord tumors. Different types of treatment are available for children with brain and spinal cord tumors. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Because cancer in children is rare, taking part in a clinical trial should be considered. Clinical trials are taking place in many parts of the country. Some clinical trials are open only to patients who have not started treatment. Children with brain or spinal cord tumors should have their treatment planned by a team of health care providers who are experts in treating childhood brain and spinal cord tumors. Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other health care providers who are experts in treating children with brain tumors and who specialize in certain areas of medicine. These may include the following specialists: - Pediatrician. - Neurosurgeon. - Neurologist. - Neuro-oncologist. - Neuropathologist. - Neuroradiologist. - Radiation oncologist. - Endocrinologist. - Psychologist. - Ophthalmologist. - Rehabilitation specialist. - Social worker. - Nurse specialist. Childhood brain and spinal cord tumors may cause signs or symptoms that begin before the cancer is diagnosed and continue for months or years. Childhood brain and spinal cord tumors may cause signs or symptoms that continue for months or years. Signs or symptoms caused by the tumor may begin before diagnosis. Signs or symptoms caused by treatment may begin during or right after treatment. Some cancer treatments cause side effects months or years after treatment has ended. These are called late effects. Late effects of cancer treatment may include the following: - Physical problems. - Changes in mood, feelings, thinking, learning, or memory. - Second cancers (new types of cancer). Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information). Three types of standard treatment are used: Surgery Surgery may be used to diagnose and treat childhood brain and spinal cord tumors. See the General Information section of this summary. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type of cancer being treated. External radiation therapy is used to treat childhood brain and spinal cord tumors. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly in the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. Anticancer drugs given by mouth or vein to treat brain and spinal cord tumors cannot cross the blood-brain barrier and enter the fluid that surrounds the brain and spinal cord. Instead, an anticancer drug is injected into the fluid-filled space to kill cancer cells there. This is called intrathecal chemotherapy. New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. High-dose chemotherapy with stem cell transplant High-dose chemotherapy with stem cell transplant is a way of giving high doses of chemotherapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the bodys blood cells. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials is available from the NCI website. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.",Childhood Brain and Spinal Cord Tumors,0000006_2,CancerGov,https://www.cancer.gov/types/brain/patient/child-brain-treatment-pdq,C0037930,T191,Disorders What is (are) Childhood Astrocytomas ?,0000006_3-1,information,"Key Points - Childhood astrocytoma is a disease in which benign (noncancer) or malignant (cancer) cells form in the tissues of the brain. - Astrocytomas may be benign (not cancer) or malignant (cancer). - The central nervous system controls many important body functions. - The cause of most childhood brain tumors is not known. - The signs and symptoms of astrocytomas are not the same in every child. - Tests that examine the brain and spinal cord are used to detect (find) childhood astrocytomas. - Childhood astrocytomas are usually diagnosed and removed in surgery. - Certain factors affect prognosis (chance of recovery) and treatment options. Childhood astrocytoma is a disease in which benign (noncancer) or malignant (cancer) cells form in the tissues of the brain. Astrocytomas are tumors that start in star-shaped brain cells called astrocytes. An astrocyte is a type of glial cell. Glial cells hold nerve cells in place, bring food and oxygen to them, and help protect them from disease, such as infection. Gliomas are tumors that form from glial cells. An astrocytoma is a type of glioma. Astrocytoma is the most common type of glioma diagnosed in children. It can form anywhere in the central nervous system (brain and spinal cord). This summary is about the treatment of tumors that begin in astrocytes in the brain (primary brain tumors). Metastatic brain tumors are formed by cancer cells that begin in other parts of the body and spread to the brain. Treatment of metastatic brain tumors is not discussed here. Brain tumors can occur in both children and adults. However, treatment for children may be different than treatment for adults. See the following PDQ summaries for more information about other types of brain tumors in children and adults: - Childhood Brain and Spinal Cord Tumors Treatment Overview - Adult Central Nervous System Tumors Treatment Astrocytomas may be benign (not cancer) or malignant (cancer). Benign brain tumors grow and press on nearby areas of the brain. They rarely spread into other tissues. Malignant brain tumors are likely to grow quickly and spread into other brain tissue. When a tumor grows into or presses on an area of the brain, it may stop that part of the brain from working the way it should. Both benign and malignant brain tumors can cause signs and symptoms and almost all need treatment. The central nervous system controls many important body functions. Astrocytomas are most common in these parts of the central nervous system (CNS): - Cerebrum : The largest part of the brain, at the top of the head. The cerebrum controls thinking, learning, problem-solving, speech, emotions, reading, writing, and voluntary movement. - Cerebellum : The lower, back part of the brain (near the middle of the back of the head). The cerebellum controls movement, balance, and posture. - Brain stem : The part that connects the brain to the spinal cord, in the lowest part of the brain (just above the back of the neck). The brain stem controls breathing, heart rate, and the nerves and muscles used in seeing, hearing, walking, talking, and eating. - Hypothalamus : The area in the middle of the base of the brain. It controls body temperature, hunger, and thirst. - Visual pathway: The group of nerves that connect the eye with the brain. - Spinal cord: The column of nerve tissue that runs from the brain stem down the center of the back. It is covered by three thin layers of tissue called membranes. The spinal cord and membranes are surrounded by the vertebrae (back bones). Spinal cord nerves carry messages between the brain and the rest of the body, such as a message from the brain to cause muscles to move or a message from the skin to the brain to feel touch.",Childhood Astrocytomas,0000006_3,CancerGov,https://www.cancer.gov/types/brain/patient/child-astrocytoma-treament-pdq,C1321865,T191,Disorders What causes Childhood Astrocytomas ?,0000006_3-2,causes,The cause of most childhood brain tumors is not known.,Childhood Astrocytomas,0000006_3,CancerGov,https://www.cancer.gov/types/brain/patient/child-astrocytoma-treament-pdq,C1321865,T191,Disorders Who is at risk for Childhood Astrocytomas? ?,0000006_3-3,susceptibility,"Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesnt mean that you will not get cancer. Talk with your child's doctor if you think your child may be at risk. Possible risk factors for astrocytoma include: - Past radiation therapy to the brain. - Having certain genetic disorders, such as neurofibromatosis type 1 (NF1) or tuberous sclerosis.",Childhood Astrocytomas,0000006_3,CancerGov,https://www.cancer.gov/types/brain/patient/child-astrocytoma-treament-pdq,C1321865,T191,Disorders What are the symptoms of Childhood Astrocytomas ?,0000006_3-4,symptoms,"The signs and symptoms of astrocytomas are not the same in every child. Signs and symptoms depend on the following: - Where the tumor forms in the brain or spinal cord. - The size of the tumor. - How fast the tumor grows. - The child's age and development. Some tumors do not cause signs or symptoms. Signs and symptoms may be caused by childhood astrocytomas or by other conditions. Check with your child's doctor if your child has any of the following: - Morning headache or headache that goes away after vomiting. - Nausea and vomiting. - Vision, hearing, and speech problems. - Loss of balance and trouble walking. - Worsening handwriting or slow speech. - Weakness or change in feeling on one side of the body. - Unusual sleepiness. - More or less energy than usual. - Change in personality or behavior. - Seizures. - Weight loss or weight gain for no known reason. - Increase in the size of the head (in infants).",Childhood Astrocytomas,0000006_3,CancerGov,https://www.cancer.gov/types/brain/patient/child-astrocytoma-treament-pdq,C1321865,T191,Disorders How to diagnose Childhood Astrocytomas ?,0000006_3-5,exams and tests,"Tests that examine the brain and spinal cord are used to detect (find) childhood astrocytomas. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health. This includes checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Neurological exam : A series of questions and tests to check the brain, spinal cord, and nerve function. The exam checks a persons mental status, coordination, and ability to walk normally, and how well the muscles, senses, and reflexes work. This may also be called a neuro exam or a neurologic exam. - Visual field exam: An exam to check a persons field of vision (the total area in which objects can be seen). This test measures both central vision (how much a person can see when looking straight ahead) and peripheral vision (how much a person can see in all other directions while staring straight ahead). The eyes are tested one at a time. The eye not being tested is covered. - MRI (magnetic resonance imaging) with gadolinium : A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of the brain and spinal cord. A substance called gadolinium is injected into a vein. The gadolinium collects around the cancer cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI). Sometimes magnetic resonance spectroscopy (MRS) is done during the same MRI scan to look at the chemical makeup of the brain tissue. Childhood astrocytomas are usually diagnosed and removed in surgery. If doctors think there may be an astrocytoma, a biopsy may be done to remove a sample of tissue. For tumors in the brain, a part of the skull is removed and a needle is used to remove tissue. Sometimes, the needle is guided by a computer. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are found, the doctor may remove as much tumor as safely possible during the same surgery. Because it can be hard to tell the difference between types of brain tumors, you may want to have your child's tissue sample checked by a pathologist who has experience in diagnosing brain tumors. The following test may be done on the tissue that was removed: - Immunohistochemistry : A test that uses antibodies to check for certain antigens in a sample of tissue. The antibody is usually linked to a radioactive substance or a dye that causes the tissue to light up under a microscope. This type of test may be used to tell the difference between different types of cancer. An MIB-1 test is a type of immunohistochemistry that checks tumor tissue for an antigen called MIB-1. This may show how fast a tumor is growing. Sometimes tumors form in a place that makes them hard to remove. If removing the tumor may cause severe physical, emotional, or learning problems, a biopsy is done and more treatment is given after the biopsy. Children who have NF1 may form a low-grade astrocytoma in the area of the brain that controls vision and may not need a biopsy. If the tumor does not continue to grow or symptoms do not occur, surgery to remove the tumor may not be needed.",Childhood Astrocytomas,0000006_3,CancerGov,https://www.cancer.gov/types/brain/patient/child-astrocytoma-treament-pdq,C1321865,T191,Disorders What is the outlook for Childhood Astrocytomas ?,0000006_3-6,outlook,"Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - Whether the tumor is a low-grade or high-grade astrocytoma. - Where the tumor has formed in the CNS and if it has spread to nearby tissue or to other parts of the body. - How fast the tumor is growing. - The child's age. - Whether cancer cells remain after surgery. - Whether there are changes in certain genes. - Whether the child has NF1 or tuberous sclerosis. - Whether the child has diencephalic syndrome (a condition which slows physical growth). - Whether the child has intracranial hypertension (cerebrospinal fluid pressure within the skull is high) at the time of diagnosis. - Whether the astrocytoma has just been diagnosed or has recurred (come back). For recurrent astrocytoma, prognosis and treatment depend on how much time passed between the time treatment ended and the time the astrocytoma recurred.",Childhood Astrocytomas,0000006_3,CancerGov,https://www.cancer.gov/types/brain/patient/child-astrocytoma-treament-pdq,C1321865,T191,Disorders What are the stages of Childhood Astrocytomas ?,0000006_3-7,stages,"Key Points - The grade of the tumor is used to plan cancer treatment. - Low-grade astrocytomas - High-grade astrocytomas - An MRI is done after surgery. The grade of the tumor is used to plan cancer treatment. Staging is the process used to find out how much cancer there is and if cancer has spread. It is important to know the stage in order to plan treatment. There is no standard staging system for childhood astrocytoma. Treatment is based on the following: - Whether the tumor is low grade or high grade. - Whether the tumor is newly diagnosed or recurrent (has come back after treatment). The grade of the tumor describes how abnormal the cancer cells look under a microscope and how quickly the tumor is likely to grow and spread. The following grades are used: Low-grade astrocytomas Low-grade astrocytomas are slow-growing and rarely spread to other parts of the brain and spinal cord or other parts of the body. There are many types of low-grade astrocytomas. Low-grade astrocytomas can be either: - Grade I tumors pilocytic astrocytoma, subependymal giant cell tumor, or angiocentric glioma. - Grade II tumors diffuse astrocytoma, pleomorphic xanthoastrocytoma, or choroid glioma of the third ventricle. Children who have neurofibromatosis type 1 may have more than one low-grade tumor in the brain. Children who have tuberous sclerosis have an increased risk of subependymal giant cell astrocytoma. High-grade astrocytomas High-grade astrocytomas are fast-growing and often spread within the brain and spinal cord. There are several types of high-grade astrocytomas. High grade astrocytomas can be either: - Grade III tumors anaplastic astrocytoma or anaplastic pleomorphic xanthoastrocytoma. - Grade IV tumors glioblastoma or diffuse midline glioma. Childhood astrocytomas usually do not spread to other parts of the body. An MRI is done after surgery. An MRI (magnetic resonance imaging) is done in the first few days after surgery. This is to find out how much tumor, if any, remains after surgery and to plan further treatment.",Childhood Astrocytomas,0000006_3,CancerGov,https://www.cancer.gov/types/brain/patient/child-astrocytoma-treament-pdq,C1321865,T191,Disorders what research (or clinical trials) is being done for Childhood Astrocytomas ?,0000006_3-8,research,"New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Other drug therapy Lenalidomide is a type of angiogenesis inhibitor. It prevents the growth of new blood vessels that are needed by a tumor to grow. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Childhood Astrocytomas,0000006_3,CancerGov,https://www.cancer.gov/types/brain/patient/child-astrocytoma-treament-pdq,C1321865,T191,Disorders What are the treatments for Childhood Astrocytomas ?,0000006_3-9,treatment,"Key Points - There are different types of treatment for patients with childhood astrocytoma. - Children with astrocytomas should have their treatment planned by a team of health care providers who are experts in treating childhood brain tumors. - Childhood brain tumors may cause signs or symptoms that begin before the cancer is diagnosed and continue for months or years. - Some cancer treatments cause side effects months or years after treatment has ended. - Six types of treatment are used: - Surgery - Observation - Radiation therapy - Chemotherapy - High-dose chemotherapy with stem cell transplant - Targeted therapy - New types of treatment are being tested in clinical trials. - Other drug therapy - If fluid builds up around the brain and spinal cord, a cerebrospinal fluid diversion procedure may be done. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with childhood astrocytoma. Different types of treatment are available for children with astrocytomas. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment. Children with astrocytomas should have their treatment planned by a team of health care providers who are experts in treating childhood brain tumors. Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other healthcare providers who are experts in treating children with brain tumors and who specialize in certain areas of medicine. These may include the following specialists: - Pediatrician. - Pediatric neurosurgeon. - Neurologist. - Neuropathologist. - Neuroradiologist. - Rehabilitation specialist. - Radiation oncologist. - Endocrinologist. - Psychologist. Childhood brain tumors may cause signs or symptoms that begin before the cancer is diagnosed and continue for months or years. Signs or symptoms caused by the tumor may begin before diagnosis. These signs or symptoms may continue for months or years. It is important to talk with your child's doctors about signs or symptoms caused by the tumor that may continue after treatment. Some cancer treatments cause side effects months or years after treatment has ended. Side effects from cancer treatment that begin during or after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include the following: - Physical problems. - Changes in mood, feelings, thinking, learning, or memory. - Second cancers (new types of cancer). Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information.) Six types of treatment are used: Surgery Surgery is used to diagnose and treat childhood astrocytoma, as discussed in the General Information section of this summary. If cancer cells remain after surgery, further treatment depends on: - Where the remaining cancer cells are. - The grade of the tumor. - The age of the child. Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that remain. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Observation Observation is closely monitoring a patients condition without giving any treatment until signs or symptoms appear or change. Observation may be used: - If the patient has no symptoms, such as patients with neurofibromatosis type1. - If the tumor is small and is found when a different health problem is being diagnosed or treated. - After the tumor is removed by surgery until signs or symptoms appear or change. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. Certain ways of giving radiation therapy can help keep radiation from damaging nearby healthy tissue. These types of radiation therapy include the following: - Conformal radiation therapy: Conformal radiation therapy is a type of external radiation therapy that uses a computer to make a 3-dimensional (3-D) picture of the tumor and shapes the radiation beams to fit the tumor. - Intensity-modulated radiation therapy (IMRT): IMRT is a type of 3-dimensional (3-D) external radiation therapy that uses a computer to make pictures of the size and shape of the tumor. Thin beams of radiation of different intensities (strengths) are aimed at the tumor from many angles. - Stereotactic radiation therapy: Stereotactic radiation therapy is a type of external radiation therapy. A rigid head frame is attached to the skull to keep the head still during the radiation treatment. A machine aims radiation directly at the tumor. The total dose of radiation is divided into several smaller doses given over several days. This procedure is also called stereotactic external-beam radiation therapy and stereotaxic radiation therapy. - Proton beam radiation therapy: Proton-beam therapy is a type of high-energy, external radiation therapy. A radiation therapy machine aims streams of protons (tiny, invisible, positively-charged particles) at the cancer cells to kill them. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type of tumor and where the tumor formed in the brain or spinal cord. External radiation therapy is used to treat childhood astrocytomas. Radiation therapy to the brain can affect growth and development, especially in young children. For children younger than 3 years, chemotherapy may be given instead, to delay or reduce the need for radiation therapy. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is the use of more than one anticancer drug. The way the chemotherapy is given depends on the type of tumor and where the tumor formed in the brain or spinal cord. Systemic combination chemotherapy is used in the treatment of children with astrocytoma. High-dose chemotherapy may be used in the treatment of children with newly diagnosed high-grade astrocytoma. High-dose chemotherapy with stem cell transplant High-dose chemotherapy with stem cell transplant is a way of giving high doses of chemotherapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. For high-grade astrocytoma that has come back after treatment, high-dose chemotherapy with stem cell transplant is used if there is only a small amount of tumor. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. There are different types of targeted therapy: - Monoclonal antibody therapy uses antibodies made in the laboratory, from a single type of immune system cell, to stop cancer cells. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion into a vein. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. There are different types of monoclonal antibody therapy: - Vascular endothelial growth factor (VEGF) inhibitor therapy: Cancer cells make a substance called VEGF, which causes new blood vessels to form (angiogenesis) and helps the cancer grow. VEGF inhibitors block VEGF and stop new blood vessels from forming. This may kill cancer cells because they need new blood vessels to grow. Bevacizumab is a VEGF inhibitor and angiogenesis inhibitor being used to treat childhood astrocytoma. - Immune checkpoint inhibitor therapy: PD-1 is a protein on the surface of T cells that helps keep the bodys immune responses in check. When PD-1 attaches to another protein called PDL-1 on a cancer cell, it stops the T cell from killing the cancer cell. PD-1 inhibitors attach to PDL-1 and allow the T cells to kill cancer cells. PD-1 inhibitors are being studied to treat high-grade astrocytoma that has recurred. - Protein kinase inhibitors work in different ways. There are several kinds of protein kinase inhibitors. - mTOR inhibitors stop cells from dividing and may prevent the growth of new blood vessels that tumors need to grow. Everolimus and sirolimus are mTOR inhibitors used to treat childhood subependymal giant cell astrocytomas. mTOR inhibitors also are being studied to treat low-grade astrocytoma that has recurred. - BRAF inhibitors block proteins needed for cell growth and may kill cancer cells. The BRAF inhibitor dabrafenib is being studied to treat low-grade astrocytoma that has recurred. Vemurafenib and dabrafenib have been used to treat high-grade astrocytomas that have recurred but more study is needed to know how well they work in children. - MEK inhibitors block proteins needed for cell growth and may kill cancer cells. MEK inhibitors such as selumetinib are being studied to treat low-grade astrocytoma that has recurred. See Drugs Approved for Brain Tumors for more information. New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Other drug therapy Lenalidomide is a type of angiogenesis inhibitor. It prevents the growth of new blood vessels that are needed by a tumor to grow. If fluid builds up around the brain and spinal cord, a cerebrospinal fluid diversion procedure may be done. Cerebrospinal fluid diversion is a method used to drain fluid that has built up around the brain and spinal cord. A shunt (long, thin tube) is placed in a ventricle (fluid-filled space) of the brain and threaded under the skin to another part of the body, usually the abdomen. The shunt carries extra fluid away from the brain so it may be absorbed elsewhere in the body. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. (See the General Information section for a list of tests.) Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Regular MRIs will continue to be done after treatment has ended. The results of the MRI can show if your child's condition has changed or if the astrocytoma has recurred (come back). If the results of the MRI show a mass in the brain, a biopsy may be done to find out if it is made up of dead tumor cells or if new cancer cells are growing. Treatment Options for Childhood Astrocytomas Newly Diagnosed Childhood Low-Grade Astrocytomas When the tumor is first diagnosed, treatment for childhood low-grade astrocytoma depends on where the tumor is, and is usually surgery. An MRI is done after surgery to see if there is tumor remaining. If the tumor was completely removed by surgery, more treatment may not be needed and the child is closely watched to see if signs or symptoms appear or change. This is called observation. If there is tumor remaining after surgery, treatment may include the following: - Observation. - A second surgery to remove the tumor. - Radiation therapy, which may include conformal radiation therapy, intensity-modulated radiation therapy, proton beam radiation therapy, or stereotactic radiation therapy, when the tumor begins to grow again. - Combination chemotherapy with or without radiation therapy. In some cases, observation is used for children who have a visual pathway glioma. In other cases, treatment may include surgery to remove the tumor, radiation therapy, or chemotherapy. A goal of treatment is to save as much vision as possible. The effect of tumor growth on the child's vision will be closely followed during treatment. Children with neurofibromatosis type 1 (NF1) may not need treatment unless the tumor grows or signs or symptoms, such as vision problems, appear. When the tumor grows or signs or symptoms appear, treatment may include surgery to remove the tumor, radiation therapy, and/or chemotherapy. Children with tuberous sclerosis may develop benign (not cancer) tumors in the brain called subependymal giant cell astrocytomas (SEGAs). Targeted therapy with everolimus or sirolimus may be used instead of surgery, to shrink the tumors. Check the list of NCI-supported cancer clinical trials that are now accepting patients with childhood low-grade untreated astrocytoma or other tumor of glial origin. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Recurrent Childhood Low-Grade Astrocytomas When low-grade astrocytoma recurs after treatment, it usually comes back where the tumor first formed. Before more cancer treatment is given, imaging tests, biopsy, or surgery are done to find out if there is cancer and how much there is. Treatment of recurrent childhood low-grade astrocytoma may include the following: - A second surgery to remove the tumor, if surgery was the only treatment given when the tumor was first diagnosed. - Radiation therapy to the tumor only, if radiation therapy was not used when the tumor was first diagnosed. Conformal radiation therapy may be given. - Chemotherapy, if the tumor recurred where it cannot be removed by surgery or the patient had radiation therapy when the tumor was first diagnosed. - Targeted therapy with a monoclonal antibody (bevacizumab) with or without chemotherapy. - A clinical trial of targeted therapy with a BRAF inhibitor (dabrafenib), an mTOR inhibitor (everolimus), or a MEK inhibitor (selumetinib). - A clinical trial of lenalidomide. Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent childhood astrocytoma or other tumor of glial origin. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Newly Diagnosed Childhood High-Grade Astrocytomas Treatment of childhood high-grade astrocytoma may include the following: - Surgery to remove the tumor, followed by chemotherapy and/or radiation therapy. - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with childhood high-grade untreated astrocytoma or other tumor of glial origin. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Recurrent Childhood High-Grade Astrocytomas When high-grade astrocytoma recurs after treatment, it usually comes back where the tumor first formed. Before more cancer treatment is given, imaging tests, biopsy, or surgery are done to find out if there is cancer and how much there is. Treatment of recurrent childhood high-grade astrocytoma may include the following: - Surgery to remove the tumor. - High-dose chemotherapy with stem cell transplant. - Targeted therapy with a BRAF inhibitor (vemurafenib or dabrafenib). - A clinical trial of targeted therapy with an immune checkpoint inhibitor. - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent childhood astrocytoma or other tumor of glial origin. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website.",Childhood Astrocytomas,0000006_3,CancerGov,https://www.cancer.gov/types/brain/patient/child-astrocytoma-treament-pdq,C1321865,T191,Disorders What is (are) Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor ?,0000006_4-1,information,"Key Points - Central nervous system atypical teratoid/rhabdoid tumor is a disease in which malignant (cancer) cells form in the tissues of the brain. - Certain genetic changes may increase the risk of atypical teratoid/rhabdoid tumor. - The signs and symptoms of atypical teratoid/rhabdoid tumor are not the same in every patient. - Tests that examine the brain and spinal cord are used to detect (find) CNS atypical teratoid/rhabdoid tumor. - Childhood atypical teratoid/rhabdoid tumor is diagnosed and may be removed in surgery. - Certain factors affect prognosis (chance of recovery) and treatment options. Central nervous system atypical teratoid/rhabdoid tumor is a disease in which malignant (cancer) cells form in the tissues of the brain. Central nervous system (CNS) atypical teratoid/rhabdoid tumor (AT/RT) is a very rare, fast-growing tumor of the brain and spinal cord. It usually occurs in children aged three years and younger, although it can occur in older children and adults. About half of these tumors form in the cerebellum or brain stem. The cerebellum is the part of the brain that controls movement, balance, and posture. The brain stem controls breathing, heart rate, and the nerves and muscles used in seeing, hearing, walking, talking, and eating. AT/RT may also be found in other parts of the central nervous system (brain and spinal cord). This summary describes the treatment of primary brain tumors (tumors that begin in the brain). Treatment for metastatic brain tumors, which are tumors formed by cancer cells that begin in other parts of the body and spread to the brain, is not covered in this summary. For more information, see the PDQ summary on Childhood Brain and Spinal Cord Tumors Treatment Overview about the different types of childhood brain and spinal cord tumors. Brain tumors can occur in both children and adults; however, treatment for children may be different than treatment for adults. See the PDQ treatment summary on Adult Central Nervous System Tumors Treatment for more information.",Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor,0000006_4,CancerGov,https://www.cancer.gov/types/brain/patient/child-cns-atrt-treatment-pdq,C1266184,T191,Disorders Who is at risk for Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor? ?,0000006_4-2,susceptibility,"Certain genetic changes may increase the risk of atypical teratoid/rhabdoid tumor. Anything that increases the risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesnt mean that you will not get cancer. Talk with your child's doctor if you think your child may be at risk. Atypical teratoid/rhabdoid tumor may be linked to changes in the tumor suppressor genes SMARCB1 or SMARCA4. Genes of this type make a protein that helps control cell growth. Changes in the DNA of tumor suppressor genes like SMARCB1 or SMARCA4 may lead to cancer. Changes in the SMARCB1 or SMARCA4 genes may be inherited (passed on from parents to offspring). When this gene change is inherited, tumors may form in two parts of the body at the same time (for example, in the brain and the kidney). For patients with AT/RT, genetic counseling (a discussion with a trained professional about inherited diseases and a possible need for gene testing) may be recommended.",Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor,0000006_4,CancerGov,https://www.cancer.gov/types/brain/patient/child-cns-atrt-treatment-pdq,C1266184,T191,Disorders What are the symptoms of Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor ?,0000006_4-3,symptoms,"The signs and symptoms of atypical teratoid/rhabdoid tumor are not the same in every patient. Signs and symptoms depend on the following: - The child's age. - Where the tumor has formed. Because atypical teratoid/rhabdoid tumor is fast growing, signs and symptoms may develop quickly and get worse over a period of days or weeks. Signs and symptoms may be caused by AT/RT or by other conditions. Check with your child's doctor if your child has any of the following: - Morning headache or headache that goes away after vomiting. - Nausea and vomiting. - Unusual sleepiness or change in activity level. - Loss of balance, lack of coordination, or trouble walking. - Increase in head size (in infants).",Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor,0000006_4,CancerGov,https://www.cancer.gov/types/brain/patient/child-cns-atrt-treatment-pdq,C1266184,T191,Disorders How to diagnose Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor ?,0000006_4-4,exams and tests,"Tests that examine the brain and spinal cord are used to detect (find) CNS atypical teratoid/rhabdoid tumor. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient's health habits and past illnesses and treatments will also be taken. - Neurological exam : A series of questions and tests to check the brain, spinal cord, and nerve function. The exam checks a persons mental status, coordination, and ability to walk normally, and how well the muscles, senses, and reflexes work. This may also be called a neuro exam or a neurologic exam. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the brain and spinal cord. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Lumbar puncture : A procedure used to collect cerebrospinal fluid (CSF) from the spinal column. This is done by placing a needle between two bones in the spine and into the CSF around the spinal cord and removing a sample of fluid. The sample of CSF is checked under a microscope for signs of tumor cells. The sample may also be checked for the amounts of protein and glucose. A higher than normal amount of protein or lower than normal amount of glucose may be a sign of a tumor. This procedure is also called an LP or spinal tap. - SMARCB1 and SMARCA4 gene testing: A laboratory test in which a sample of blood or tissue is tested for the SMARCB1 and SMARCA4 genes. Childhood atypical teratoid/rhabdoid tumor is diagnosed and may be removed in surgery. If doctors think there might be a brain tumor, a biopsy may be done to remove a sample of tissue. For tumors in the brain, the biopsy is done by removing part of the skull and using a needle to remove a sample of tissue. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are found, the doctor may remove as much tumor as safely possible during the same surgery. The pathologist checks the cancer cells to find out the type of brain tumor. It is often difficult to completely remove AT/RT because of where the tumor is in the brain and because it may already have spread at the time of diagnosis. The following test may be done on the sample of tissue that is removed: - Immunohistochemistry : A test that uses antibodies to check for certain antigens in a sample of tissue. The antibody is usually linked to a radioactive substance or a dye that causes the tissue to light up under a microscope. This test is used to tell the difference between AT/RT and other brain tumors.",Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor,0000006_4,CancerGov,https://www.cancer.gov/types/brain/patient/child-cns-atrt-treatment-pdq,C1266184,T191,Disorders What is the outlook for Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor ?,0000006_4-5,outlook,Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - Whether there are certain inherited gene changes. - The age of the child. - The amount of tumor remaining after surgery. - Whether the cancer has spread to other parts of the central nervous system (brain and spinal cord) or to the kidney at the time of diagnosis.,Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor,0000006_4,CancerGov,https://www.cancer.gov/types/brain/patient/child-cns-atrt-treatment-pdq,C1266184,T191,Disorders What are the stages of Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor ?,0000006_4-6,stages,"Key Points - There is no standard staging system for central nervous system atypical teratoid/rhabdoid tumor. There is no standard staging system for central nervous system atypical teratoid/rhabdoid tumor. The extent or spread of cancer is usually described as stages. There is no standard staging system for central nervous system atypical teratoid/rhabdoid tumor. For treatment, this tumor is grouped as newly diagnosed or recurrent. Treatment depends on the following: - The age of the child. - How much cancer remains after surgery to remove the tumor. Results from the following procedure are also used to plan treatment: - Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs, such as the kidney, and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later. This procedure is done to check for tumors that may also have formed in the kidney.",Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor,0000006_4,CancerGov,https://www.cancer.gov/types/brain/patient/child-cns-atrt-treatment-pdq,C1266184,T191,Disorders what research (or clinical trials) is being done for Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor ?,0000006_4-7,research,"New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. Targeted therapy is being studied in the treatment of recurrent childhood central nervous system atypical teratoid/rhabdoid tumor. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor,0000006_4,CancerGov,https://www.cancer.gov/types/brain/patient/child-cns-atrt-treatment-pdq,C1266184,T191,Disorders What are the treatments for Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor ?,0000006_4-8,treatment,"Key Points - There are different types of treatment for patients with central nervous system atypical teratoid/rhabdoid tumor. - Children with atypical teratoid/rhabdoid tumor should have their treatment planned by a team of health care providers who are experts in treating cancer in children. - Childhood brain tumors may cause signs or symptoms that begin before the cancer is diagnosed and continue for months or years. - Some cancer treatments cause side effects months or years after treatment has ended. - Four types of treatment are used: - Surgery - Chemotherapy - Radiation therapy - High-dose chemotherapy with stem cell transplant - New types of treatment are being tested in clinical trials. - Targeted therapy - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with central nervous system atypical teratoid/rhabdoid tumor. Different types of treatment are available for patients with central nervous system atypical teratoid/rhabdoid tumor (AT/RT). Treatment for AT/RT is usually within a clinical trial. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. Clinical trials are taking place in many parts of the country. Information about ongoing clinical trials is available from the NCI website. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team. Children with atypical teratoid/rhabdoid tumor should have their treatment planned by a team of health care providers who are experts in treating cancer in children. Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other pediatric health care providers who are experts in treating children with central nervous system cancer and who specialize in certain areas of medicine. These may include the following specialists: - Pediatrician. - Pediatric neurosurgeon. - Radiation oncologist. - Neurologist. - Pediatric nurse specialist. - Rehabilitation specialist. - Psychologist. - Social worker. - Geneticist or genetic counselor. Childhood brain tumors may cause signs or symptoms that begin before the cancer is diagnosed and continue for months or years. Signs or symptoms caused by the tumor may begin before diagnosis. These signs or symptoms may continue for months or years. It is important to talk with your child's doctors about signs or symptoms caused by the tumor that may continue after treatment. Some cancer treatments cause side effects months or years after treatment has ended. Side effects from cancer treatment that begin during or after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include the following: - Physical problems. - Changes in mood, feelings, thinking, learning, or memory. - Second cancers (new types of cancer). Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information). Four types of treatment are used: Surgery Surgery is used to diagnose and treat CNS atypical teratoid/rhabdoid tumor. See the General Information section of this summary. Even if the doctor removes all the cancer that can be seen at the time of the surgery, most patients will be given chemotherapy and possibly radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. - When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect tumor cells in those areas (regional chemotherapy). Regular doses of anticancer drugs given by mouth or vein to treat brain and spinal cord tumors cannot cross the blood-brain barrier and reach the tumor. Anticancer drugs injected into the cerebrospinal fluid are able to reach the tumor. This is called intrathecal chemotherapy. - When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach tumor cells throughout the body (systemic chemotherapy). High doses of some anticancer drugs given into a vein can cross the blood-brain barrier and reach the tumor. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type of tumor being treated and whether it has spread. External radiation therapy may be given to the brain and spinal cord. Because radiation therapy can affect growth and brain development in young children, especially children who are three years old or younger, the dose of radiation therapy may be lower than in older children. High-dose chemotherapy with stem cell transplant High-dose chemotherapy with stem cell transplant is a method of giving high doses of chemotherapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the bodys blood cells. New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. Targeted therapy is being studied in the treatment of recurrent childhood central nervous system atypical teratoid/rhabdoid tumor. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Newly Diagnosed Childhood CNS Atypical Teratoid/Rhabdoid Tumor Key Points - There is no standard treatment for patients with central nervous system atypical teratoid/rhabdoid tumor. - Combinations of treatments are used for patients with atypical teratoid/rhabdoid tumor. There is no standard treatment for patients with central nervous system atypical teratoid/rhabdoid tumor. Combinations of treatments are used for patients with atypical teratoid/rhabdoid tumor. Because atypical teratoid/rhabdoid tumor (AT/RT) is fast-growing, a combination of treatments is usually given. After surgery to remove the tumor, treatments for AT/RT may include combinations of the following: - Chemotherapy. - Radiation therapy. - High-dose chemotherapy with stem cell transplant. Clinical trials of new treatments should be considered for patients with newly diagnosed atypical teratoid/rhabdoid tumor.",Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor,0000006_4,CancerGov,https://www.cancer.gov/types/brain/patient/child-cns-atrt-treatment-pdq,C1266184,T191,Disorders What is (are) Childhood Brain Stem Glioma ?,0000006_5-1,information,"Key Points - Childhood brain stem glioma is a disease in which benign (noncancer) or malignant (cancer) cells form in the tissues of the brain stem. - Brain tumors may be benign (not cancer) or malignant (cancer). - There are two types of brain stem gliomas in children. - The cause of most childhood brain tumors is unknown. - The signs and symptoms of brain stem glioma are not the same in every child. - Tests that examine the brain are used to detect (find) childhood brain stem glioma. - A biopsy may be done to diagnose certain types of brain stem glioma. - Certain factors affect prognosis (chance of recovery) and treatment options. Childhood brain stem glioma is a disease in which benign (noncancer) or malignant (cancer) cells form in the tissues of the brain stem. Gliomas are tumors formed from glial cells. Glial cells in the brain hold nerve cells in place, bring food and oxygen to them, and help protect them from disease, such as infection. The brain stem is the part of the brain connected to the spinal cord. It is in the lowest part of the brain, just above the back of the neck. The brain stem is the part of the brain that controls breathing, heart rate, and the nerves and muscles used in seeing, hearing, walking, talking, and eating. Most childhood brain stem gliomas are pontine gliomas, which form in a part of the brain stem called the pons. Brain tumors are the third most common type of cancer in children. This summary refers to the treatment of primary brain tumors (tumors that begin in the brain). Treatment for metastatic brain tumors, which are tumors formed by cancer cells that begin in other parts of the body and spread to the brain, is not discussed in this summary. Brain tumors can occur in both children and adults; however, treatment for children may be different than treatment for adults. See the following PDQ treatment summaries for more information: - Childhood Brain and Spinal Cord Tumors Treatment Overview - Adult Central Nervous System Tumors Treatment Brain tumors may be benign (not cancer) or malignant (cancer). Benign brain tumors grow and press on nearby areas of the brain. They rarely spread into other tissues. Malignant brain tumors are likely to grow quickly and spread into other brain tissue. When a tumor grows into or presses on an area of the brain, it may stop that part of the brain from working the way it should. Both benign and malignant brain tumors can cause signs and symptoms and need treatment. There are two types of brain stem gliomas in children. Childhood brain stem glioma is either a diffuse intrinsic pontine glioma (DIPG) or a focal glioma. - DIPG is a high-grade tumor that is fast-growing and spreads all through the brain stem. It is hard to treat and has a poor prognosis (chance of recovery). Children younger than 3 years diagnosed with DIPG may have a better prognosis than children who are 3 years and older. - A focal glioma is slow-growing and is in one area of the brain stem. It is easier to treat than DIPG and has a better prognosis.",Childhood Brain Stem Glioma,0000006_5,CancerGov,https://www.cancer.gov/types/brain/patient/child-glioma-treatment-pdq,C0278600,T191,Disorders What causes Childhood Brain Stem Glioma ?,0000006_5-2,causes,The cause of most childhood brain tumors is unknown.,Childhood Brain Stem Glioma,0000006_5,CancerGov,https://www.cancer.gov/types/brain/patient/child-glioma-treatment-pdq,C0278600,T191,Disorders Who is at risk for Childhood Brain Stem Glioma? ?,0000006_5-3,susceptibility,"Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesnt mean that you will not get cancer. Talk with your child's doctor if you think your child may be at risk. Possible risk factors for brain stem glioma include: - Having certain genetic disorders, such as neurofibromatosis type 1 (NF1).",Childhood Brain Stem Glioma,0000006_5,CancerGov,https://www.cancer.gov/types/brain/patient/child-glioma-treatment-pdq,C0278600,T191,Disorders What are the symptoms of Childhood Brain Stem Glioma ?,0000006_5-4,symptoms,The signs and symptoms of brain stem glioma are not the same in every child. Signs and symptoms depend on the following: - Where the tumor forms in the brain. - The size of the tumor and whether it has spread all through the brain stem. - How fast the tumor grows. - The child's age and development. Some tumors do not cause signs or symptoms. Signs and symptoms may be caused by childhood brain stem gliomas or by other conditions. Check with your child's doctor if your child has any of the following: - Loss of ability to move one side of the face and/or body. - Loss of balance and trouble walking. - Vision and hearing problems. - Morning headache or headache that goes away after vomiting. - Nausea and vomiting. - Unusual sleepiness. - More or less energy than usual. - Changes in behavior. - Trouble learning in school.,Childhood Brain Stem Glioma,0000006_5,CancerGov,https://www.cancer.gov/types/brain/patient/child-glioma-treatment-pdq,C0278600,T191,Disorders How to diagnose Childhood Brain Stem Glioma ?,0000006_5-5,exams and tests,"Tests that examine the brain are used to detect (find) childhood brain stem glioma. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Neurological exam : A series of questions and tests to check the brain, spinal cord, and nerve function. The exam checks a persons mental status, coordination, and ability to walk normally, and how well the muscles, senses, and reflexes work. This may also be called a neuro exam or a neurologic exam. - MRI (magnetic resonance imaging) with gadolinium : A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the brain. A substance called gadolinium is injected into a vein. The gadolinium collects around the cancer cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI). - A biopsy may be done to diagnose certain types of brain stem glioma. If the MRI scan looks like the tumor is a DIPG, a biopsy is usually not done and the tumor is not removed. If the MRI scan looks like a focal brain stem glioma, a biopsy may be done. A part of the skull is removed and a needle is used to remove a sample of the brain tissue. Sometimes, the needle is guided by a computer. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are found, the doctor will remove as much tumor as safely possible during the same surgery. The following test may be done on the tissue that was removed: - Immunohistochemistry : A test that uses antibodies to check for certain antigens in a sample of tissue. The antibody is usually linked to a radioactive substance or a dye that causes the tissue to light up under a microscope. This type of test may be used to tell the difference between brain stem glioma and other brain tumors.",Childhood Brain Stem Glioma,0000006_5,CancerGov,https://www.cancer.gov/types/brain/patient/child-glioma-treatment-pdq,C0278600,T191,Disorders What is the outlook for Childhood Brain Stem Glioma ?,0000006_5-6,outlook,Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis and treatment options depend on: - The type of brain stem glioma. - Where the tumor is found in the brain and if it has spread within the brain stem. - The age of the child when diagnosed. - Whether or not the child has a condition called neurofibromatosis type 1. - Whether the tumor has just been diagnosed or has recurred (come back).,Childhood Brain Stem Glioma,0000006_5,CancerGov,https://www.cancer.gov/types/brain/patient/child-glioma-treatment-pdq,C0278600,T191,Disorders What are the stages of Childhood Brain Stem Glioma ?,0000006_5-7,stages,Key Points - The plan for cancer treatment depends on whether the tumor is in one area of the brain or has spread all through the brain. The plan for cancer treatment depends on whether the tumor is in one area of the brain or has spread all through the brain. Staging is the process used to find out how much cancer there is and if cancer has spread. It is important to know the stage in order to plan treatment. There is no standard staging system for childhood brain stem glioma. Treatment is based on the following: - Whether the tumor is newly diagnosed or recurrent (has come back after treatment). - The type of tumor (either a diffuse intrinsic pontine glioma or a focal glioma).,Childhood Brain Stem Glioma,0000006_5,CancerGov,https://www.cancer.gov/types/brain/patient/child-glioma-treatment-pdq,C0278600,T191,Disorders what research (or clinical trials) is being done for Childhood Brain Stem Glioma ?,0000006_5-8,research,"New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Childhood Brain Stem Glioma,0000006_5,CancerGov,https://www.cancer.gov/types/brain/patient/child-glioma-treatment-pdq,C0278600,T191,Disorders What are the treatments for Childhood Brain Stem Glioma ?,0000006_5-9,treatment,"Key Points - There are different types of treatment for children with brain stem glioma. - Children with brain stem glioma should have their treatment planned by a team of health care providers who are experts in treating childhood brain tumors. - Childhood brain stem gliomas may cause signs or symptoms that begin before the cancer is diagnosed and continue for months or years. - Some cancer treatments cause side effects months or years after treatment has ended. - Six types of standard treatment are used: - Surgery - Radiation therapy - Chemotherapy - Cerebrospinal fluid diversion - Observation - Targeted therapy - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for children with brain stem glioma. Different types of treatment are available for children with brain stem glioma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment. Children with brain stem glioma should have their treatment planned by a team of health care providers who are experts in treating childhood brain tumors. Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other pediatric health care providers who are experts in treating children with brain tumors and who specialize in certain areas of medicine. These may include the following specialists: - Pediatrician. - Neurosurgeon. - Neuropathologist. - Radiation oncologist. - Neuro-oncologist. - Neurologist. - Rehabilitation specialist. - Neuroradiologist. - Endocrinologist. - Psychologist. Childhood brain stem gliomas may cause signs or symptoms that begin before the cancer is diagnosed and continue for months or years. Childhood brain stem gliomas may cause signs or symptoms that continue for months or years. Signs or symptoms caused by the tumor may begin before diagnosis. Signs or symptoms caused by treatment may begin during or right after treatment. Some cancer treatments cause side effects months or years after treatment has ended. These are called late effects. Late effects may include the following: - Physical problems. - Changes in mood, feelings, thinking, learning, or memory. - Second cancers (new types of cancer). Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information). Six types of standard treatment are used: Surgery Surgery may be used to diagnose and treat childhood brain stem glioma as discussed in the General Information section of this summary. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type of the cancer being treated. External radiation therapy is used to treat DIPG. External and/or internal radiation therapy may be used to treat focal brain stem gliomas. Several months after radiation therapy to the brain, imaging tests may show changes to the brain tissue. These changes may be caused by the radiation therapy or may mean the tumor is growing. It is important to be sure the tumor is growing before any more treatment is given. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly in the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type of the cancer being treated. Because radiation therapy to the brain can affect growth and brain development in young children, clinical trials are studying ways of using chemotherapy to delay or reduce the need for radiation therapy. Cerebrospinal fluid diversion Cerebrospinal fluid diversion is a method used to drain fluid that has built up in the brain. A shunt (long, thin tube) is placed in a ventricle (fluid-filled space) of the brain and threaded under the skin to another part of the body, usually the abdomen. The shunt carries extra fluid away from the brain so it may be absorbed elsewhere in the body. Observation Observation is closely monitoring a patients condition without giving any treatment until signs or symptoms appear or change. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Some focal brain stem gliomas that cannot be removed by surgery may be treated with BRAF kinase inhibitor therapy. BRAF kinase inhibitors block the BRAF protein. BRAF proteins help control cell growth and may be mutated (changed) in some types of brain stem glioma. Blocking mutated BRAF kinase proteins may help keep cancer cells from growing. New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. If the results of imaging tests done after treatment show a mass in the brain, a biopsy may be done to find out if it is made up of dead tumor cells or if new cancer cells are growing. In children who are expected to live a long time, regular MRIs may be done to see if the cancer has come back. Treatment Options for Childhood Brain Stem Glioma Newly Diagnosed Childhood Brain Stem Glioma Newly diagnosed childhood brain stem glioma is a tumor for which no treatment has been given. The child may have received drugs or treatment to relieve signs or symptoms caused by the tumor. Standard treatment of diffuse intrinsic pontine glioma (DIPG) may include the following: - Radiation therapy. - Chemotherapy (in infants). Standard treatment of focal glioma may include the following: - Surgery that may be followed by chemotherapy and/or radiation therapy. - Observation for small tumors that grow slowly. Cerebrospinal fluid diversion may be done when there is extra fluid in the brain. - Internal radiation therapy with radioactive seeds, with or without chemotherapy, when the tumor cannot be removed by surgery. - Targeted therapy with a BRAF kinase inhibitor, for certain tumors that cannot be removed by surgery. Treatment of brain stem glioma in children with neurofibromatosis type 1 may be observation. The tumors are slow-growing in these children and may not need specific treatment for years. Check the list of NCI-supported cancer clinical trials that are now accepting patients with untreated childhood brain stem glioma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Progressive or Recurrent Childhood Brain Stem Glioma When cancer does not get better with treatment or comes back, palliative care is an important part of the child's treatment plan. It includes physical, psychological, social, and spiritual support for the child and family. The goal of palliative care is to help control symptoms and give the child the best quality of life possible. Parents may not be sure about whether to continue treatment or what kind of treatment is best for their child. The healthcare team can give parents information to help them make these decisions. There is no standard treatment for progressive or recurrent diffuse intrinsic pontine glioma. The child may be treated in a clinical trial of a new treatment. Treatment of recurrent focal childhood brain stem glioma may include the following: - A second surgery to remove the tumor. - External radiation therapy. - Chemotherapy. - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent childhood brain stem glioma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website.",Childhood Brain Stem Glioma,0000006_5,CancerGov,https://www.cancer.gov/types/brain/patient/child-glioma-treatment-pdq,C0278600,T191,Disorders What is (are) Childhood Central Nervous System Embryonal Tumors ?,0000006_6-1,information,"Key Points - Central nervous system (CNS) embryonal tumors may begin in embryonic (fetal) cells that remain in the brain after birth. - There are different types of CNS embryonal tumors. - Pineoblastomas form in cells of the pineal gland. - Certain genetic conditions increase the risk of childhood CNS embryonal tumors. - Signs and symptoms of childhood CNS embryonal tumors or pineoblastomas depend on the child's age and where the tumor is. - Tests that examine the brain and spinal cord are used to detect (find) childhood CNS embryonal tumors or pineoblastomas. - A biopsy may be done to be sure of the diagnosis of CNS embryonal tumor or pineoblastoma. - Certain factors affect prognosis (chance of recovery) and treatment options. Central nervous system (CNS) embryonal tumors may begin in embryonic (fetal) cells that remain in the brain after birth. Central nervous system (CNS) embryonal tumors form in embryonic cells that remain in the brain after birth. CNS embryonal tumors tend to spread through the cerebrospinal fluid (CSF) to other parts of the brain and spinal cord. The tumors may be malignant (cancer) or benign (not cancer). Most CNS embryonal tumors in children are malignant. Malignant brain tumors are likely to grow quickly and spread into other parts of the brain. When a tumor grows into or presses on an area of the brain, it may stop that part of the brain from working the way it should. Benign brain tumors grow and press on nearby areas of the brain. They rarely spread to other parts of the brain. Both benign and malignant brain tumors can cause signs or symptoms and need treatment. Although cancer is rare in children, brain tumors are the third most common type of childhood cancer, after leukemia and lymphoma. This summary is about the treatment of primary brain tumors (tumors that begin in the brain). The treatment of metastatic brain tumors, which begin in other parts of the body and spread to the brain, is not discussed in this summary. For information about the different types of brain and spinal cord tumors, see the PDQ summary on Childhood Brain and Spinal Cord Tumors Treatment Overview. Brain tumors occur in both children and adults. Treatment for adults may be different from treatment for children. See the PDQ summary on Adult Central Nervous System Tumors Treatment for more information on the treatment of adults. There are different types of CNS embryonal tumors. The different types of CNS embryonal tumors include: - Medulloblastomas Most CNS embryonal tumors are medulloblastomas. Medulloblastomas are fast-growing tumors that form in brain cells in the cerebellum. The cerebellum is at the lower back part of the brain between the cerebrum and the brain stem. The cerebellum controls movement, balance, and posture. Medulloblastomas sometimes spread to the bone, bone marrow, lung, or other parts of the body, but this is rare. - Nonmedulloblastoma embryonal tumors Nonmedulloblastoma embryonal tumors are fast-growing tumors that usually form in brain cells in the cerebrum. The cerebrum is at the top of the head and is the largest part of the brain. The cerebrum controls thinking, learning, problem-solving, emotions, speech, reading, writing, and voluntary movement. Nonmedulloblastoma embryonal tumors may also form in the brain stem or spinal cord. There are four types of nonmedulloblastoma embryonal tumors: - Embryonal tumors with multilayered rosettes Embryonal tumors with multilayered rosettes (ETMR) are rare tumors that form in the brain and spinal cord. ETMR most commonly occur in young children and are fast-growing tumors. - Medulloepitheliomas Medulloepitheliomas are fast-growing tumors that usually form in the brain, spinal cord or nerves just outside the spinal column. They occur most often in infants and young children. - CNS neuroblastomas CNS neuroblastomas are a very rare type of neuroblastoma that form in the nerve tissue of the cerebrum or the layers of tissue that cover the brain and spinal cord. CNS neuroblastomas may be large and spread to other parts of the brain or spinal cord. - CNS ganglioneuroblastomas CNS ganglioneuroblastomas are rare tumors that form in nerve tissue of the brain and spinal cord. They may form in one area and be fast growing or form in more than one area and be slow growing. Childhood CNS atypical teratoid/rhabdoid tumor is also a type of embryonal tumor, but it is treated differently than other childhood CNS embryonal tumors. See the PDQ summary on Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor Treatment for more information. Pineoblastomas form in cells of the pineal gland. The pineal gland is a tiny organ in the center of the brain. The gland makes melatonin, a substance that helps control our sleep cycle. Pineoblastomas form in cells of the pineal gland and are usually malignant. Pineoblastomas are fast-growing tumors with cells that look very different from normal pineal gland cells. Pineoblastomas are not a type of CNS embryonal tumor but treatment for them is a lot like treatment for CNS embryonal tumors. Pineoblastoma is linked with inherited changes in the retinoblastoma (RB1) gene. A child with the inherited form of retinoblastoma (cancer than forms in the tissues of the retina) has an increased risk of pineoblastoma. When retinoblastoma forms at the same time as a tumor in or near the pineal gland, it is called trilateral retinoblastoma. MRI (magnetic resonance imaging) testing in children with retinoblastoma may detect pineoblastoma at an early stage when it can be treated successfully.",Childhood Central Nervous System Embryonal Tumors,0000006_6,CancerGov,https://www.cancer.gov/types/brain/patient/child-cns-embryonal-treatment-pdq,C1333378,T191,Disorders Who is at risk for Childhood Central Nervous System Embryonal Tumors? ?,0000006_6-2,susceptibility,"Certain genetic conditions increase the risk of childhood CNS embryonal tumors. Anything that increases the risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesnt mean that you will not get cancer. Talk with your childs doctor if you think your child may be at risk. Risk factors for CNS embryonal tumors include having the following inherited diseases: - Turcot syndrome. - Rubinstein-Taybi syndrome. - Nevoid basal cell carcinoma (Gorlin) syndrome. - Li-Fraumeni syndrome. - Fanconi anemia. In most cases, the cause of CNS embryonal tumors is not known.",Childhood Central Nervous System Embryonal Tumors,0000006_6,CancerGov,https://www.cancer.gov/types/brain/patient/child-cns-embryonal-treatment-pdq,C1333378,T191,Disorders What are the symptoms of Childhood Central Nervous System Embryonal Tumors ?,0000006_6-3,symptoms,"Signs and symptoms of childhood CNS embryonal tumors or pineoblastomas depend on the child's age and where the tumor is. These and other signs and symptoms may be caused by childhood CNS embryonal tumors, pineoblastomas, or other conditions. Check with your child's doctor if your child has any of the following: - Loss of balance, trouble walking, worsening handwriting, or slow speech. - Lack of coordination. - Headache, especially in the morning, or headache that goes away after vomiting. - Double vision or other eye problems. - Nausea and vomiting. - General weakness or weakness on one side of the face. - Unusual sleepiness or change in energy level. - Seizures. Infants and young children with these tumors may be irritable or grow slowly. Also they may not eat well or meet developmental milestones such as sitting, walking, and talking in sentences.",Childhood Central Nervous System Embryonal Tumors,0000006_6,CancerGov,https://www.cancer.gov/types/brain/patient/child-cns-embryonal-treatment-pdq,C1333378,T191,Disorders How to diagnose Childhood Central Nervous System Embryonal Tumors ?,0000006_6-4,exams and tests,"Tests that examine the brain and spinal cord are used to detect (find) childhood CNS embryonal tumors or pineoblastomas. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Neurological exam : A series of questions and tests to check the brain, spinal cord, and nerve function. The exam checks a patient's mental status, coordination, and ability to walk normally, and how well the muscles, senses, and reflexes work. This may also be called a neuro exam or a neurologic exam. - MRI (magnetic resonance imaging) of the brain and spinal cord with gadolinium : A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the brain and spinal cord. A substance called gadolinium is injected into a vein. The gadolinium collects around the cancer cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI). Sometimes magnetic resonance spectroscopy (MRS) is done during the MRI scan to look at the chemicals in brain tissue. - Lumbar puncture : A procedure used to collect cerebrospinal fluid (CSF) from the spinal column. This is done by placing a needle between two bones in the spine and into the CSF around the spinal cord and removing a sample of the fluid. The sample of CSF is checked under a microscope for signs of tumor cells. The sample may also be checked for the amounts of protein and glucose. A higher than normal amount of protein or lower than normal amount of glucose may be a sign of a tumor. This procedure is also called an LP or spinal tap. A biopsy may be done to be sure of the diagnosis of CNS embryonal tumor or pineoblastoma. If doctors think your child may have a CNS embryonal tumor or pineoblastoma, a biopsy may be done. For brain tumors, the biopsy is done by removing part of the skull and using a needle to remove a sample of tissue. Sometimes, a computer-guided needle is used to remove the tissue sample. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are found, the doctor may remove as much tumor as safely possible during the same surgery. The piece of skull is usually put back in place after the procedure. The following test may be done on the sample of tissue that is removed: - Immunohistochemistry : A test that uses antibodies to check for certain antigens in a sample of tissue. The antibody is usually linked to a radioactive substance or a dye that causes the tissue to light up under a microscope. This type of test may be used to tell the difference between different types of brain tumors.",Childhood Central Nervous System Embryonal Tumors,0000006_6,CancerGov,https://www.cancer.gov/types/brain/patient/child-cns-embryonal-treatment-pdq,C1333378,T191,Disorders What is the outlook for Childhood Central Nervous System Embryonal Tumors ?,0000006_6-5,outlook,"Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on: - The type of tumor and where it is in the brain. - Whether the cancer has spread within the brain and spinal cord when the tumor is found. - The age of the child when the tumor is found. - How much of the tumor remains after surgery. - Whether there are certain changes in the chromosomes, genes, or brain cells. - Whether the tumor has just been diagnosed or has recurred (come back).",Childhood Central Nervous System Embryonal Tumors,0000006_6,CancerGov,https://www.cancer.gov/types/brain/patient/child-cns-embryonal-treatment-pdq,C1333378,T191,Disorders what research (or clinical trials) is being done for Childhood Central Nervous System Embryonal Tumors ?,0000006_6-6,research,"New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Childhood Central Nervous System Embryonal Tumors,0000006_6,CancerGov,https://www.cancer.gov/types/brain/patient/child-cns-embryonal-treatment-pdq,C1333378,T191,Disorders What are the treatments for Childhood Central Nervous System Embryonal Tumors ?,0000006_6-8,treatment,"Key Points - There are different types of treatment for children who have central nervous system (CNS) embryonal tumors. - Children who have CNS embryonal tumors should have their treatment planned by a team of health care providers who are experts in treating brain tumors in children. - Childhood brain tumors may cause signs or symptoms that begin before the cancer is diagnosed and continue for months or years. - Some cancer treatments cause side effects months or years after treatment has ended. - Five types of treatment are used: - Surgery - Radiation therapy - Chemotherapy - High-dose chemotherapy with stem cell rescue - Targeted therapy - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for children who have central nervous system (CNS) embryonal tumors. Different types of treatment are available for children with central nervous system (CNS) embryonal tumors. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment. Children who have CNS embryonal tumors should have their treatment planned by a team of health care providers who are experts in treating brain tumors in children. Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other pediatric health care providers who are experts in treating children with brain tumors and who specialize in certain areas of medicine. These may include the following specialists: - Pediatrician. - Neurosurgeon. - Neurologist. - Neuropathologist. - Neuroradiologist. - Rehabilitation specialist. - Radiation oncologist. - Psychologist. Childhood brain tumors may cause signs or symptoms that begin before the cancer is diagnosed and continue for months or years. Signs or symptoms caused by the tumor may begin before the cancer is diagnosed and continue for months or years. It is important to talk with your child's doctors about signs or symptoms caused by the tumor that may continue after treatment. Some cancer treatments cause side effects months or years after treatment has ended. Side effects from cancer treatment that begin during or after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include the following: - Physical problems. - Changes in mood, feelings, thinking, learning, or memory. - Second cancers (new types of cancer). Children diagnosed with medulloblastoma may have certain problems after surgery or radiation therapy such as changes in the ability to think, learn, and pay attention. Also, cerebellar mutism syndrome may occur after surgery. Signs of this syndrome include the following: - Delayed ability to speak. - Trouble swallowing and eating. - Loss of balance, trouble walking, and worsening handwriting. - Loss of muscle tone. - Mood swings and changes in personality. Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information). Five types of treatment are used: Surgery Surgery is used to diagnose and treat a childhood CNS embryonal tumor as described in the General Information section of this summary. Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy and/or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. Certain ways of giving radiation therapy can help keep radiation from damaging nearby healthy tissue. These types of radiation therapy include the following: - Conformal radiation therapy: Conformal radiation therapy is a type of external radiation therapy that uses a computer to make a 3-dimensional (3-D) picture of the tumor and shapes the radiation beams to fit the tumor. This allows a high dose of radiation to reach the tumor and causes less damage to nearby healthy tissue. - Stereotactic radiation therapy: Stereotactic radiation therapy is a type of external radiation therapy. A rigid head frame is attached to the skull to keep the head still during the radiation treatment. A machine aims radiation directly at the tumor, causing less damage to nearby healthy tissue. The total dose of radiation is divided into several smaller doses given over several days. This procedure is also called stereotactic external-beam radiation therapy and stereotaxic radiation therapy. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. Radiation therapy to the brain can affect growth and development in young children. For this reason, clinical trials are studying new ways of giving radiation that may have fewer side effects than standard methods. The way the radiation therapy is given depends on the type of cancer being treated. External radiation therapy is used to treat childhood CNS embryonal tumors. Because radiation therapy can affect growth and brain development in young children, especially children who are three years old or younger, chemotherapy may be given to delay or reduce the need for radiation therapy. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is treatment using more than one anticancer drug. The way the chemotherapy is given depends on the type of cancer being treated. Regular dose anticancer drugs given by mouth or vein to treat central nervous system tumors cannot cross the blood-brain barrier and enter the fluid that surrounds the brain and spinal cord. Instead, an anticancer drug is injected into the fluid-filled space to kill cancer cells that may have spread there. This is called intrathecal or intraventricular chemotherapy. High-dose chemotherapy with stem cell rescue High-dose chemotherapy with stem cell rescue is a way of giving high doses of chemotherapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the bodys blood cells. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to attack cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. Signal transduction inhibitors are a type of targeted therapy used to treat recurrent medulloblastoma. Signal transduction inhibitors block signals that are passed from one molecule to another inside a cell. Blocking these signals may kill cancer cells. Vismodegib is a type of signal transduction inhibitor. New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. (See the General Information section for a list of tests.) Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. This is sometimes called re-staging. Some of the imaging tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the brain tumor has recurred (come back). If the imaging tests show abnormal tissue in the brain, a biopsy may also be done to find out if the tissue is made up of dead tumor cells or if new cancer cells are growing. These tests are sometimes called follow-up tests or check-ups. Treatment Options for Childhood Central Nervous System Embryonal Tumors and Childhood Pineoblastoma Newly Diagnosed Childhood Medulloblastoma In newly diagnosed childhood medulloblastoma, the tumor itself has not been treated. The child may have received drugs or treatment to relieve signs or symptoms caused by the tumor. Children older than 3 years with average-risk medulloblastoma Standard treatment of average-risk medulloblastoma in children older than 3 years includes the following: - Surgery to remove as much of the tumor as possible. This is followed by radiation therapy to the brain and spinal cord. Chemotherapy is also given during and after radiation therapy. - Surgery to remove the tumor, radiation therapy, and high-dose chemotherapy with stem cell rescue. Children older than 3 years with high-risk medulloblastoma Standard treatment of high-risk medulloblastoma in children older than 3 years includes the following: - Surgery to remove as much of the tumor as possible. This is followed by a larger dose of radiation therapy to the brain and spinal cord than the dose given for average-risk medulloblastoma. Chemotherapy is also given during and after radiation therapy. - Surgery to remove the tumor, radiation therapy, and high-dose chemotherapy with stem cell rescue. - A clinical trial of new combinations of radiation therapy and chemotherapy. Children aged 3 years and younger Standard treatment of medulloblastoma in children aged 3 years and younger is: - Surgery to remove as much of the tumor as possible, followed by chemotherapy. Other treatments that may be given after surgery include the following: - Chemotherapy with or without radiation therapy to the area where the tumor was removed. - High-dose chemotherapy with stem cell rescue. Check the list of NCI-supported cancer clinical trials that are now accepting patients with untreated childhood medulloblastoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Newly Diagnosed Childhood Nonmedulloblastoma Embryonal Tumors In newly diagnosed childhood nonmedulloblastoma embryonal tumors, the tumor itself has not been treated. The child may have received drugs or treatment to relieve symptoms caused by the tumor. Children older than 3 years Standard treatment of nonmedulloblastoma embryonal tumors in children older than 3 years is: - Surgery to remove as much of the tumor as possible. This is followed by radiation therapy to the brain and spinal cord. Chemotherapy is also given during and after radiation therapy. Children aged 3 years and younger Standard treatment of nonmedulloblastoma embryonal tumors in children aged 3 years and younger is: - Surgery to remove as much of the tumor as possible, followed by chemotherapy. Other treatments that may be given after surgery include the following: - Chemotherapy and radiation therapy to the area where the tumor was removed. - High-dose chemotherapy with stem cell rescue. Check the list of NCI-supported cancer clinical trials that are now accepting patients with untreated childhood nonmedulloblastoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Newly Diagnosed Childhood Medulloepithelioma In newly diagnosed childhood medulloepithelioma, the tumor itself has not been treated. The child may have received drugs or treatment to relieve symptoms caused by the tumor. Children older than 3 years Standard treatment of medulloepithelioma in children older than 3 years includes the following: - Surgery to remove as much of the tumor as possible. This is followed by radiation therapy to the brain and spinal cord. Chemotherapy is also given during and after radiation therapy. - Surgery to remove the tumor, radiation therapy, and high-dose chemotherapy with stem cell rescue. - A clinical trial of new combinations of radiation therapy and chemotherapy. Children aged 3 years and younger Standard treatment of medulloepithelioma in children aged 3 years and younger includes the following: - Surgery to remove as much of the tumor as possible, followed by chemotherapy. - High-dose chemotherapy with stem cell rescue. - Radiation therapy, when the child is older. - A clinical trial of new combinations and schedules of chemotherapy or new combinations of chemotherapy with stem cell rescue. Treatment of medulloepithelioma in children aged 3 years and younger is often within a clinical trial. Check the list of NCI-supported cancer clinical trials that are now accepting patients with childhood medulloepithelioma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Newly Diagnosed Childhood Pineoblastoma In newly diagnosed childhood pineoblastoma, the tumor itself has not been treated. The child may have received drugs or treatment to relieve symptoms caused by the tumor. Children older than 3 years Standard treatment of pineoblastoma in children older than 3 years includes the following: - Surgery to remove the tumor. The tumor usually cannot be completely removed because of where it is in the brain. Surgery is often followed by radiation therapy to the brain and spinal cord and chemotherapy. - A clinical trial of high-dose chemotherapy after radiation therapy and stem cell rescue. - A clinical trial of chemotherapy during radiation therapy. Children aged 3 years and younger Standard treatment of pineoblastoma in children aged 3 years and younger includes the following: - Biopsy to diagnose pineoblastoma followed by chemotherapy. - If the tumor responds to chemotherapy, radiation therapy is given when the child is older. - High-dose chemotherapy with stem cell rescue. Check the list of NCI-supported cancer clinical trials that are now accepting patients with untreated childhood pineoblastoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Recurrent Childhood Central Nervous System Embryonal Tumors and Pineoblastomas The treatment of central nervous system (CNS) embryonal tumors and pineoblastoma that recur (come back) depends on: - The type of tumor. - Whether the tumor recurred where it first formed or has spread to other parts of the brain, spinal cord, or body. - The type of treatment given in the past. - How much time has passed since the initial treatment ended. - Whether the patient has signs or symptoms. Treatment for recurrent childhood CNS embryonal tumors and pineoblastomas may include the following: - For children who previously received radiation therapy and chemotherapy, treatment may include repeat radiation at the site where the cancer started and where the tumor has spread. Stereotactic radiation therapy and/or chemotherapy may also be used. - For infants and young children who previously received chemotherapy only and have a local recurrence, treatment may be chemotherapy with radiation therapy to the tumor and the area close to it. Surgery to remove the tumor may also be done. - For patients who previously received radiation therapy, high-dose chemotherapy and stem cell rescue may be used. It is not known whether this treatment improves survival. - Targeted therapy with a signal transduction inhibitor for patients whose cancer has certain changes in the genes. Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent childhood central nervous system embryonal tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website.",Childhood Central Nervous System Embryonal Tumors,0000006_6,CancerGov,https://www.cancer.gov/types/brain/patient/child-cns-embryonal-treatment-pdq,C1333378,T191,Disorders What is (are) Childhood Central Nervous System Germ Cell Tumors ?,0000006_7-1,information,"Key Points - Childhood central nervous system (CNS) germ cell tumors form from germ cells. - There are different types of childhood CNS germ cell tumors. - Germinomas - Nongerminomas - The cause of most childhood CNS germ cell tumors is not known. - Signs and symptoms of childhood CNS germ cell tumors include unusual thirst, frequent urination, early puberty, or vision changes. - Imaging studies and tests are used to detect (find) and diagnose childhood CNS germ cell tumors. - A biopsy may be done to be sure of the diagnosis of CNS germ cell tumor. - Certain factors affect prognosis (chance of recovery). Childhood central nervous system (CNS) germ cell tumors form from germ cells. Germ cells are a type of cell that form as a fetus (unborn baby) develops. These cells later become sperm in the testicles or eggs in the ovaries. Sometimes while the fetus is forming, germ cells travel to other parts of the body and grow into germ cell tumors. Germ cells tumors that form in the brain or spinal cord are called CNS germ cell tumors. The most common places for one or more central nervous system (CNS) germ cell tumors to form is near the pineal gland and in an area of the brain that includes the pituitary gland and the tissue just above it. Sometimes germ cell tumors may form in other areas of the brain. This summary is about germ cell tumors that start in the central nervous system (brain and spinal cord). Germ cell tumors may also form in other parts of the body. See the PDQ summary on Childhood Extracranial Germ Cell Tumors Treatment for information on germ cell tumors that are extracranial (outside the brain). CNS germ cell tumors usually occur in children, but may occur in adults. Treatment for children may be different than treatment for adults. See the following PDQ summaries for information about treatment for adults: - Adult Central Nervous System Tumors Treatment - Extragonadal Germ Cell Tumors Treatment For information about other types of childhood brain and spinal cord tumors, see the PDQ summary on Childhood Brain and Spinal Cord Tumors Treatment Overview. There are different types of childhood CNS germ cell tumors. There are different types of CNS germ cell tumors. The type of CNS germ cell tumor depends on what the cells look like under a microscope. This summary is about the treatment of the following types of CNS germ cell tumors: Germinomas Germinomas are the most common type of CNS germ cell tumor and have a good prognosis. Nongerminomas Some nongerminomas make hormones. CNS teratomas are a type of nongerminoma that does not make hormones. They may have different kinds of tissue in them, such as hair, muscle, and bone. Teratomas are described as mature or immature, based on how normal the cells look under a microscope. Sometimes teratomas are a mix of mature and immature cells. Other types of nongerminomas include the following: - Choriocarcinomas make the hormone beta-human chorionic gonadotropin (-hCG). - Embryonal carcinomas do not make hormones. - Yolk sac tumors make the hormone alpha-fetoprotein (AFP). - Mixed germ cell tumors are made of more than one kind of germ cell.",Childhood Central Nervous System Germ Cell Tumors,0000006_7,CancerGov,https://www.cancer.gov/types/brain/patient/child-cns-germ-cell-treatment-pdq,C1332880,T191,Disorders What are the stages of Childhood Central Nervous System Germ Cell Tumors ?,0000006_7-2,stages,Key Points - Childhood central nervous system (CNS) germ cell tumors rarely spread outside of the brain and spinal cord. Childhood central nervous system (CNS) germ cell tumors rarely spread outside of the brain and spinal cord. Staging is the process used to find out how much cancer there is and if cancer has spread. There is no standard staging system for childhood central nervous system (CNS) germ cell tumors. The treatment plan depends on the following: - The type of germ cell tumor. - Whether the tumor has spread within the CNS or to other parts of the body. - The results of tests and procedures done to diagnose childhood CNS germ cell tumors. - Whether the tumor is newly diagnosed or has recurred (come back) after treatment.,Childhood Central Nervous System Germ Cell Tumors,0000006_7,CancerGov,https://www.cancer.gov/types/brain/patient/child-cns-germ-cell-treatment-pdq,C1332880,T191,Disorders What causes Childhood Central Nervous System Germ Cell Tumors ?,0000006_7-3,causes,The cause of most childhood CNS germ cell tumors is not known.,Childhood Central Nervous System Germ Cell Tumors,0000006_7,CancerGov,https://www.cancer.gov/types/brain/patient/child-cns-germ-cell-treatment-pdq,C1332880,T191,Disorders What are the symptoms of Childhood Central Nervous System Germ Cell Tumors ?,0000006_7-4,symptoms,"Signs and symptoms of childhood CNS germ cell tumors include unusual thirst, frequent urination, early puberty, or vision changes. Signs and symptoms depend on the following: - Where the tumor has formed. - The size of the tumor. - Whether the tumor makes hormones. Signs and symptoms may be caused by childhood CNS germ cell tumors or by other conditions. Check with your childs doctor if your child has any of the following: - Being very thirsty. - Making large amounts of urine that is clear or almost clear. - Frequent urination. - Bed wetting or getting up at night to urinate. - Trouble moving the eyes or trouble seeing clearly. - Loss of appetite. - Weight loss for no known reason. - Early or late puberty. - Short stature (being shorter than normal). - Headaches. - Nausea and vomiting. - Feeling very tired. - Having problems with school work.",Childhood Central Nervous System Germ Cell Tumors,0000006_7,CancerGov,https://www.cancer.gov/types/brain/patient/child-cns-germ-cell-treatment-pdq,C1332880,T191,Disorders How to diagnose Childhood Central Nervous System Germ Cell Tumors ?,0000006_7-5,exams and tests,"Imaging studies and tests are used to detect (find) and diagnose childhood CNS germ cell tumors. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient's health habits and past illnesses and treatments will also be taken. - Neurological exam : A series of questions and tests to check the brain, spinal cord, and nerve function. The exam checks a persons mental status, coordination, and ability to walk normally, and how well the muscles, senses, and reflexes work. This may also be called a neuro exam or a neurologic exam. - Visual field exam: An exam to check a persons field of vision (the total area in which objects can be seen). This test measures both central vision (how much a person can see when looking straight ahead) and peripheral vision (how much a person can see in all other directions while staring straight ahead). The eyes are tested one at a time. The eye not being tested is covered. - MRI (magnetic resonance imaging) with gadolinium : A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the brain and spinal cord. A substance called gadolinium is injected into a vein. The gadolinium collects around the cancer cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Lumbar puncture : A procedure used to collect cerebrospinal fluid (CSF) from the spinal column. This is done by placing a needle between two bones in the spine and into the CSF around the spinal cord and removing a sample of the fluid. The sample of CSF is checked under a microscope for signs of tumor cells. The sample may also be checked for the amounts of protein and glucose. A higher than normal amount of protein or lower than normal amount of glucose may be a sign of a tumor. This procedure is also called an LP or spinal tap. - Tumor marker tests : A procedure in which a sample of blood or cerebrospinal fluid (CSF) is checked to measure the amounts of certain substances released into the blood and CSF by organs, tissues, or tumor cells in the body. Certain substances are linked to specific types of cancer when found in increased levels in the blood. These are called tumor markers. The following tumor markers are used to diagnose some CNS germ cell tumors: - Alpha-fetoprotein (AFP). - Beta-human chorionic gonadotropin (-hCG). - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher- or lower-than-normal) amount of a substance can be a sign of disease. - Blood hormone studies: A procedure in which a blood sample is checked to measure the amounts of certain hormones released into the blood by organs and tissues in the body. An unusual (higher- or lower-than-normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. The blood will be checked for the levels of hormones made by the pituitary gland and other glands. A biopsy may be done to be sure of the diagnosis of CNS germ cell tumor. If doctors think your child may have a CNS germ cell tumor, a biopsy may be done. For brain tumors, the biopsy is done by removing part of the skull and using a needle to remove a sample of tissue. Sometimes, a needle guided by a computer is used to remove the tissue sample. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are found, the doctor may remove as much tumor as safely possible during the same surgery. The piece of skull is usually put back in place after the procedure. Sometimes the diagnosis can be made based on the results of imaging and tumor marker tests and a biopsy is not needed. The following test may be done on the sample of tissue that is removed: - Immunohistochemistry : A test that uses antibodies to check for certain antigens in a sample of tissue. The antibody is usually linked to a radioactive substance or a dye that causes the tissue to light up under a microscope. This type of test may be used to tell the difference between different types of brain tumors.",Childhood Central Nervous System Germ Cell Tumors,0000006_7,CancerGov,https://www.cancer.gov/types/brain/patient/child-cns-germ-cell-treatment-pdq,C1332880,T191,Disorders What is the outlook for Childhood Central Nervous System Germ Cell Tumors ?,0000006_7-6,outlook,Certain factors affect prognosis (chance of recovery). The prognosis (chance of recovery) depends on the following: - The type of germ cell tumor. - The type and level of any tumor markers. - Where the tumor is in the brain or in the spinal cord. - Whether the cancer has spread within the brain and spinal cord or to other parts of the body. - Whether the tumor is newly diagnosed or has recurred (come back) after treatment.,Childhood Central Nervous System Germ Cell Tumors,0000006_7,CancerGov,https://www.cancer.gov/types/brain/patient/child-cns-germ-cell-treatment-pdq,C1332880,T191,Disorders what research (or clinical trials) is being done for Childhood Central Nervous System Germ Cell Tumors ?,0000006_7-7,research,"Check the list of NCI-supported cancer clinical trials that are now accepting patients with childhood central nervous system germ cell tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Current Clinical Trials section that follows for links to current treatment clinical trials. These have been retrieved from the NCI's listing of clinical trials.",Childhood Central Nervous System Germ Cell Tumors,0000006_7,CancerGov,https://www.cancer.gov/types/brain/patient/child-cns-germ-cell-treatment-pdq,C1332880,T191,Disorders What are the treatments for Childhood Central Nervous System Germ Cell Tumors ?,0000006_7-8,treatment,"Key Points - There are different types of treatment for patients with childhood central nervous system (CNS) germ cell tumors. - Children with childhood CNS germ cell tumors should have their treatment planned by a team of health care providers who are experts in treating cancer in children. - Childhood CNS germ cell tumors may cause signs or symptoms that begin before the cancer is diagnosed and continue for months or years. - Some cancer treatments cause side effects months or years after treatment has ended. - Four types of treatment are used: - Radiation therapy - Chemotherapy - Surgery - High-dose chemotherapy with stem cell rescue - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with childhood central nervous system (CNS) germ cell tumors. Different types of treatment are available for children with childhood central nervous system (CNS) germ cell tumors. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment. Children with childhood CNS germ cell tumors should have their treatment planned by a team of health care providers who are experts in treating cancer in children. Treatment will be overseen by a pediatric oncologist and/or a radiation oncologist,. A pediatric oncologist is a doctor who specializes in treating children with cancer. A radiation oncologist specializes in treating cancer with radiation therapy. These doctors work with other pediatric health care providers who are experts in treating children with childhood CNS germ cell tumors and who specialize in certain areas of medicine. These may include the following specialists: - Pediatrician. - Pediatric neurosurgeon. - Neurologist. - Endocrinologist. - Ophthalmologist. - Pediatric nurse specialist. - Rehabilitation specialist. - Psychologist. - Social worker. Childhood CNS germ cell tumors may cause signs or symptoms that begin before the cancer is diagnosed and continue for months or years. Childhood CNS germ cell tumors may cause signs or symptoms that continue for months or years. Signs or symptoms caused by the tumor may begin before the cancer is diagnosed. Signs or symptoms caused by treatment may begin during or right after treatment. Some cancer treatments cause side effects months or years after treatment has ended. Some cancer treatments cause side effects that continue or appear months or years after cancer treatment has ended. These are called late effects. Late effects of cancer treatment may include: - Physical problems. - Changes in mood, feelings, thinking, learning, or memory. - Second cancers (new types of cancer). Some late effects may be treated or controlled. It is important to talk with your child's doctors about the possible late effects caused by some treatments. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information). Four types of treatment are used: Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. Certain ways of giving radiation therapy can help keep radiation from damaging nearby healthy tissue. This type of radiation therapy may include the following: - Stereotactic radiosurgery: Stereotactic radiosurgery is a type of external radiation therapy. A rigid head frame is attached to the skull to keep the head still during the radiation treatment. A machine aims a single large dose of radiation directly at the tumor. This procedure does not involve surgery. It is also called stereotaxic radiosurgery, radiosurgery, and radiation surgery. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type of cancer being treated. External radiation therapy is used to treat childhood CNS germ cell tumors. Radiation therapy to the brain can affect growth and development in young children. Certain ways of giving radiation therapy can lessen the damage to healthy brain tissue. For children younger than 3 years, chemotherapy may be given instead. This can delay or reduce the need for radiation therapy. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type of cancer being treated. Surgery Whether surgery to remove the tumor can be done depends on where the tumor is in the brain. Surgery to remove the tumor may cause severe, long-term side effects. Surgery may be done to remove teratomas and may be used for germ cell tumors that come back. Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. High-dose chemotherapy with stem cell rescue High-dose chemotherapy with stem cell rescue is a way of giving high doses of chemotherapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the bodys blood cells. New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Current Clinical Trials section that follows for links to current treatment clinical trials. These have been retrieved from the NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Children whose cancer affected their pituitary gland when the cancer was diagnosed will usually need to have their blood hormone levels checked. If the blood hormone level is low, replacement hormone medicine is given. Children who had a high tumor marker level (alpha-fetoprotein or beta-human chorionic gonadotropin) when the cancer was diagnosed usually need to have their blood tumor marker level checked. If the tumor marker level increases after initial treatment, the tumor may have recurred. Treatment Options for Childhood CNS Germ Cell Tumors Newly Diagnosed CNS Germinomas Treatment of newly diagnosed central nervous system (CNS) germinomas may include the following: - Radiation therapy to the tumor and ventricles (fluid -filled spaces) of the brain. A higher dose of radiation is given to the tumor than the area around the tumor. - Chemotherapy followed by radiation therapy, for younger children. - A clinical trial of chemotherapy followed by radiation therapy given in lower doses depending on how the tumor responds to treatment. Newly Diagnosed CNS Teratomas Treatment of newly diagnosed mature and immature central nervous system (CNS) teratomas may include the following: - Surgery to remove as much of the tumor as possible. Radiation therapy and/or chemotherapy may be given if any tumor remains after surgery. Newly Diagnosed CNS Nongerminomas It is not clear what treatment is best for newly diagnosed central nervous system (CNS) nongerminomas is. Treatment of choriocarcinoma, embryonal carcinoma, yolk sac tumor, or mixed germ cell tumor may include the following: - Chemotherapy followed by radiation therapy. If a mass remains after chemotherapy, surgery may be needed to check if the mass is a mature teratoma, fibrosis, or a growing tumor. - If the mass is a mature teratoma or fibrosis, radiation therapy is given. - If the mass is a growing tumor, other treatments may be given. - Surgery to remove as much of the mass as possible and check for tumor cells, if tumor marker levels are normal and the mass continues to grow (called growing teratoma syndrome). - A clinical trial of chemotherapy followed by radiation therapy given in lower doses depending on how the tumor responds to treatment. Recurrent Childhood CNS Germ Cell Tumors Treatment of recurrent childhood central nervous system (CNS) germ cell tumors may include the following: - Chemotherapy followed by radiation therapy. - High-dose chemotherapy with stem cell rescue using the patient's stem cells. - A clinical trial of a new treatment.",Childhood Central Nervous System Germ Cell Tumors,0000006_7,CancerGov,https://www.cancer.gov/types/brain/patient/child-cns-germ-cell-treatment-pdq,C1332880,T191,Disorders What is (are) Childhood Craniopharyngioma ?,0000006_8-1,information,"Key Points - Childhood craniopharyngiomas are benign brain tumors found near the pituitary gland. - There are no known risk factors for childhood craniopharyngioma. - Signs of childhood craniopharyngioma include vision changes and slow growth. - Tests that examine the brain, vision, and hormone levels are used to detect (find) childhood craniopharyngiomas. - Childhood craniopharyngiomas are diagnosed and may be removed in the same surgery. - Certain factors affect prognosis (chance of recovery) and treatment options. Childhood craniopharyngiomas are benign brain tumors found near the pituitary gland. Childhood craniopharyngiomas are rare tumors usually found near the pituitary gland (a pea-sized organ at the bottom of the brain that controls other glands) and the hypothalamus (a small cone-shaped organ connected to the pituitary gland by nerves). Craniopharyngiomas are usually part solid mass and part fluid -filled cyst. They are benign (not cancer) and do not spread to other parts of the brain or to other parts of the body. However, they may grow and press on nearby parts of the brain or other areas, including the pituitary gland, the optic chiasm, optic nerves, and fluid-filled spaces in the brain. Craniopharyngiomas may affect many functions of the brain. They may affect hormone making, growth, and vision. Benign brain tumors need treatment. This summary is about the treatment of primary brain tumors (tumors that begin in the brain). Treatment for metastatic brain tumors, which are tumors formed by cancer cells that begin in other parts of the body and spread to the brain, is not covered in this summary. See the PDQ treatment summary on Childhood Brain and Spinal Cord Tumors Treatment Overview for information about the different types of childhood brain and spinal cord tumors. Brain tumors can occur in both children and adults; however, treatment for children may be different than treatment for adults. (See the PDQ summary on Adult Central Nervous System Tumors Treatment for more information.)",Childhood Craniopharyngioma,0000006_8,CancerGov,https://www.cancer.gov/types/brain/patient/child-cranio-treatment-pdq,C0278652,T191,Disorders Who is at risk for Childhood Craniopharyngioma? ?,0000006_8-2,susceptibility,There are no known risk factors for childhood craniopharyngioma. Craniopharyngiomas are rare in children younger than 2 years of age and are most often diagnosed in children aged 5 to 14 years. It is not known what causes these tumors.,Childhood Craniopharyngioma,0000006_8,CancerGov,https://www.cancer.gov/types/brain/patient/child-cranio-treatment-pdq,C0278652,T191,Disorders What are the symptoms of Childhood Craniopharyngioma ?,0000006_8-3,symptoms,"Signs of childhood craniopharyngioma include vision changes and slow growth. These and other signs and symptoms may be caused by craniopharyngiomas or by other conditions. Check with your childs doctor if your child has any of the following: - Headaches, including morning headache or headache that goes away after vomiting. - Vision changes. - Nausea and vomiting. - Loss of balance or trouble walking. - Increase in thirst or urination. - Unusual sleepiness or change in energy level. - Changes in personality or behavior. - Short stature or slow growth. - Hearing loss. - Weight gain.",Childhood Craniopharyngioma,0000006_8,CancerGov,https://www.cancer.gov/types/brain/patient/child-cranio-treatment-pdq,C0278652,T191,Disorders How to diagnose Childhood Craniopharyngioma ?,0000006_8-4,exams and tests,"Tests that examine the brain, vision, and hormone levels are used to detect (find) childhood craniopharyngiomas. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Neurological exam : A series of questions and tests to check the brain, spinal cord, and nerve function. The exam checks a persons mental status, coordination, and ability to walk normally, and how well the muscles, senses, and reflexes work. This may also be called a neuro exam or a neurologic exam. - Visual field exam: An exam to check a persons field of vision (the total area in which objects can be seen). This test measures both central vision (how much a person can see when looking straight ahead) and peripheral vision (how much a person can see in all other directions while staring straight ahead). Any loss of vision may be a sign of a tumor that has damaged or pressed on the parts of the brain that affect eyesight. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - MRI (magnetic resonance imaging) of the brain and spinal cord with gadolinium : A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the brain. A substance called gadolinium is injected into a vein. The gadolinium collects around the tumor cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. - Blood hormone studies: A procedure in which a blood sample is checked to measure the amounts of certain hormones released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. For example, the blood may be checked for unusual levels of thyroid-stimulating hormone (TSH) or adrenocorticotropic hormone (ACTH). TSH and ACTH are made by the pituitary gland in the brain. Childhood craniopharyngiomas are diagnosed and may be removed in the same surgery. Doctors may think a mass is a craniopharyngioma based on where it is in the brain and how it looks on a CT scan or MRI. In order to be sure, a sample of tissue is needed. One of the following types of biopsy procedures may be used to take the sample of tissue: - Open biopsy: A hollow needle is inserted through a hole in the skull into the brain. - Computer-guided needle biopsy: A hollow needle guided by a computer is inserted through a small hole in the skull into the brain. - Transsphenoidal biopsy: Instruments are inserted through the nose and sphenoid bone (a butterfly-shaped bone at the base of the skull) and into the brain. A pathologist views the tissue under a microscope to look for tumor cells. If tumor cells are found, as much tumor as safely possible may be removed during the same surgery. The following laboratory test may be done on the sample of tissue that is removed: - Immunohistochemistry : A test that uses antibodies to check for certain antigens in a sample of tissue. The antibody is usually linked to a radioactive substance or a dye that causes the tissue to light up under a microscope. This type of test may be used to tell the difference between different types of cancer.",Childhood Craniopharyngioma,0000006_8,CancerGov,https://www.cancer.gov/types/brain/patient/child-cranio-treatment-pdq,C0278652,T191,Disorders What is the outlook for Childhood Craniopharyngioma ?,0000006_8-5,outlook,Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - The size of the tumor. - Where the tumor is in the brain. - Whether there are tumor cells left after surgery. - The child's age. - Side effects that may occur months or years after treatment. - Whether the tumor has just been diagnosed or has recurred (come back).,Childhood Craniopharyngioma,0000006_8,CancerGov,https://www.cancer.gov/types/brain/patient/child-cranio-treatment-pdq,C0278652,T191,Disorders What are the stages of Childhood Craniopharyngioma ?,0000006_8-6,stages,The process used to find out if cancer has spread within the brain or to other parts of the body is called staging. There is no standard system for staging childhood craniopharyngioma. Craniopharyngioma is described as newly diagnosed disease or recurrent disease. The results of the tests and procedures done to diagnose craniopharyngioma are used to help make decisions about treatment.,Childhood Craniopharyngioma,0000006_8,CancerGov,https://www.cancer.gov/types/brain/patient/child-cranio-treatment-pdq,C0278652,T191,Disorders what research (or clinical trials) is being done for Childhood Craniopharyngioma ?,0000006_8-7,research,"Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the medical research process. Clinical trials are done to find out if new treatments are safe and effective or better than the standard treatment. Many of today's standard treatments are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way diseases will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Check the list of NCI-supported cancer clinical trials that are now accepting patients with childhood craniopharyngioma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Patients can enter clinical trials before, during, or after starting their treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients who have not improved. There are also clinical trials that test new ways to stop a disease from recurring (coming back) or reduce the side effects of treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's clinical trials database.",Childhood Craniopharyngioma,0000006_8,CancerGov,https://www.cancer.gov/types/brain/patient/child-cranio-treatment-pdq,C0278652,T191,Disorders What are the treatments for Childhood Craniopharyngioma ?,0000006_8-8,treatment,"Key Points - There are different types of treatment for children with craniopharyngioma. - Children with craniopharyngioma should have their treatment planned by a team of health care providers who are experts in treating brain tumors in children. - Childhood brain tumors may cause signs or symptoms that begin before the cancer is diagnosed and continue for months or years. - Some treatments for tumors cause side effects months or years after treatment has ended. - Five types of treatment are used: - Surgery (resection) - Surgery and radiation therapy - Surgery with cyst drainage - Chemotherapy - Biologic therapy - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their treatment. - Follow-up tests may be needed. There are different types of treatment for children with craniopharyngioma. Different types of treatments are available for children with craniopharyngioma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with tumors. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Because tumors in children are rare, taking part in a clinical trial should be considered. Clinical trials are taking place in many parts of the country. Information about ongoing clinical trials is available from the NCI website. Choosing the most appropriate treatment is a decision that ideally involves the patient, family, and health care team. Children with craniopharyngioma should have their treatment planned by a team of health care providers who are experts in treating brain tumors in children. Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with tumors. The pediatric oncologist works with other pediatric healthcare providers who are experts in treating children with brain tumors and who specialize in certain areas of medicine. These may include the following specialists: - Pediatrician. - Neurosurgeon. - Radiation oncologist. - Neurologist. - Endocrinologist. - Ophthalmologist. - Rehabilitation specialist. - Psychologist. - Social worker. - Nurse specialist. Childhood brain tumors may cause signs or symptoms that begin before the cancer is diagnosed and continue for months or years. Signs or symptoms caused by the tumor may begin before diagnosis and continue for months or years. It is important to talk with your child's doctors about signs or symptoms caused by the tumor that may continue after treatment. Some treatments for tumors cause side effects months or years after treatment has ended. Side effects from tumor treatment that begin during or after treatment and continue for months or years are called late effects. Late effects of tumor treatment may include the following: - Physical problems such as seizures. - Behavior problems. - Changes in mood, feelings, thinking, learning, or memory. - Second cancers (new types of cancer). The following serious physical problems may occur if the pituitary gland, hypothalamus, optic nerves, or carotid artery are affected during surgery or radiation therapy: - Obesity. - Metabolic syndrome, including fatty liver disease not caused by drinking alcohol. - Vision problems, including blindness. - Blood vessel problems or stroke. - Loss of the ability to make certain hormones. Some late effects may be treated or controlled. Life-long hormone replacement therapy with several medicines may be needed. It is important to talk with your child's doctors about the effects tumor treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information). Five types of treatment are used: Surgery (resection) The way the surgery is done depends on the size of the tumor and where it is in the brain. It also depends on whether the tumor has grown into nearby tissue in a finger-like way and expected late effects after surgery. The types of surgery that may be used to remove all of the tumor that can be seen with the eye include the following: - Transsphenoidal surgery: A type of surgery in which the instruments are inserted into part of the brain by going through an incision (cut) made under the upper lip or at the bottom of the nose between the nostrils and then through the sphenoid bone (a butterfly-shaped bone at the base of the skull). - Craniotomy: Surgery to remove the tumor through an opening made in the skull. Sometimes all of the tumor that can be seen is removed in surgery and no further treatment is needed. At other times, it is hard to remove the tumor because it is growing into or pressing on nearby organs. If there is tumor remaining after the surgery, radiation therapy is usually given to kill any tumor cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Surgery and radiation therapy Partial resection is used to treat some craniopharyngiomas. It is used to diagnose the tumor, remove fluid from a cyst, and relieve pressure on the optic nerves. If the tumor is near the pituitary gland or hypothalamus, it is not removed. This reduces the number of serious side effects after surgery. Partial resection is followed by radiation therapy. Radiation therapy is a tumor treatment that uses high-energy x-rays or other types of radiation to kill tumor cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the tumor. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the tumor. The way the radiation therapy is given depends on the type of tumor, whether the tumor is newly diagnosed or has come back, and where the tumor formed in the brain. External and internal radiation therapy are used to treat childhood craniopharyngioma. Because radiation therapy to the brain can affect growth and development in young children, ways of giving radiation therapy that have fewer side effects are being used. These include: - Stereotactic radiosurgery: For very small craniopharyngiomas at the base of the brain, stereotactic radiosurgery may be used. Stereotactic radiosurgery is a type of external radiation therapy. A rigid head frame is attached to the skull to keep the head still during the radiation treatment. A machine aims a single large dose of radiation directly at the tumor. This procedure does not involve surgery. It is also called stereotaxic radiosurgery, radiosurgery, and radiation surgery. - Intracavitary radiation therapy: Intracavitary radiation therapy is a type of internal radiation therapy that may be used in tumors that are part solid mass and part fluid-filled cyst. Radioactive material is placed inside the tumor. This type of radiation therapy causes less damage to the nearby hypothalamus and optic nerves. - Intensity-modulated proton therapy: A type of radiation therapy that uses streams of protons (tiny particles with a positive charge) to kill tumor cells. A computer is used to target the exact shape and location of the tumor with proton therapy. This type of 3-dimensional radiation therapy may cause less damage to healthy tissue in the brain and other parts of the body. Proton radiation is different from x-ray radiation. Surgery with cyst drainage Surgery may be done to drain tumors that are mostly fluid-filled cysts. This lowers pressure in the brain and relieves symptoms. A catheter (thin tube) is inserted into the cyst and a small container is placed under the skin. The fluid drains into the container and is later removed. Sometimes, after the cyst is drained, a drug is put through the catheter into the cyst. This causes the inside wall of the cyst to scar and stops the cyst from making fluid or increases the amount of the time it takes for the fluid to build up again. Surgery to remove the tumor may be done after the cyst is drained. Chemotherapy Chemotherapy is a treatment that uses anticancer drugs to stop the growth of tumor cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach tumor cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid or an organ, the drugs mainly affect tumor cells in those areas (regional chemotherapy). Intracavitary chemotherapy is a type of regional chemotherapy that places drugs directly into a cavity, such as a cyst. It is used for craniopharyngioma that has come back after treatment. Biologic therapy Biologic therapy is a treatment that uses the patients immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the bodys natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy. For craniopharyngioma that has come back after treatment, the biologic therapy drug is placed inside the tumor using a catheter (intracavitary) or in a vein (intravenous). Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the medical research process. Clinical trials are done to find out if new treatments are safe and effective or better than the standard treatment. Many of today's standard treatments are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way diseases will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Check the list of NCI-supported cancer clinical trials that are now accepting patients with childhood craniopharyngioma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Patients can enter clinical trials before, during, or after starting their treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients who have not improved. There are also clinical trials that test new ways to stop a disease from recurring (coming back) or reduce the side effects of treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's clinical trials database. Follow-up tests may be needed. Some of the tests that were done to diagnose the disease or decide how to treat it may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed. These tests are sometimes called follow-up tests or check-ups. After treatment, follow-up testing with MRI will be done for several years to check if the tumor has come back. Treatment Options for Childhood Craniopharyngioma Newly Diagnosed Childhood Craniopharyngioma Treatment of newly diagnosed childhood craniopharyngioma may include the following: - Surgery (resection) with or without radiation therapy. - Partial resection followed by radiation therapy. - Cyst drainage with or without radiation therapy or surgery. Check the list of NCI-supported cancer clinical trials that are now accepting patients with childhood craniopharyngioma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Recurrent Childhood Craniopharyngioma Craniopharyngioma may recur (come back) no matter how it was treated the first time. Treatment options for recurrent childhood craniopharyngioma depend on the type of treatment that was given when the tumor was first diagnosed and the needs of the child. Treatment may include the following: - Surgery (resection). - External-beam radiation therapy. - Stereotactic radiosurgery. - Intracavitary radiation therapy. - Intracavitary chemotherapy or intracavitary biologic therapy. - Intravenous biologic therapy. - Cyst drainage. - A clinical trial of biologic therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with childhood craniopharyngioma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website.",Childhood Craniopharyngioma,0000006_8,CancerGov,https://www.cancer.gov/types/brain/patient/child-cranio-treatment-pdq,C0278652,T191,Disorders What is (are) Childhood Ependymoma ?,0000006_9-1,information,"Key Points - Childhood ependymoma is a disease in which malignant (cancer) cells form in the tissues of the brain and spinal cord. - There are different types of ependymomas. - The part of the brain that is affected depends on where the ependymoma forms. - The cause of most childhood brain tumors is unknown. - The signs and symptoms of childhood ependymoma are not the same in every child. - Tests that examine the brain and spinal cord are used to detect (find) childhood ependymoma. - Childhood ependymoma is diagnosed and removed in surgery. - Certain factors affect prognosis (chance of recovery) and treatment options. Childhood ependymoma is a disease in which malignant (cancer) cells form in the tissues of the brain and spinal cord. The brain controls vital functions such as memory and learning, emotion, and the senses (hearing, sight, smell, taste, and touch). The spinal cord is made up of bundles of nerve fibers that connect the brain with nerves in most parts of the body. Ependymomas form from ependymal cells that line the ventricles and passageways in the brain and the spinal cord. Ependymal cells make cerebrospinal fluid (CSF). This summary is about the treatment of primary brain tumors (tumors that begin in the brain). Treatment of metastatic brain tumors, which are tumors that begin in other parts of the body and spread to the brain, is not discussed in this summary. There are many different types of brain tumors. Brain tumors can occur in both children and adults. However, treatment for children is different than treatment for adults. See the following PDQ summaries for more information: - Childhood Brain and Spinal Cord Tumors Treatment Overview - Adult Central Nervous System Tumors Treatment There are different types of ependymomas. The World Health Organization (WHO) groups ependymal tumors into five main subtypes: - Subependymoma (WHO Grade I). - Myxopapillary ependymoma (WHO Grade I). - Ependymoma (WHO Grade II). - RELA fusionpositive ependymoma (WHO Grade II or Grade III with change in the RELA gene). - Anaplastic ependymoma (WHO Grade III). The grade of a tumor describes how abnormal the cancer cells look under a microscope and how quickly the tumor is likely to grow and spread. Low-grade (Grade I) cancer cells look more like normal cells than high-grade cancer cells (Grade II and III). They also tend to grow and spread more slowly than Grade II and III cancer cells. The part of the brain that is affected depends on where the ependymoma forms. Ependymomas can form anywhere in the fluid -filled ventricles and passageways in the brain and spinal cord. Most ependymomas form in the fourth ventricle and affect the cerebellum and the brain stem. Once an ependymoma forms, areas of the brain that may be affected include: - Cerebrum: The largest part of the brain, at the top of the head. The cerebrum controls thinking, learning, problem-solving, speech, emotions, reading, writing, and voluntary movement. - Cerebellum: The lower, back part of the brain (near the middle of the back of the head). The cerebellum controls movement, balance, and posture. - Brain stem: The part that connects the brain to the spinal cord, in the lowest part of the brain (just above the back of the neck). The brain stem controls breathing, heart rate, and the nerves and muscles used in seeing, hearing, walking, talking, and eating. - Spinal cord: The column of nerve tissue that runs from the brain stem down the center of the back. It is covered by three thin layers of tissue called membranes. The spinal cord and membranes are surrounded by the vertebrae (back bones). Spinal cord nerves carry messages between the brain and the rest of the body, such as a message from the brain to cause muscles to move or a message from the skin to the brain to feel touch.",Childhood Ependymoma,0000006_9,CancerGov,https://www.cancer.gov/types/brain/patient/child-ependymoma-treatment-pdq,C1851584,T191,Disorders What causes Childhood Ependymoma ?,0000006_9-2,causes,The cause of most childhood brain tumors is unknown.,Childhood Ependymoma,0000006_9,CancerGov,https://www.cancer.gov/types/brain/patient/child-ependymoma-treatment-pdq,C1851584,T191,Disorders What are the symptoms of Childhood Ependymoma ?,0000006_9-3,symptoms,The signs and symptoms of childhood ependymoma are not the same in every child. Signs and symptoms depend on the following: - The child's age. - Where the tumor has formed. Signs and symptoms may be caused by childhood ependymoma or by other conditions. Check with your child's doctor if your child has any of the following: - Frequent headaches. - Seizures. - Nausea and vomiting. - Pain or stiffness in the neck. - Loss of balance or trouble walking. - Weakness in the legs. - Blurry vision. - Back pain. - A change in bowel function. - Trouble urinating. - Confusion or irritability.,Childhood Ependymoma,0000006_9,CancerGov,https://www.cancer.gov/types/brain/patient/child-ependymoma-treatment-pdq,C1851584,T191,Disorders How to diagnose Childhood Ependymoma ?,0000006_9-4,exams and tests,"Tests that examine the brain and spinal cord are used to detect (find) childhood ependymoma. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Neurological exam : A series of questions and tests to check the brain, spinal cord, and nerve function. The exam checks a persons mental status, coordination, and ability to walk normally, and how well the muscles, senses, and reflexes work. This may also be called a neuro exam or a neurologic exam. - MRI (magnetic resonance imaging) with gadolinium : A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the brain and spinal cord. A substance called gadolinium is injected into a vein and travels through the bloodstream. The gadolinium collects around the cancer cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Lumbar puncture : A procedure used to collect cerebrospinal fluid (CSF) from the spinal column. This is done by placing a needle between two bones in the spine and into the CSF around the spinal cord and removing a sample of fluid. The sample of CSF is checked under a microscope for signs of tumor cells. The sample may also be checked for the amounts of protein and glucose. A higher than normal amount of protein or lower than normal amount of glucose may be a sign of a tumor. This procedure is also called an LP or spinal tap. Childhood ependymoma is diagnosed and removed in surgery. If the diagnostic tests show there may be a brain tumor, a biopsy is done by removing part of the skull and using a needle to remove a sample of the brain tissue. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are found, the doctor will remove as much tumor as safely possible during the same surgery. The following test may be done on the tissue that was removed: - Immunohistochemistry : A test that uses antibodies to check for certain antigens in a sample of tissue. The antibody is usually linked to a radioactive substance or a dye that causes the tissue to light up under a microscope. This type of test may be used to tell the difference between brain stem glioma and other brain tumors. An MRI is often done after the tumor is removed to find out whether any tumor remains.",Childhood Ependymoma,0000006_9,CancerGov,https://www.cancer.gov/types/brain/patient/child-ependymoma-treatment-pdq,C1851584,T191,Disorders What is the outlook for Childhood Ependymoma ?,0000006_9-5,outlook,Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on: - Where the tumor has formed in the central nervous system (CNS). - Whether there are certain changes in the genes or chromosomes. - Whether any cancer cells remain after surgery to remove the tumor. - The type of ependymoma. - The age of the child when the tumor is diagnosed. - Whether the cancer has spread to other parts of the brain or spinal cord. - Whether the tumor has just been diagnosed or has recurred (come back). Prognosis also depends on the type and dose of radiation therapy that is given.,Childhood Ependymoma,0000006_9,CancerGov,https://www.cancer.gov/types/brain/patient/child-ependymoma-treatment-pdq,C1851584,T191,Disorders what research (or clinical trials) is being done for Childhood Ependymoma ?,0000006_9-6,research,"New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Childhood Ependymoma,0000006_9,CancerGov,https://www.cancer.gov/types/brain/patient/child-ependymoma-treatment-pdq,C1851584,T191,Disorders What are the stages of Childhood Ependymoma ?,0000006_9-7,stages,Key Points - The area where the tumor is found and the childs age are used in place of a staging system to plan cancer treatment. - The information from tests and procedures done to detect (find) childhood ependymoma is used to plan cancer treatment. The area where the tumor is found and the childs age are used in place of a staging system to plan cancer treatment. Staging is the process used to find out how much cancer there is and if cancer has spread. There is no standard staging system for childhood ependymoma. Treatment is based on where the cancer is in the body and the age of the child. The information from tests and procedures done to detect (find) childhood ependymoma is used to plan cancer treatment. Some of the tests used to detect childhood ependymoma are repeated after the tumor is removed by surgery. (See the General Information section.) This is to find out how much tumor remains after surgery.,Childhood Ependymoma,0000006_9,CancerGov,https://www.cancer.gov/types/brain/patient/child-ependymoma-treatment-pdq,C1851584,T191,Disorders What are the treatments for Childhood Ependymoma ?,0000006_9-8,treatment,"Key Points - There are different types of treatment for children with ependymoma. - Children with ependymoma should have their treatment planned by a team of health care providers who are experts in treating childhood brain tumors. - Childhood brain and spinal cord tumors may cause signs or symptoms that begin before the cancer is diagnosed and continue for months or years. - Some cancer treatments cause side effects months or years after treatment has ended. - Four types of standard treatment are used: - Surgery - Radiation therapy - Chemotherapy - Observation - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for children with ependymoma. Different types of treatment are available for children with ependymoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment. Children with ependymoma should have their treatment planned by a team of health care providers who are experts in treating childhood brain tumors. Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other pediatric health care providers who are experts in treating children with brain tumors and who specialize in certain areas of medicine. These may include the following specialists: - Pediatric neurosurgeon. - Neurologist. - Neuropathologist. - Neuroradiologist. - Pediatrician. - Rehabilitation specialist. - Radiation oncologist. - Medical oncologist. - Endocrinologist. - Psychologist. Childhood brain and spinal cord tumors may cause signs or symptoms that begin before the cancer is diagnosed and continue for months or years. Childhood brain and spinal cord tumors may cause signs or symptoms that continue for months or years. Signs or symptoms caused by the tumor may begin before diagnosis. Signs or symptoms caused by treatment may begin during or right after treatment. Some cancer treatments cause side effects months or years after treatment has ended. These are called late effects. Late effects of cancer treatment may include the following: - Physical problems. - Changes in mood, feelings, thinking, learning, or memory. - Second cancers (new types of cancer). Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information). Four types of standard treatment are used: Surgery If the results of diagnostic tests show there may be a brain tumor, a biopsy is done by removing part of the skull and using a needle to remove a sample of the brain tissue. A pathologist views the tissue under a microscope to check for cancer cells. If cancer cells are found, the doctor will remove as much tumor as safely possible during the same surgery. An MRI is often done after the tumor is removed to find out whether any tumor remains. If tumor remains, a second surgery to remove as much of the remaining tumor as possible may be done. Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment to lower the risk that the cancer will come back after surgery is called adjuvant therapy. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. Certain ways of giving radiation therapy can help keep radiation from damaging nearby healthy tissue. These types of radiation therapy include the following: - Conformal radiation therapy: Conformal radiation therapy is a type of external radiation therapy that uses a computer to make a 3-dimensional (3-D) picture of the tumor and shapes the radiation beams to fit the tumor. - Intensity-modulated radiation therapy (IMRT): IMRT is a type of 3-dimensional (3-D) radiation therapy that uses a computer to make pictures of the size and shape of the tumor. Thin beams of radiation of different intensities (strengths) are aimed at the tumor from many angles. - Proton-beam radiation therapy: Proton-beam therapy is a type of high-energy, external radiation therapy. A radiation therapy machine aims streams of protons (tiny, invisible, positively-charged particles) at the cancer cells to kill them. - Stereotactic radiosurgery: Stereotactic radiosurgery is a type of external radiation therapy. A rigid head frame is attached to the skull to keep the head still during the radiation treatment. A machine aims a single large dose of radiation directly at the tumor. This procedure does not involve surgery. It is also called stereotaxic radiosurgery, radiosurgery, and radiation surgery. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type of cancer being treated. External radiation therapy is used to treat childhood ependymoma. Children younger than 3 years who receive radiation therapy to the brain have a higher risk of problems with growth and development than older children. 3-D conformal radiation therapy and proton-beam therapy are being studied in children younger than 3 years to see if the effects of radiation on growth and development are lessened. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type of cancer being treated. Observation Observation is closely monitoring a patients condition without giving any treatment until signs or symptoms appear or change. Observation may be used to treat a child with a subependymoma who has no symptoms and whose tumor is found while treating another condition. New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Follow-up tests for childhood ependymoma include an MRI (magnetic resonance imaging) of the brain and spinal cord every 3 months for the first 1 or 2 years after treatment. After 2 years, MRIs may be done every 6 months for the next 3 years. Treatment Options for Childhood Ependymoma Newly Diagnosed Childhood Ependymoma A child with a newly diagnosed ependymoma has not had treatment for the tumor. The child may have had treatment to relieve signs or symptoms caused by the tumor. Subependymoma Treatment of newly diagnosed subependymoma (WHO Grade I) is: - Surgery. - Observation (rarely). Myxopapillary ependymoma Treatment of newly diagnosed myxopapillary ependymoma (WHO Grade I) is: - Surgery with or without radiation therapy. Childhood ependymoma, anaplastic ependymoma, or RELA fusionpositive ependymoma Treatment of newly diagnosed childhood ependymoma (WHO Grade II), anaplastic ependymoma (WHO Grade III), or RELA fusionpositive ependymoma (WHO Grade II or Grade III) is: - Surgery. After surgery, the plan for further treatment depends on the following: - Whether any cancer cells remain after surgery. - Whether the cancer has spread to other parts of the brain or spinal cord. - The age of the child. When the tumor is completely removed and cancer cells have not spread, treatment may include the following: - Radiation therapy. - A clinical trial of radiation therapy followed by chemotherapy. - A clinical trial of observation for patients whose tumor is completely removed or who have no sign of cancer after chemotherapy. When part of the tumor remains after surgery, but cancer cells have not spread, treatment may include the following: - A second surgery to remove as much of the remaining tumor as possible. - Radiation therapy. - Chemotherapy followed by radiation therapy. - A clinical trial of chemotherapy given before and after radiation therapy. When cancer cells have spread within the brain and spinal cord, treatment may include the following: - Radiation therapy to the brain and spinal cord. Treatment for children younger than 3 years of age may include the following: - Chemotherapy. - Radiation therapy. - A clinical trial of 3-dimensional (3-D) conformal radiation therapy or proton-beam radiation therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with newly diagnosed childhood ependymoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Recurrent Childhood Ependymoma Treatment of recurrent childhood ependymoma may include the following: - Surgery. - Radiation therapy, which may include stereotactic radiosurgery, intensity-modulated radiation therapy, or proton-beam radiation therapy. - Chemotherapy. - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent childhood ependymoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website.",Childhood Ependymoma,0000006_9,CancerGov,https://www.cancer.gov/types/brain/patient/child-ependymoma-treatment-pdq,C1851584,T191,Disorders What is (are) Adult Primary Liver Cancer ?,0000007_1-1,information,"Key Points - Adult primary liver cancer is a disease in which malignant (cancer) cells form in the tissues of the liver. - There are two types of adult primary liver cancer. - Having hepatitis or cirrhosis can affect the risk of adult primary liver cancer. - Signs and symptoms of adult primary liver cancer include a lump or pain on the right side. - Tests that examine the liver and the blood are used to detect (find) and diagnose adult primary liver cancer. - Certain factors affect prognosis (chance of recovery) and treatment options. Adult primary liver cancer is a disease in which malignant (cancer) cells form in the tissues of the liver. The liver is one of the largest organs in the body. It has four lobes and fills the upper right side of the abdomen inside the rib cage. Three of the many important functions of the liver are: - To filter harmful substances from the blood so they can be passed from the body in stools and urine. - To make bile to help digest fat that comes from food. - To store glycogen (sugar), which the body uses for energy. There are two types of adult primary liver cancer. The two types of adult primary liver cancer are: - Hepatocellular carcinoma. - Cholangiocarcinoma (bile duct cancer). (See the PDQ summary on Bile Duct Cancer Treatment for more information.) The most common type of adult primary liver cancer is hepatocellular carcinoma. This type of liver cancer is the third leading cause of cancer-related deaths worldwide. This summary is about the treatment of primary liver cancer (cancer that begins in the liver). Treatment of cancer that begins in other parts of the body and spreads to the liver is not covered in this summary. Primary liver cancer can occur in both adults and children. However, treatment for children is different than treatment for adults. (See the PDQ summary on Childhood Liver Cancer Treatment for more information.)",Adult Primary Liver Cancer,0000007_1,CancerGov,https://www.cancer.gov/types/liver/patient/adult-liver-treatment-pdq,C0220630,T191,Disorders Who is at risk for Adult Primary Liver Cancer? ?,0000007_1-2,susceptibility,"Having hepatitis or cirrhosis can affect the risk of adult primary liver cancer. Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesnt mean that you will not get cancer. Talk with your doctor if you think you may be at risk. The following are risk factors for adult primary liver cancer: - Having hepatitis B or hepatitis C. Having both hepatitis B and hepatitis C increases the risk even more. - Having cirrhosis, which can be caused by: - hepatitis (especially hepatitis C); or - drinking large amounts of alcohol for many years or being an alcoholic. - Having metabolic syndrome, a set of conditions that occur together, including extra fat around the abdomen, high blood sugar, high blood pressure, high levels of triglycerides and low levels of high-density lipoproteins in the blood. - Having liver injury that is long-lasting, especially if it leads to cirrhosis. - Having hemochromatosis, a condition in which the body takes up and stores more iron than it needs. The extra iron is stored in the liver, heart, and pancreas - Eating foods tainted with aflatoxin (poison from a fungus that can grow on foods, such as grains and nuts, that have not been stored properly).",Adult Primary Liver Cancer,0000007_1,CancerGov,https://www.cancer.gov/types/liver/patient/adult-liver-treatment-pdq,C0220630,T191,Disorders What are the symptoms of Adult Primary Liver Cancer ?,0000007_1-3,symptoms,"Signs and symptoms of adult primary liver cancer include a lump or pain on the right side. These and other signs and symptoms may be caused by adult primary liver cancer or by other conditions. Check with your doctor if you have any of the following: - A hard lump on the right side just below the rib cage. - Discomfort in the upper abdomen on the right side. - A swollen abdomen. - Pain near the right shoulder blade or in the back. - Jaundice (yellowing of the skin and whites of the eyes). - Easy bruising or bleeding. - Unusual tiredness or weakness. - Nausea and vomiting. - Loss of appetite or feelings of fullness after eating a small meal. - Weight loss for no known reason. - Pale, chalky bowel movements and dark urine. - Fever.",Adult Primary Liver Cancer,0000007_1,CancerGov,https://www.cancer.gov/types/liver/patient/adult-liver-treatment-pdq,C0220630,T191,Disorders How to diagnose Adult Primary Liver Cancer ?,0000007_1-4,exams and tests,"Tests that examine the liver and the blood are used to detect (find) and diagnose adult primary liver cancer. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Serum tumor marker test : A procedure in which a sample of blood is examined to measure the amounts of certain substances released into the blood by organs, tissues, or tumor cells in the body. Certain substances are linked to specific types of cancer when found in increased levels in the blood. These are called tumor markers. An increased level of alpha-fetoprotein (AFP) in the blood may be a sign of liver cancer. Other cancers and certain noncancerous conditions, including cirrhosis and hepatitis, may also increase AFP levels. Sometimes the AFP level is normal even when there is liver cancer. - Liver function tests : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by the liver. A higher than normal amount of a substance can be a sign of liver cancer. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the abdomen, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. Images may be taken at three different times after the dye is injected, to get the best picture of abnormal areas in the liver. This is called triple-phase CT. A spiral or helical CT scan makes a series of very detailed pictures of areas inside the body using an x-ray machine that scans the body in a spiral path. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body, such as the liver. This procedure is also called nuclear magnetic resonance imaging (NMRI). To create detailed pictures of blood vessels in and near the liver, dye is injected into a vein. This procedure is called MRA (magnetic resonance angiography). Images may be taken at three different times after the dye is injected, to get the best picture of abnormal areas in the liver. This is called triple-phase MRI. - Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later. - Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. Procedures used to collect the sample of cells or tissues include the following: - Fine-needle aspiration biopsy : The removal of cells, tissue or fluid using a thin needle. - Core needle biopsy : The removal of cells or tissue using a slightly wider needle. - Laparoscopy : A surgical procedure to look at the organs inside the abdomen to check for signs of disease. Small incisions (cuts) are made in the wall of the abdomen and a laparoscope (a thin, lighted tube) is inserted into one of the incisions. Another instrument is inserted through the same or another incision to remove the tissue samples. A biopsy is not always needed to diagnose adult primary liver cancer.",Adult Primary Liver Cancer,0000007_1,CancerGov,https://www.cancer.gov/types/liver/patient/adult-liver-treatment-pdq,C0220630,T191,Disorders What is the outlook for Adult Primary Liver Cancer ?,0000007_1-5,outlook,"Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - The stage of the cancer (the size of the tumor, whether it affects part or all of the liver, or has spread to other places in the body). - How well the liver is working. - The patients general health, including whether there is cirrhosis of the liver.",Adult Primary Liver Cancer,0000007_1,CancerGov,https://www.cancer.gov/types/liver/patient/adult-liver-treatment-pdq,C0220630,T191,Disorders What are the stages of Adult Primary Liver Cancer ?,0000007_1-6,stages,"Key Points - After adult primary liver cancer has been diagnosed, tests are done to find out if cancer cells have spread within the liver or to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - The Barcelona Clinic Liver Cancer Staging System may be used to stage adult primary liver cancer. - The following groups are used to plan treatment. - BCLC stages 0, A, and B - BCLC stages C and D After adult primary liver cancer has been diagnosed, tests are done to find out if cancer cells have spread within the liver or to other parts of the body. The process used to find out if cancer has spread within the liver or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following tests and procedures may be used in the staging process: - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the chest, abdomen, and pelvis, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if primary liver cancer spreads to the lung, the cancer cells in the lung are actually liver cancer cells. The disease is metastatic liver cancer, not lung cancer. The Barcelona Clinic Liver Cancer Staging System may be used to stage adult primary liver cancer. There are several staging systems for liver cancer. The Barcelona Clinic Liver Cancer (BCLC) Staging System is widely used and is described below. This system is used to predict the patient's chance of recovery and to plan treatment, based on the following: - Whether the cancer has spread within the liver or to other parts of the body. - How well the liver is working. - The general health and wellness of the patient. - The symptoms caused by the cancer. The BCLC staging system has five stages: - Stage 0: Very early - Stage A: Early - Stage B: Intermediate - Stage C: Advanced - Stage D: End-stage The following groups are used to plan treatment. BCLC stages 0, A, and B Treatment to cure the cancer is given for BCLC stages 0, A, and B. BCLC stages C and D Treatment to relieve the symptoms caused by liver cancer and improve the patient's quality of life is given for BCLC stages C and D. Treatments are not likely to cure the cancer.",Adult Primary Liver Cancer,0000007_1,CancerGov,https://www.cancer.gov/types/liver/patient/adult-liver-treatment-pdq,C0220630,T191,Disorders what research (or clinical trials) is being done for Adult Primary Liver Cancer ?,0000007_1-7,research,"New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Adult Primary Liver Cancer,0000007_1,CancerGov,https://www.cancer.gov/types/liver/patient/adult-liver-treatment-pdq,C0220630,T191,Disorders What are the treatments for Adult Primary Liver Cancer ?,0000007_1-8,treatment,"Key Points - There are different types of treatment for patients with adult primary liver cancer. - Patients with liver cancer are treated by a team of specialists who are experts in treating liver cancer. - Seven types of standard treatment are used: - Surveillance - Surgery - Liver transplant - Ablation therapy - Embolization therapy - Targeted therapy - Radiation therapy - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with adult primary liver cancer. Different types of treatments are available for patients with adult primary liver cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Patients with liver cancer are treated by a team of specialists who are experts in treating liver cancer. The patient's treatment will be overseen by a medical oncologist, a doctor who specializes in treating people with cancer. The medical oncologist may refer the patient to other health professionals who have special training in treating patients with liver cancer. These may include the following specialists: - Hepatologist (specialist in liver disease). - Surgical oncologist. - Transplant surgeon. - Radiation oncologist. - Interventional radiologist (a specialist who diagnoses and treats diseases using imaging and the smallest incisions possible). - Pathologist. Seven types of standard treatment are used: Surveillance Surveillance for lesions smaller than 1 centimeter found during screening. Follow-up every three months is common. Surgery A partial hepatectomy (surgery to remove the part of the liver where cancer is found) may be done. A wedge of tissue, an entire lobe, or a larger part of the liver, along with some of the healthy tissue around it is removed. The remaining liver tissue takes over the functions of the liver and may regrow. Liver transplant In a liver transplant, the entire liver is removed and replaced with a healthy donated liver. A liver transplant may be done when the disease is in the liver only and a donated liver can be found. If the patient has to wait for a donated liver, other treatment is given as needed. Ablation therapy Ablation therapy removes or destroys tissue. Different types of ablation therapy are used for liver cancer: - Radiofrequency ablation: The use of special needles that are inserted directly through the skin or through an incision in the abdomen to reach the tumor. High-energy radio waves heat the needles and tumor which kills cancer cells. - Microwave therapy: A type of treatment in which the tumor is exposed to high temperatures created by microwaves. This can damage and kill cancer cells or make them more sensitive to the effects of radiation and certain anticancer drugs. - Percutaneous ethanol injection: A cancer treatment in which a small needle is used to inject ethanol (pure alcohol) directly into a tumor to kill cancer cells. Several treatments may be needed. Usually local anesthesia is used, but if the patient has many tumors in the liver, general anesthesia may be used. - Cryoablation: A treatment that uses an instrument to freeze and destroy cancer cells. This type of treatment is also called cryotherapy and cryosurgery. The doctor may use ultrasound to guide the instrument. - Electroporation therapy: A treatment that sends electrical pulses through an electrode placed in a tumor to kill cancer cells. Electroporation therapy is being studied in clinical trials. Embolization therapy Embolization therapy is the use of substances to block or decrease the flow of blood through the hepatic artery to the tumor. When the tumor does not get the oxygen and nutrients it needs, it will not continue to grow. Embolization therapy is used for patients who cannot have surgery to remove the tumor or ablation therapy and whose tumor has not spread outside the liver. The liver receives blood from the hepatic portal vein and the hepatic artery. Blood that comes into the liver from the hepatic portal vein usually goes to the healthy liver tissue. Blood that comes from the hepatic artery usually goes to the tumor. When the hepatic artery is blocked during embolization therapy, the healthy liver tissue continues to receive blood from the hepatic portal vein. There are two main types of embolization therapy: - Transarterial embolization (TAE): A small incision (cut) is made in the inner thigh and a catheter (thin, flexible tube) is inserted and threaded up into the hepatic artery. Once the catheter is in place, a substance that blocks the hepatic artery and stops blood flow to the tumor is injected. - Transarterial chemoembolization (TACE): This procedure is like TAE except an anticancer drug is also given. The procedure can be done by attaching the anticancer drug to small beads that are injected into the hepatic artery or by injecting the anticancer drug through the catheter into the hepatic artery and then injecting the substance to block the hepatic artery. Most of the anticancer drug is trapped near the tumor and only a small amount of the drug reaches other parts of the body. This type of treatment is also called chemoembolization. Targeted therapy Targeted therapy is a treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Adult liver cancer may be treated with a targeted therapy drug that stops cells from dividing and prevents the growth of new blood vessels that tumors need to grow. See Drugs Approved for Liver Cancer for more information. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. Certain ways of giving radiation therapy can help keep radiation from damaging nearby healthy tissue. These types of external radiation therapy include the following: - Conformal radiation therapy: Conformal radiation therapy is a type of external radiation therapy that uses a computer to make a 3-dimensional (3-D) picture of the tumor and shapes the radiation beams to fit the tumor. This allows a high dose of radiation to reach the tumor and causes less damage to nearby healthy tissue. - Stereotactic body radiation therapy: Stereotactic body radiation therapy is a type of external radiation therapy. Special equipment is used to place the patient in the same position for each radiation treatment. Once a day for several days, a radiation machine aims a larger than usual dose of radiation directly at the tumor. By having the patient in the same position for each treatment, there is less damage to nearby healthy tissue. This procedure is also called stereotactic external-beam radiation therapy and stereotaxic radiation therapy. - Proton beam radiation therapy: Proton-beam therapy is a type of high-energy, external radiation therapy. A radiation therapy machine aims streams of protons (tiny, invisible, positively-charged particles) at the cancer cells to kill them. This type of treatment causes less damage to nearby healthy tissue. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External radiation therapy is used to treat adult primary liver cancer. New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Adult Primary Liver Cancer Stages 0, A, and B Adult Primary Liver Cancer Treatment of stages 0, A, and B adult primary liver cancer may include the following: - Surveillance for lesions smaller than 1 centimeter. - Partial hepatectomy. - Total hepatectomy and liver transplant. - Ablation of the tumor using one of the following methods: - Radiofrequency ablation. - Microwave therapy. - Percutaneous ethanol injection. - Cryoablation. - A clinical trial of electroporation therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage 0 adult primary liver cancer (BCLC), stage A adult primary liver cancer (BCLC) and stage B adult primary liver cancer (BCLC). For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stages C and D Adult Primary Liver Cancer Treatment of stages C and D adult primary liver cancer may include the following: - Embolization therapy using one of the following methods: - Transarterial embolization (TAE). - Transarterial chemoembolization (TACE). - Targeted therapy. - Radiation therapy. - A clinical trial of targeted therapy after chemoembolization or combined with chemotherapy. - A clinical trial of new targeted therapy drugs. - A clinical trial of targeted therapy with or without stereotactic body radiation therapy. - A clinical trial of stereotactic body radiation therapy or proton-beam radiation therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage C adult primary liver cancer (BCLC) and stage D adult primary liver cancer (BCLC). For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Adult Primary Liver Cancer,0000007_1,CancerGov,https://www.cancer.gov/types/liver/patient/adult-liver-treatment-pdq,C0220630,T191,Disorders What is (are) Bile Duct Cancer (Cholangiocarcinoma) ?,0000007_2-1,information,"Key Points - Bile duct cancer is a rare disease in which malignant (cancer) cells form in the bile ducts. - Having colitis or certain liver diseases can increase the risk of bile duct cancer. - Signs of bile duct cancer include jaundice and pain in the abdomen. - Tests that examine the bile ducts and nearby organs are used to detect (find), diagnose, and stage bile duct cancer. - Different procedures may be used to obtain a sample of tissue and diagnose bile duct cancer. - Certain factors affect prognosis (chance of recovery) and treatment options. Bile duct cancer is a rare disease in which malignant (cancer) cells form in the bile ducts. A network of tubes, called ducts, connects the liver, gallbladder, and small intestine. This network begins in the liver where many small ducts collect bile (a fluid made by the liver to break down fats during digestion). The small ducts come together to form the right and left hepatic ducts, which lead out of the liver. The two ducts join outside the liver and form the common hepatic duct. The cystic duct connects the gallbladder to the common hepatic duct. Bile from the liver passes through the hepatic ducts, common hepatic duct, and cystic duct and is stored in the gallbladder. When food is being digested, bile stored in the gallbladder is released and passes through the cystic duct to the common bile duct and into the small intestine. Bile duct cancer is also called cholangiocarcinoma. There are two types of bile duct cancer: - Intrahepatic bile duct cancer : This type of cancer forms in the bile ducts inside the liver. Only a small number of bile duct cancers are intrahepatic. Intrahepatic bile duct cancers are also called intrahepatic cholangiocarcinomas. - Extrahepatic bile duct cancer : The extrahepatic bile duct is made up of the hilum region and the distal region. Cancer can form in either region: - Perihilar bile duct cancer: This type of cancer is found in the hilum region, the area where the right and left bile ducts exit the liver and join to form the common hepatic duct. Perihilar bile duct cancer is also called a Klatskin tumor or perihilar cholangiocarcinoma. - Distal extrahepatic bile duct cancer: This type of cancer is found in the distal region. The distal region is made up of the common bile duct which passes through the pancreas and ends in the small intestine. Distal extrahepatic bile duct cancer is also called extrahepatic cholangiocarcinoma.",Bile Duct Cancer (Cholangiocarcinoma),0000007_2,CancerGov,https://www.cancer.gov/types/liver/patient/bile-duct-treatment-pdq,C0206698,T191,Disorders Who is at risk for Bile Duct Cancer (Cholangiocarcinoma)? ?,0000007_2-2,susceptibility,"Having colitis or certain liver diseases can increase the risk of bile duct cancer. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. People who think they may be at risk should discuss this with their doctor. Risk factors for bile duct cancer include the following conditions: - Primary sclerosing cholangitis (a progressive disease in which the bile ducts become blocked by inflammation and scarring). - Chronic ulcerative colitis. - Cysts in the bile ducts (cysts block the flow of bile and can cause swollen bile ducts, inflammation, and infection). - Infection with a Chinese liver fluke parasite.",Bile Duct Cancer (Cholangiocarcinoma),0000007_2,CancerGov,https://www.cancer.gov/types/liver/patient/bile-duct-treatment-pdq,C0206698,T191,Disorders What are the symptoms of Bile Duct Cancer (Cholangiocarcinoma) ?,0000007_2-3,symptoms,Signs of bile duct cancer include jaundice and pain in the abdomen. These and other signs and symptoms may be caused by bile duct cancer or by other conditions. Check with your doctor if you have any of the following: - Jaundice (yellowing of the skin or whites of the eyes). - Dark urine. - Clay colored stool. - Pain in the abdomen. - Fever. - Itchy skin. - Nausea and vomiting. - Weight loss for an unknown reason.,Bile Duct Cancer (Cholangiocarcinoma),0000007_2,CancerGov,https://www.cancer.gov/types/liver/patient/bile-duct-treatment-pdq,C0206698,T191,Disorders How to diagnose Bile Duct Cancer (Cholangiocarcinoma) ?,0000007_2-4,exams and tests,"Tests that examine the bile ducts and nearby organs are used to detect (find), diagnose, and stage bile duct cancer. Procedures that make pictures of the bile ducts and the nearby area help diagnose bile duct cancer and show how far the cancer has spread. The process used to find out if cancer cells have spread within and around the bile ducts or to distant parts of the body is called staging. In order to plan treatment, it is important to know if the bile duct cancer can be removed by surgery. Tests and procedures to detect, diagnose, and stage bile duct cancer are usually done at the same time. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Liver function tests : A procedure in which a blood sample is checked to measure the amounts of bilirubin and alkaline phosphatase released into the blood by the liver. A higher than normal amount of these substances can be a sign of liver disease that may be caused by bile duct cancer. - Laboratory tests : Medical procedures that test samples of tissue, blood, urine, or other substances in the body. These tests help to diagnose disease, plan and check treatment, or monitor the disease over time. - Carcinoembryonic antigen (CEA) and CA 19-9 tumor marker test : A procedure in which a sample of blood, urine, or tissue is checked to measure the amounts of certain substances made by organs, tissues, or tumor cells in the body. Certain substances are linked to specific types of cancer when found in increased levels in the body. These are called tumor markers. Higher than normal levels of carcinoembryonic antigen (CEA) and CA 19-9 may mean there is bile duct cancer. - Ultrasound exam : A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs, such as the abdomen, and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the abdomen, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - MRCP (magnetic resonance cholangiopancreatography): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body such as the liver, bile ducts, gallbladder, pancreas, and pancreatic duct. Different procedures may be used to obtain a sample of tissue and diagnose bile duct cancer. Cells and tissues are removed during a biopsy so they can be viewed under a microscope by a pathologist to check for signs of cancer. Different procedures may be used to obtain the sample of cells and tissue. The type of procedure used depends on whether the patient is well enough to have surgery. Types of biopsy procedures include the following: - Laparoscopy : A surgical procedure to look at the organs inside the abdomen, such as the bile ducts and liver, to check for signs of cancer. Small incisions (cuts) are made in the wall of the abdomen and a laparoscope (a thin, lighted tube) is inserted into one of the incisions. Other instruments may be inserted through the same or other incisions to perform procedures such as taking tissue samples to be checked for signs of cancer. - Percutaneous transhepatic cholangiography (PTC): A procedure used to x-ray the liver and bile ducts. A thin needle is inserted through the skin below the ribs and into the liver. Dye is injected into the liver or bile ducts and an x-ray is taken. A sample of tissue is removed and checked for signs of cancer. If the bile duct is blocked, a thin, flexible tube called a stent may be left in the liver to drain bile into the small intestine or a collection bag outside the body. This procedure may be used when a patient cannot have surgery. - Endoscopic retrograde cholangiopancreatography (ERCP): A procedure used to x-ray the ducts (tubes) that carry bile from the liver to the gallbladder and from the gallbladder to the small intestine. Sometimes bile duct cancer causes these ducts to narrow and block or slow the flow of bile, causing jaundice. An endoscope is passed through the mouth and stomach and into the small intestine. Dye is injected through the endoscope (thin, tube-like instrument with a light and a lens for viewing) into the bile ducts and an x-ray is taken. A sample of tissue is removed and checked for signs of cancer. If the bile duct is blocked, a thin tube may be inserted into the duct to unblock it. This tube (or stent) may be left in place to keep the duct open. This procedure may be used when a patient cannot have surgery.",Bile Duct Cancer (Cholangiocarcinoma),0000007_2,CancerGov,https://www.cancer.gov/types/liver/patient/bile-duct-treatment-pdq,C0206698,T191,Disorders What is the outlook for Bile Duct Cancer (Cholangiocarcinoma) ?,0000007_2-5,outlook,"Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - Whether the cancer is in the upper or lower part of the bile duct system. - The stage of the cancer (whether it affects only the bile ducts or has spread to the liver, lymph nodes, or other places in the body). - Whether the cancer has spread to nearby nerves or veins. - Whether the cancer can be completely removed by surgery. - Whether the patient has other conditions, such as primary sclerosing cholangitis. - Whether the level of CA 19-9 is higher than normal. - Whether the cancer has just been diagnosed or has recurred (come back). Treatment options may also depend on the symptoms caused by the cancer. Bile duct cancer is usually found after it has spread and can rarely be completely removed by surgery. Palliative therapy may relieve symptoms and improve the patient's quality of life.",Bile Duct Cancer (Cholangiocarcinoma),0000007_2,CancerGov,https://www.cancer.gov/types/liver/patient/bile-duct-treatment-pdq,C0206698,T191,Disorders what research (or clinical trials) is being done for Bile Duct Cancer (Cholangiocarcinoma) ?,0000007_2-6,research,"New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Liver transplant In a liver transplant, the entire liver is removed and replaced with a healthy donated liver. A liver transplant may be done in patients with perihilar bile duct cancer. If the patient has to wait for a donated liver, other treatment is given as needed. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Bile Duct Cancer (Cholangiocarcinoma),0000007_2,CancerGov,https://www.cancer.gov/types/liver/patient/bile-duct-treatment-pdq,C0206698,T191,Disorders What are the stages of Bile Duct Cancer (Cholangiocarcinoma) ?,0000007_2-7,stages,"Key Points - The results of diagnostic and staging tests are used to find out if cancer cells have spread. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - Stages are used to describe the different types of bile duct cancer. - Intrahepatic bile duct cancer - Perihilar bile duct cancer - Distal extrahepatic bile duct cancer - The following groups are used to plan treatment: - Resectable (localized) bile duct cancer - Unresectable, metastatic, or recurrent bile duct cancer The results of diagnostic and staging tests are used to find out if cancer cells have spread. The process used to find out if cancer has spread to other parts of the body is called staging. For bile duct cancer, the information gathered from tests and procedures is used to plan treatment, including whether the tumor can be removed by surgery. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if bile duct cancer spreads to the liver, the cancer cells in the liver are actually bile duct cancer cells. The disease is metastatic bile duct cancer, not liver cancer. Stages are used to describe the different types of bile duct cancer. Intrahepatic bile duct cancer - Stage 0: Abnormal cells are found in the innermost layer of tissue lining the intrahepatic bile duct. These abnormal cells may become cancer and spread into nearby normal tissue. Stage 0 is also called carcinoma in situ. - Stage I: There is one tumor that has spread into the intrahepatic bile duct and it has not spread into any blood vessels. - Stage II: There is one tumor that has spread through the wall of the bile duct and into a blood vessel, or there are multiple tumors that may have spread into a blood vessel. - Stage III: The tumor has spread through the tissue that lines the abdominal wall or has spread to organs or tissues near the liver such as the duodenum, colon, and stomach. - Stage IV: Stage IV is divided into stage IVA and stage IVB. - Stage IVA: The cancer has spread along the outside of the intrahepatic bile ducts or the cancer has spread to nearby lymph nodes. - Stage IVB: The cancer has spread to organs in other parts of the body. Perihilar bile duct cancer - Stage 0: Abnormal cells are found in the innermost layer of tissue lining the perihilar bile duct. These abnormal cells may become cancer and spread into nearby normal tissue. Stage 0 is also called carcinoma in situ. - Stage I: Cancer has formed in the innermost layer of the wall of the perihilar bile duct and has spread into the muscle layer or fibrous tissue layer of the wall. - Stage II: Cancer has spread through the wall of the perihilar bile duct to nearby fatty tissue or to the liver. - Stage III: Stage III is divided into stage IIIA and stage IIIB. - Stage IIIA: Cancer has spread to branches on one side of the hepatic artery or of the portal vein. - Stage IIIB: Cancer has spread to nearby lymph nodes. Cancer may have spread into the wall of the perihilar bile duct or through the wall to nearby fatty tissue, the liver, or to branches on one side of the hepatic artery or of the portal vein. - Stage IV: Stage IV is divided into stage IVA and stage IVB. - Stage IVA: Cancer has spread to one or more of the following: - the main part of the portal vein and/or common hepatic artery; - the branches of the portal vein and/or common hepatic artery on both sides; - the right hepatic duct and the left branch of the hepatic artery or of the portal vein; - the left hepatic duct and the right branch of the hepatic artery or of the portal vein. Cancer may have spread to nearby lymph nodes. - Stage IVB: Cancer has spread to lymph nodes in more distant parts of the abdomen, or to organs in other parts of the body. Distal extrahepatic bile duct cancer - Stage 0: Abnormal cells are found in the innermost layer of tissue lining the distal extrahepatic bile duct. These abnormal cells may become cancer and spread into nearby normal tissue. Stage 0 is also called carcinoma in situ. - Stage I: Stage I is divided into stage IA and stage IB. - Stage IA: Cancer has formed and is found in the distal extrahepatic bile duct wall only. - Stage IB: Cancer has formed and has spread through the wall of the distal extrahepatic bile duct but has not spread to nearby organs. - Stage II: Stage II is divided into stage IIA and stage IIB. - Stage IIA: Cancer has spread from the distal extrahepatic bile duct to the gallbladder, pancreas, duodenum, or other nearby organs. - Stage IIB: Cancer has spread from the distal extrahepatic bile duct to nearby lymph nodes. Cancer may have spread through the wall of the duct or to nearby organs. - Stage III: Cancer has spread to the large vessels that carry blood to the organs in the abdomen. Cancer may have spread to nearby lymph nodes. - Stage IV: Cancer has spread to organs in distant parts of the body. The following groups are used to plan treatment: Resectable (localized) bile duct cancer The cancer is in an area, such as the lower part of the common bile duct or perihilar area, where it can be removed completely by surgery. Unresectable, metastatic, or recurrent bile duct cancer Unresectable cancer cannot be removed completely by surgery. Most patients with bile duct cancer cannot have their cancer completely removed by surgery. Metastasis is the spread of cancer from the primary site (place where it started) to other places in the body. Metastatic bile duct cancer may have spread to the liver, other parts of the abdominal cavity, or to distant parts of the body. Recurrent bile duct cancer is cancer that has recurred (come back) after it has been treated. The cancer may come back in the bile ducts, liver, or gallbladder. Less often, it may come back in distant parts of the body.",Bile Duct Cancer (Cholangiocarcinoma),0000007_2,CancerGov,https://www.cancer.gov/types/liver/patient/bile-duct-treatment-pdq,C0206698,T191,Disorders What are the treatments for Bile Duct Cancer (Cholangiocarcinoma) ?,0000007_2-8,treatment,"Key Points - There are different types of treatment for patients with bile duct cancer. - Three types of standard treatment are used: - Surgery - Radiation therapy - Chemotherapy - New types of treatment are being tested in clinical trials. - Liver transplant - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with bile duct cancer. Different types of treatments are available for patients with bile duct cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Three types of standard treatment are used: Surgery The following types of surgery are used to treat bile duct cancer: - Removal of the bile duct: A surgical procedure to remove part of the bile duct if the tumor is small and in the bile duct only. Lymph nodes are removed and tissue from the lymph nodes is viewed under a microscope to see if there is cancer. - Partial hepatectomy: A surgical procedure in which the part of the liver where cancer is found is removed. The part removed may be a wedge of tissue, an entire lobe, or a larger part of the liver, along with some normal tissue around it. - Whipple procedure: A surgical procedure in which the head of the pancreas, the gallbladder, part of the stomach, part of the small intestine, and the bile duct are removed. Enough of the pancreas is left to make digestive juices and insulin. Even if the doctor removes all the cancer that can be seen at the time of the operation, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. It is not yet known whether chemotherapy or radiation therapy given after surgery helps keep the cancer from coming back. The following types of palliative surgery may be done to relieve symptoms caused by a blocked bile duct and improve quality of life: - Biliary bypass: A surgical procedure in which the part of the bile duct before the blockage is connected with part of the bile duct that is past the blockage or to the small intestine. This allows bile to flow to the gallbladder or small intestine. - Stent placement: A surgical procedure in which a stent (a thin, flexible tube or metal tube) is placed in the bile duct to open it and allow bile to flow into the small intestine or through a catheter that goes to a collection bag outside of the body. - Percutaneous transhepatic biliary drainage: A procedure used to x-ray the liver and bile ducts. A thin needle is inserted through the skin below the ribs and into the liver. Dye is injected into the liver or bile ducts and an x-ray is taken. If the bile duct is blocked, a thin, flexible tube called a stent may be left in the liver to drain bile into the small intestine or a collection bag outside the body. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. External and internal radiation therapy are used to treat bile duct cancer. It is not yet known whether external radiation therapy helps in the treatment of resectable bile duct cancer. In unresectable, metastatic, or recurrent bile duct cancer, new ways to improve the effect of external radiation therapy on cancer cells are being studied: - Hyperthermia therapy: A treatment in which body tissue is exposed to high temperatures to make cancer cells more sensitive to the effects of radiation therapy and certain anticancer drugs. - Radiosensitizers: Drugs that make cancer cells more sensitive to radiation therapy. Combining radiation therapy with radiosensitizers may kill more cancer cells. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Systemic chemotherapy is used to treat unresectable, metastatic, or recurrent bile duct cancer. It is not yet known whether systemic chemotherapy helps in the treatment of resectable bile duct cancer. In unresectable, metastatic, or recurrent bile duct cancer, intra-arterial embolization is being studied. It is a procedure in which the blood supply to a tumor is blocked after anticancer drugs are given in blood vessels near the tumor. Sometimes, the anticancer drugs are attached to small beads that are injected into an artery that feeds the tumor. The beads block blood flow to the tumor as they release the drug. This allows a higher amount of drug to reach the tumor for a longer period of time, which may kill more cancer cells. New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Liver transplant In a liver transplant, the entire liver is removed and replaced with a healthy donated liver. A liver transplant may be done in patients with perihilar bile duct cancer. If the patient has to wait for a donated liver, other treatment is given as needed. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Bile Duct Cancer Intrahepatic Bile Duct Cancer Resectable Intrahepatic Bile Duct Cancer Treatment of resectable intrahepatic bile duct cancer may include: - Surgery to remove the cancer, which may include partial hepatectomy. Embolization may be done before surgery. - Surgery followed by chemotherapy and/or radiation therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I intrahepatic bile duct cancer and stage II intrahepatic bile duct cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Unresectable, Recurrent, or Metastatic Intrahepatic Bile Duct Cancer Treatment of unresectable, recurrent, or metastatic intrahepatic bile duct cancer may include the following: - Stent placement as palliative treatment to relieve symptoms and improve quality of life. - External or internal radiation therapy as palliative treatment to relieve symptoms and improve the quality of life. - Chemotherapy. - A clinical trial of external radiation therapy combined with hyperthermia therapy, radiosensitizer drugs, or chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III intrahepatic bile duct cancer, stage IV intrahepatic bile duct cancer and recurrent intrahepatic bile duct cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Perihilar Bile Duct Cancer Resectable Perihilar Bile Duct Cancer Treatment of resectable perihilar bile duct cancer may include the following: - Surgery to remove the cancer, which may include partial hepatectomy. - Stent placement or percutaneous transhepatic biliary drainage as palliative therapy, to relieve jaundice and other symptoms and improve the quality of life. - Surgery followed by radiation therapy and/or chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I perihilar bile duct cancer and stage II perihilar bile duct cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Unresectable, Recurrent, or Metastatic Perihilar Bile Duct Cancer Treatment of unresectable, recurrent, or metastatic perihilar bile duct cancer may include the following: - Stent placement or biliary bypass as palliative treatment to relieve symptoms and improve the quality of life. - External or internal radiation therapy as palliative treatment to relieve symptoms and improve the quality of life. - Chemotherapy. - A clinical trial of external radiation therapy combined with hyperthermia therapy, radiosensitizer drugs, or chemotherapy. - A clinical trial of chemotherapy and radiation therapy followed by a liver transplant. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III perihilar bile duct cancer, stage IV perihilar bile duct cancer and recurrent perihilar bile duct cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Distal Extrahepatic Bile Duct Cancer Resectable Distal Extrahepatic Bile Duct Cancer Treatment of resectable distal extrahepatic bile duct cancer may include the following: - Surgery to remove the cancer, which may include a Whipple procedure. - Stent placement or percutaneous transhepatic biliary drainage as palliative therapy, to relieve jaundice and other symptoms and improve the quality of life. - Surgery followed by radiation therapy and/or chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with localized extrahepatic bile duct cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Unresectable, Recurrent, or Metastatic Distal Extrahepatic Bile Duct Cancer Treatment of unresectable, recurrent, or metastatic distal extrahepatic bile duct cancer may include the following: - Stent placement or biliary bypass as palliative treatment to relieve symptoms and improve the quality of life. - External or internal radiation therapy as palliative treatment to relieve symptoms and improve the quality of life. - Chemotherapy. - A clinical trial of external radiation therapy combined with hyperthermia therapy, radiosensitizer drugs, or chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with unresectable extrahepatic bile duct cancer, recurrent extrahepatic bile duct cancer and metastatic extrahepatic bile duct cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Bile Duct Cancer (Cholangiocarcinoma),0000007_2,CancerGov,https://www.cancer.gov/types/liver/patient/bile-duct-treatment-pdq,C0206698,T191,Disorders What is (are) Childhood Liver Cancer ?,0000007_3-1,information,"Key Points - Childhood liver cancer is a disease in which malignant (cancer) cells form in the tissues of the liver. - There are different types of childhood liver cancer. - Certain diseases and disorders can increase the risk of childhood liver cancer. - Signs and symptoms of childhood liver cancer include a lump or pain in the abdomen. - Tests that examine the liver and the blood are used to detect (find) and diagnose childhood liver cancer and find out whether the cancer has spread. - Certain factors affect prognosis (chance of recovery) and treatment options. Childhood liver cancer is a disease in which malignant (cancer) cells form in the tissues of the liver. The liver is one of the largest organs in the body. It has four lobes and fills the upper right side of the abdomen inside the rib cage. Three of the many important functions of the liver are: - To filter harmful substances from the blood so they can be passed from the body in stools and urine. - To make bile to help digest fats from food. - To store glycogen (sugar), which the body uses for energy. Liver cancer is rare in children and adolescents. There are different types of childhood liver cancer. There are two main types of childhood liver cancer: - Hepatoblastoma: Hepatoblastoma is the most common type of childhood liver cancer. It usually affects children younger than 3 years of age. In hepatoblastoma, the histology (how the cancer cells look under a microscope) affects the way the cancer is treated. The histology for hepatoblastoma may be one of the following: - Pure fetal histology. - Small cell undifferentiated histology. - Non-pure fetal histology, non-small cell undifferentiated histology. - Hepatocellular carcinoma: Hepatocellular carcinoma usually affects older children and adolescents. It is more common in areas of Asia that have high rates of hepatitis infection than in the U.S. The treatment of two less common types of childhood liver cancer is also discussed in this summary: - Undifferentiated embryonal sarcoma of the liver: This type of liver cancer usually occurs in children between 5 and 10 years of age. It often spreads all through the liver and/or to the lungs. - Infantile choriocarcinoma of the liver is a very rare tumor that starts in the placenta and spreads to the fetus. The tumor is usually found during the first few months of life. Also, the mother of the child may be diagnosed with choriocarcinoma. Choriocarcinoma is a type of gestational trophoblastic disease and needs treatment. See the Gestational Trophoblastic Disease Treatment summary for information on the treatment of choriocarcinoma. This summary is about the treatment of primary liver cancer (cancer that begins in the liver). Treatment of metastatic liver cancer, which is cancer that begins in other parts of the body and spreads to the liver, is not discussed in this summary. Primary liver cancer can occur in both adults and children. However, treatment for children is different than treatment for adults. See the PDQ summary on Adult Primary Liver Cancer Treatment for more information on the treatment of adults.",Childhood Liver Cancer,0000007_3,CancerGov,https://www.cancer.gov/types/liver/patient/child-liver-treatment-pdq,C2945767,T191,Disorders Who is at risk for Childhood Liver Cancer? ?,0000007_3-2,susceptibility,"Certain diseases and disorders can increase the risk of childhood liver cancer. Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesnt mean that you will not get cancer. Talk with your childs doctor if you think your child may be at risk. Risk factors for hepatoblastoma include the following syndromes or conditions: - Aicardi syndrome. - Beckwith-Wiedemann syndrome. - Familial adenomatous polyposis (FAP). - Glycogen storage disease. - A very low weight at birth. - Simpson-Golabi-Behmel syndrome. - Certain genetic changes, such as Trisomy 18. Risk factors for hepatocellular carcinoma include the following syndromes or conditions: - Alagille syndrome. - Glycogen storage disease. - Hepatitis B virus infection that was passed from mother to child at birth. - Progressive familial intrahepatic disease. - Tyrosinemia. Some patients with tyrosinemia or progressive familial intrahepatic disease will have a liver transplant before there are signs or symptoms of cancer.",Childhood Liver Cancer,0000007_3,CancerGov,https://www.cancer.gov/types/liver/patient/child-liver-treatment-pdq,C2945767,T191,Disorders What are the symptoms of Childhood Liver Cancer ?,0000007_3-3,symptoms,Signs and symptoms of childhood liver cancer include a lump or pain in the abdomen. Signs and symptoms are more common after the tumor gets big. Other conditions can cause the same signs and symptoms. Check with your childs doctor if your child has any of the following: - A lump in the abdomen that may be painful. - Swelling in the abdomen. - Weight loss for no known reason. - Loss of appetite. - Nausea and vomiting.,Childhood Liver Cancer,0000007_3,CancerGov,https://www.cancer.gov/types/liver/patient/child-liver-treatment-pdq,C2945767,T191,Disorders How to diagnose Childhood Liver Cancer ?,0000007_3-4,exams and tests,"Tests that examine the liver and the blood are used to detect (find) and diagnose childhood liver cancer and find out whether the cancer has spread. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient's health habits and past illnesses and treatments will also be taken. - Serum tumor marker test : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs, tissues, or tumor cells in the body. Certain substances are linked to specific types of cancer when found in increased levels in the blood. These are called tumor markers. The blood of children who have liver cancer may have increased amounts of a hormone called beta-human chorionic gonadotropin (-hCG) or a protein called alpha-fetoprotein (AFP). Other cancers and certain noncancer conditions, including cirrhosis and hepatitis, can also increase AFP levels. - Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following: - The number of red blood cells, white blood cells, and platelets. - The amount of hemoglobin (the protein that carries oxygen) in the red blood cells. - The portion of the blood sample made up of red blood cells. - Liver function tests : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by the liver. A higher than normal amount of a substance can be a sign of liver damage or cancer. - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances, such as bilirubin or lactate dehydrogenase (LDH), released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. - Epstein-Barr virus (EBV) test: A blood test to check for antibodies to the EBV and DNA markers of the EBV. These are found in the blood of patients who have been infected with EBV. - Hepatitis assay : A procedure in which a blood sample is checked for pieces of the hepatitis virus. - MRI (magnetic resonance imaging) with gadolinium : A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the liver. A substance called gadolinium is injected into a vein. The gadolinium collects around the cancer cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later. In childhood liver cancer, an ultrasound exam of the abdomen to check the large blood vessels is usually done. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. In childhood liver cancer, a CT scan of the chest and abdomen is usually done. - Abdominal x-ray : An x-ray of the organs in the abdomen. An x-ray is a type of energy beam that can go through the body onto film, making a picture of areas inside the body. - Biopsy : The removal of a sample of cells or tissues so it can be viewed under a microscope to check for signs of cancer. The sample may be taken during surgery to remove or view the tumor. A pathologist looks at the sample under a microscope to find out the type of liver cancer. The following test may be done on the sample of tissue that is removed: - Immunohistochemistry : A test that uses antibodies to check for certain antigens in a sample of tissue. The antibody is usually linked to a radioactive substance or a dye that causes the tissue to light up under a microscope. This type of test is used to check for a certain gene mutation and to tell the difference between different types of cancer.",Childhood Liver Cancer,0000007_3,CancerGov,https://www.cancer.gov/types/liver/patient/child-liver-treatment-pdq,C2945767,T191,Disorders What is the outlook for Childhood Liver Cancer ?,0000007_3-5,outlook,"Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options for hepatoblastoma depend on the following: - The PRETEXT or POSTTEXT group. - Whether the cancer has spread to other places in the body, such as the lungs or certain large blood vessels. - Whether the cancer can be removed completely by surgery. - How the cancer responds to chemotherapy. - How the cancer cells look under a microscope. - Whether the AFP blood levels go down after treatment. - Whether the cancer has just been diagnosed or has recurred. - Age of the child. The prognosis (chance of recovery) and treatment options for hepatocellular carcinoma depend on the following: - The PRETEXT or POSTTEXT group. - Whether the cancer has spread to other places in the body, such as the lungs. - Whether the cancer can be removed completely by surgery. - How the cancer responds to chemotherapy. - How the cancer cells look under a microscope. - Whether the child has hepatitis B infection. - Whether the cancer has just been diagnosed or has recurred. For childhood liver cancer that recurs (comes back) after initial treatment, the prognosis and treatment options depend on: - Where in the body the tumor recurred. - The type of treatment used to treat the initial cancer. Childhood liver cancer may be cured if the tumor is small and can be completely removed by surgery. Complete removal is possible more often for hepatoblastoma than for hepatocellular carcinoma.",Childhood Liver Cancer,0000007_3,CancerGov,https://www.cancer.gov/types/liver/patient/child-liver-treatment-pdq,C2945767,T191,Disorders What are the stages of Childhood Liver Cancer ?,0000007_3-6,stages,"Key Points - After childhood liver cancer has been diagnosed, tests are done to find out if cancer cells have spread within the liver or to other parts of the body. - There are two grouping systems for childhood liver cancer. - There are four PRETEXT and POSTTEXT groups: - PRETEXT and POSTTEXT Group I - PRETEXT and POSTTEXT Group II - PRETEXT and POSTTEXT Group III - PRETEXT and POSTTEXT Group IV - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. After childhood liver cancer has been diagnosed, tests are done to find out if cancer cells have spread within the liver or to other parts of the body. The process used to find out if cancer has spread within the liver, to nearby tissues or organs, or to other parts of the body is called staging. In childhood liver cancer, the PRETEXT and POSTTEXT groups are used instead of stage to plan treatment. The results of the tests and procedures done to detect, diagnose, and find out whether the cancer has spread are used to determine the PRETEXT and POSTTEXT groups. There are two grouping systems for childhood liver cancer. Two grouping systems are used for childhood liver cancer: - The PRETEXT group describes the tumor before the patient has treatment. - The POSTTEXT group describes the tumor after the patient has treatment. There are four PRETEXT and POSTTEXT groups: The liver is divided into 4 sections. The PRETEXT and POSTTEXT groups depend on which sections of the liver have cancer. PRETEXT and POSTTEXT Group I In group I, the cancer is found in one section of the liver. Three sections of the liver that are next to each other do not have cancer in them. PRETEXT and POSTTEXT Group II In group II, cancer is found in one or two sections of the liver. Two sections of the liver that are next to each other do not have cancer in them. PRETEXT and POSTTEXT Group III In group III, one of the following is true: - Cancer is found in three sections of the liver and one section does not have cancer. - Cancer is found in two sections of the liver and two sections that are not next to each other do not have cancer in them. PRETEXT and POSTTEXT Group IV In group IV, cancer is found in all four sections of the liver. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if childhood liver cancer spreads to the lung, the cancer cells in the lung are actually liver cancer cells. The disease is metastatic liver cancer, not lung cancer.",Childhood Liver Cancer,0000007_3,CancerGov,https://www.cancer.gov/types/liver/patient/child-liver-treatment-pdq,C2945767,T191,Disorders What are the treatments for Childhood Liver Cancer ?,0000007_3-7,treatment,"Key Points - There are different types of treatment for patients with childhood liver cancer. - Children with liver cancer should have their treatment planned by a team of healthcare providers who are experts in treating this rare childhood cancer. - Some cancer treatments cause side effects months or years after treatment has ended. - Six types of standard treatment are used: - Surgery - Watchful waiting - Chemotherapy - Radiation therapy - Ablation therapy - Antiviral treatment - New types of treatment are being tested in clinical trials. - Targeted therapy - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with childhood liver cancer. Different types of treatments are available for children with liver cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Taking part in a clinical trial should be considered for all children with liver cancer. Some clinical trials are open only to patients who have not started treatment. Children with liver cancer should have their treatment planned by a team of healthcare providers who are experts in treating this rare childhood cancer. Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other healthcare providers who are experts in treating children with liver cancer and who specialize in certain areas of medicine. It is especially important to have a pediatric surgeon with experience in liver surgery who can send patients to a liver transplant program if needed. Other specialists may include the following: - Pediatrician. - Radiation oncologist. - Pediatric nurse specialist. - Rehabilitation specialist. - Psychologist. - Social worker. Some cancer treatments cause side effects months or years after treatment has ended. Side effects from cancer treatment that begin during or after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include: - Physical problems. - Changes in mood, feelings, thinking, learning, or memory. - Second cancers (new types of cancer). Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information). Six types of standard treatment are used: Surgery When possible, the cancer is removed by surgery. - Partial hepatectomy: Removal of the part of the liver where cancer is found. The part removed may be a wedge of tissue, an entire lobe, or a larger part of the liver, along with a small amount of normal tissue around it. - Total hepatectomy and liver transplant: Removal of the entire liver followed by a transplant of a healthy liver from a donor. A liver transplant may be possible when cancer has not spread beyond the liver and a donated liver can be found. If the patient has to wait for a donated liver, other treatment is given as needed. - Resection of metastases: Surgery to remove cancer that has spread outside of the liver, such as to nearby tissues, the lungs, or the brain. Factors that affect the type of surgery used include the following: - The PRETEXT group and POSTTEXT group. - The size of the primary tumor. - Whether there is more than one tumor in the liver. - Whether the cancer has spread to nearby large blood vessels. - The level of alpha-fetoprotein (AFP) in the blood. - Whether the tumor can be shrunk by chemotherapy so that it can be removed by surgery. - Whether a liver transplant is needed. Chemotherapy is sometimes given before surgery, to shrink the tumor and make it easier to remove. This is called neoadjuvant therapy. Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Watchful waiting Watchful waiting is closely monitoring a patients condition without giving any treatment until signs or symptoms appear or change. In hepatoblastoma, this treatment is only used for small tumors that have been completely removed by surgery. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Treatment using more than one anticancer drug is called combination chemotherapy. Chemoembolization of the hepatic artery (the main artery that supplies blood to the liver) is a type of regional chemotherapy used to treat childhood liver cancer. The anticancer drug is injected into the hepatic artery through a catheter (thin tube). The drug is mixed with a substance that blocks the artery, cutting off blood flow to the tumor. Most of the anticancer drug is trapped near the tumor and only a small amount of the drug reaches other parts of the body. The blockage may be temporary or permanent, depending on the substance used to block the artery. The tumor is prevented from getting the oxygen and nutrients it needs to grow. The liver continues to receive blood from the hepatic portal vein, which carries blood from the stomach and intestine. This procedure is also called transarterial chemoembolization or TACE. The way the chemotherapy is given depends on the type of the cancer being treated and the PRETEXT or POSTTEXT group. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type of the cancer being treated and the PRETEXT or POSTTEXT group. Radioembolization of the hepatic artery (the main artery that supplies blood to the liver) is a type of internal radiation therapy used to treat hepatocellular carcinoma. A very small amount of a radioactive substance is attached to tiny beads that are injected into the hepatic artery through a catheter (thin tube). The beads are mixed with a substance that blocks the artery, cutting off blood flow to the tumor. Most of the radiation is trapped near the tumor to kill the cancer cells. This is done to relieve symptoms and improve quality of life for children with hepatocellular carcinoma. External radiation therapy is used to treat hepatoblastoma that cannot be removed by surgery or has spread to other parts of the body. Ablation therapy Ablation therapy removes or destroys tissue. Different types of ablation therapy are used for liver cancer: - Radiofrequency ablation: The use of special needles that are inserted directly through the skin or through an incision in the abdomen to reach the tumor. High-energy radio waves heat the needles and tumor which kills cancer cells. Radiofrequency ablation is being used to treat recurrent hepatoblastoma. - Percutaneous ethanol injection: A small needle is used to inject ethanol (pure alcohol) directly into a tumor to kill cancer cells. Several treatments may be needed. Percutaneous ethanol injection is being used to treat recurrent hepatoblastoma. Antiviral treatment Hepatocellular carcinoma that is linked to the hepatitis B virus may be treated with antiviral drugs. New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. Targeted therapy is being studied for the treatment of undifferentiated embryonal sarcoma of the liver and liver cancer that has come back. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the treatment group may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Childhood Liver Cancer Hepatoblastoma Treatment options for hepatoblastoma that can be removed by surgery at the time of diagnosis may include the following: - Surgery to remove the tumor, followed by watchful waiting or chemotherapy, for hepatoblastoma with pure fetal histology. - Surgery to remove the tumor, with combination chemotherapy given either before surgery, after surgery, or both, for hepatoblastoma that is not pure fetal histology. For hepatoblastoma with small cell undifferentiated histology, aggressive chemotherapy is given. Treatment options for hepatoblastoma that cannot be removed by surgery or is not removed at the time of diagnosis may include the following: - Combination chemotherapy to shrink the tumor, followed by surgery to remove the tumor. - Combination chemotherapy followed by a liver transplant. - Chemoembolization of the hepatic artery to shrink the tumor, followed by surgery to remove the tumor. For hepatoblastoma that has spread to other parts of the body at the time of diagnosis, combination chemotherapy is given to shrink the cancer in the liver and cancer that has spread to other parts of the body. After chemotherapy, imaging tests are done to check whether the cancer can be removed by surgery. Treatment options may include the following: - If the cancer in the liver and other parts of the body can be removed, surgery will be done to remove the tumors followed by chemotherapy to kill any cancer cells that may remain. - If the cancer in the liver cannot be removed by surgery but there are no signs of cancer in other parts of the body, the treatment may be a liver transplant. - If the cancer in other parts of the body cannot be removed or a liver transplant is not possible, chemotherapy, chemoembolization of the hepatic artery, or radiation therapy may be given. Treatment options in clinical trials for newly diagnosed hepatoblastoma include: - A clinical trial of new treatment regimens based on how likely it is the cancer will recur after initial treatment. Hepatocellular Carcinoma Treatment options for hepatocellular carcinoma that can be removed by surgery at the time of diagnosis may include the following: - Surgery alone to remove the tumor. - Surgery to remove the tumor, followed by chemotherapy. - Combination chemotherapy followed by surgery to remove the tumor. Treatment options for hepatocellular carcinoma that cannot be removed by surgery at the time of diagnosis may include the following: - Chemotherapy to shrink the tumor, followed by surgery to completely remove the tumor. - Chemotherapy to shrink the tumor. If surgery to completely remove the tumor is not possible, further treatment may include the following: - Liver transplant. - Chemoembolization of the hepatic artery to shrink the tumor, followed by surgery to remove as much of the tumor as possible or liver transplant. - Chemoembolization of the hepatic artery alone. - Radioembolization of the hepatic artery as palliative therapy to relieve symptoms and improve the quality of life. Treatment for hepatocellular carcinoma that has spread to other parts of the body at the time of diagnosis may include: - Combination chemotherapy to shrink the tumor, followed by surgery to remove as much of the tumor as possible from the liver and other places where cancer has spread. Studies have not shown that this treatment works well but some patients may have some benefit. Treatment options for hepatocellular carcinoma related to hepatitis B virus (HBV) infection include: - Surgery to remove the tumor. - Antiviral drugs that treat infection caused by the hepatitis B virus. Undifferentiated Embryonal Sarcoma of the Liver Treatment options for undifferentiated embryonal sarcoma of the liver (UESL) may include the following: - Combination chemotherapy to shrink the tumor, followed by surgery to remove as much of the tumor as possible. Chemotherapy may also be given after surgery to remove the tumor. - Surgery to remove the tumor followed by chemotherapy. A second surgery may be done to remove tumor that remains, followed by more chemotherapy. - Liver transplant if surgery to remove the tumor is not possible. - A clinical trial of a combination of targeted therapy, chemotherapy and/or radiation therapy before surgery. Infantile Choriocarcinoma of the Liver Treatment options for choriocarcinoma of the liver in infants may include the following: - Combination chemotherapy to shrink the tumor, followed by surgery to remove the tumor. - Surgery to remove the tumor. Recurrent Childhood Liver Cancer Treatment of recurrent hepatoblastoma may include the following: - Surgery to remove isolated (single and separate) metastatic tumors with or without chemotherapy. - Combination chemotherapy. - Liver transplant. - Ablation therapy (radiofrequency ablation or percutaneous ethanol injection). - A clinical trial of a new treatment. Treatment of progressive or recurrent hepatocellular carcinoma may include the following: - Chemoembolization of the hepatic artery to shrink the tumor before liver transplant. - Liver transplant. - A clinical trial of a new treatment. Treatment Options in Clinical Trials Check the list of NCI-supported cancer clinical trials that are now accepting patients with childhood liver cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website.",Childhood Liver Cancer,0000007_3,CancerGov,https://www.cancer.gov/types/liver/patient/child-liver-treatment-pdq,C2945767,T191,Disorders what research (or clinical trials) is being done for Childhood Liver Cancer ?,0000007_3-8,research,"New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. Targeted therapy is being studied for the treatment of undifferentiated embryonal sarcoma of the liver and liver cancer that has come back. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Treatment Options in Clinical Trials Check the list of NCI-supported cancer clinical trials that are now accepting patients with childhood liver cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website.",Childhood Liver Cancer,0000007_3,CancerGov,https://www.cancer.gov/types/liver/patient/child-liver-treatment-pdq,C2945767,T191,Disorders What is (are) Liver (Hepatocellular) Cancer ?,0000007_4-1,information,"Key Points - Liver cancer is a disease in which malignant (cancer) cells form in the tissues of the liver. - Liver cancer is not common in the United States. - Being infected with certain types of the hepatitis virus can cause hepatitis and increase the risk of liver cancer. - Hepatitis A - Hepatitis B - Hepatitis C - Hepatitis D - Hepatitis E - Hepatitis G Liver cancer is a disease in which malignant (cancer) cells form in the tissues of the liver. The liver is one of the largest organs in the body. It has four lobes and fills the upper right side of the abdomen inside the rib cage. Three of the many important functions of the liver are: - To filter harmful substances from the blood so they can be passed from the body in stools and urine. - To make bile to help digest fats from food. - To store glycogen (sugar), which the body uses for energy. See the following PDQ summaries for more information about liver (hepatocellular) cancer: - Liver (Hepatocellular) Cancer Screening - Adult Primary Liver Cancer Treatment - Childhood Liver Cancer Treatment Liver cancer is not common in the United States. Liver cancer is the fourth most common cancer and the third leading cause of cancer death in the world. In the United States, men, especially Asian/Pacific Islander men, have an increased risk of liver cancer. The number of new cases of liver cancer and the number of deaths from liver cancer continue to increase, especially among middle-aged black, Hispanic, and white men. People are usually older than 40 years when they develop this cancer. Finding and treating liver cancer early may prevent death from liver cancer.",Liver (Hepatocellular) Cancer,0000007_4,CancerGov,https://www.cancer.gov/types/liver/patient/liver-prevention-pdq,C0006826,T191,Disorders Who is at risk for Liver (Hepatocellular) Cancer? ?,0000007_4-2,susceptibility,"Being infected with certain types of the hepatitis virus can cause hepatitis and increase the risk of liver cancer. Hepatitis is most commonly caused by the hepatitis virus. Hepatitis is a disease that causes inflammation (swelling) of the liver. Damage to the liver from hepatitis that lasts a long time can increase the risk of liver cancer. There are six types of the hepatitis virus. Hepatitis A (HAV), hepatitis B (HBV), and hepatitis C (HCV) are the three most common types. These three viruses cause similar symptoms, but the ways they spread and affect the liver are different. The Hepatitis A vaccine and the hepatitis B vaccine prevent infection with hepatitis A and hepatitis B. There is no vaccine to prevent infection with hepatitis C. If a person has had one type of hepatitis in the past, it is still possible to get the other types. Hepatitis viruses include: Hepatitis A Hepatitis A is caused by eating food or drinking water infected with hepatitis A virus. It does not lead to chronic disease. People with hepatitis A usually get better without treatment. Hepatitis B Hepatitis B is caused by contact with the blood, semen, or other body fluid of a person infected with hepatitis B virus. It is a serious infection that may become chronic and cause scarring of the liver (cirrhosis). This may lead to liver cancer. Blood banks test all donated blood for hepatitis B, which greatly lowers the risk of getting the virus from blood transfusions. Hepatitis C Hepatitis C is caused by contact with the blood of a person infected with hepatitis C virus. Hepatitis C may range from a mild illness that lasts a few weeks to a serious, lifelong illness. Most people who have hepatitis C develop a chronic infection that may cause scarring of the liver (cirrhosis). This may lead to liver cancer. Blood banks test all donated blood for hepatitis C, which greatly lowers the risk of getting the virus from blood transfusions. Hepatitis D Hepatitis D develops in people already infected with hepatitis B. It is caused by hepatitis D virus (HDV) and is spread through contact with infected blood or dirty needles, or by having unprotected sex with a person infected with HDV. Hepatitis D causes acute hepatitis. Hepatitis E Hepatitis E is caused by hepatitis E virus (HEV). Hepatitis E can be spread through oral- anal contact or by drinking infected water. Hepatitis E is rare in the United States. Hepatitis G Being infected with hepatitis G virus (HGV) has not been shown to cause liver cancer. Key Points - Avoiding risk factors and increasing protective factors may help prevent cancer. - The following risk factors may increase the risk of liver cancer: - Hepatitis B and C - Cirrhosis - Aflatoxin - The following protective factor may decrease the risk of liver cancer: - Hepatitis B vaccine - Cancer prevention clinical trials are used to study ways to prevent cancer. - New ways to prevent liver cancer are being studied in clinical trials. Avoiding risk factors and increasing protective factors may help prevent cancer. Avoiding cancer risk factors may help prevent certain cancers. Risk factors include smoking, being overweight, and not getting enough exercise. Increasing protective factors such as quitting smoking and exercising may also help prevent some cancers. Talk to your doctor or other health care professional about how you might lower your risk of cancer. The following risk factors may increase the risk of liver cancer: Hepatitis B and C Having chronic hepatitis B or chronic hepatitis C increases the risk of developing liver cancer. The risk is even greater for people with both hepatitis B and C. Also, the longer the hepatitis infection lasts (especially hepatitis C), the greater the risk. In a study of patients with chronic hepatitis C, those who were treated to lower their iron levels by having blood drawn and eating a low-iron diet were less likely to develop liver cancer than those who did not have this treatment. Cirrhosis The risk of developing liver cancer is increased for people who have cirrhosis, a disease in which healthy liver tissue is replaced by scar tissue. The scar tissue blocks the flow of blood through the liver and keeps it from working as it should. Chronic alcoholism and chronic hepatitis C are the most common causes of cirrhosis. Aflatoxin The risk of developing liver cancer may be increased by eating foods that contain aflatoxin (poison from a fungus that can grow on foods, such as grains and nuts, that have not been stored properly).",Liver (Hepatocellular) Cancer,0000007_4,CancerGov,https://www.cancer.gov/types/liver/patient/liver-prevention-pdq,C0006826,T191,Disorders How to prevent Liver (Hepatocellular) Cancer ?,0000007_4-3,prevention,"Key Points - Avoiding risk factors and increasing protective factors may help prevent cancer. - The following risk factors may increase the risk of liver cancer: - Hepatitis B and C - Cirrhosis - Aflatoxin - The following protective factor may decrease the risk of liver cancer: - Hepatitis B vaccine - Cancer prevention clinical trials are used to study ways to prevent cancer. - New ways to prevent liver cancer are being studied in clinical trials. Avoiding risk factors and increasing protective factors may help prevent cancer. Avoiding cancer risk factors may help prevent certain cancers. Risk factors include smoking, being overweight, and not getting enough exercise. Increasing protective factors such as quitting smoking and exercising may also help prevent some cancers. Talk to your doctor or other health care professional about how you might lower your risk of cancer. The following risk factors may increase the risk of liver cancer: Hepatitis B and C Having chronic hepatitis B or chronic hepatitis C increases the risk of developing liver cancer. The risk is even greater for people with both hepatitis B and C. Also, the longer the hepatitis infection lasts (especially hepatitis C), the greater the risk. In a study of patients with chronic hepatitis C, those who were treated to lower their iron levels by having blood drawn and eating a low-iron diet were less likely to develop liver cancer than those who did not have this treatment. Cirrhosis The risk of developing liver cancer is increased for people who have cirrhosis, a disease in which healthy liver tissue is replaced by scar tissue. The scar tissue blocks the flow of blood through the liver and keeps it from working as it should. Chronic alcoholism and chronic hepatitis C are the most common causes of cirrhosis. Aflatoxin The risk of developing liver cancer may be increased by eating foods that contain aflatoxin (poison from a fungus that can grow on foods, such as grains and nuts, that have not been stored properly). The following protective factor may decrease the risk of liver cancer: Hepatitis B vaccine Preventing hepatitis B infection (by being vaccinated for hepatitis B) has been shown to lower the risk of liver cancer in children. It is not yet known if it lowers the risk in adults. Cancer prevention clinical trials are used to study ways to prevent cancer. Cancer prevention clinical trials are used to study ways to lower the risk of developing certain types of cancer. Some cancer prevention trials are conducted with healthy people who have not had cancer but who have an increased risk for cancer. Other prevention trials are conducted with people who have had cancer and are trying to prevent another cancer of the same type or to lower their chance of developing a new type of cancer. Other trials are done with healthy volunteers who are not known to have any risk factors for cancer. The purpose of some cancer prevention clinical trials is to find out whether actions people take can prevent cancer. These may include eating fruits and vegetables, exercising, quitting smoking, or taking certain medicines, vitamins, minerals, or food supplements. New ways to prevent liver cancer are being studied in clinical trials. Clinical trials are taking place in many parts of the country. Information about clinical trials can be found in the Clinical Trials section of the NCI website. Check NCI's list of cancer clinical trials for liver cancer prevention trials that are now accepting patients.",Liver (Hepatocellular) Cancer,0000007_4,CancerGov,https://www.cancer.gov/types/liver/patient/liver-prevention-pdq,C0006826,T191,Disorders what research (or clinical trials) is being done for Liver (Hepatocellular) Cancer ?,0000007_4-4,research,"Cancer prevention clinical trials are used to study ways to prevent cancer. Cancer prevention clinical trials are used to study ways to lower the risk of developing certain types of cancer. Some cancer prevention trials are conducted with healthy people who have not had cancer but who have an increased risk for cancer. Other prevention trials are conducted with people who have had cancer and are trying to prevent another cancer of the same type or to lower their chance of developing a new type of cancer. Other trials are done with healthy volunteers who are not known to have any risk factors for cancer. The purpose of some cancer prevention clinical trials is to find out whether actions people take can prevent cancer. These may include eating fruits and vegetables, exercising, quitting smoking, or taking certain medicines, vitamins, minerals, or food supplements. New ways to prevent liver cancer are being studied in clinical trials. Clinical trials are taking place in many parts of the country. Information about clinical trials can be found in the Clinical Trials section of the NCI website. Check NCI's list of cancer clinical trials for liver cancer prevention trials that are now accepting patients.",Liver (Hepatocellular) Cancer,0000007_4,CancerGov,https://www.cancer.gov/types/liver/patient/liver-prevention-pdq,C0006826,T191,Disorders What is (are) Liver (Hepatocellular) Cancer ?,0000007_5-1,information,"Key Points - Liver cancer is a disease in which malignant (cancer) cells form in the tissues of the liver. - Liver cancer is less common in the United States than in other parts of the world. - Having hepatitis or cirrhosis can increase the risk of developing liver cancer. Liver cancer is a disease in which malignant (cancer) cells form in the tissues of the liver. The liver is one of the largest organs in the body. It has four lobes and fills the upper right side of the abdomen inside the rib cage. Three of the many important functions of the liver are: - To filter harmful substances from the blood so they can be passed from the body in stools and urine. - To make bile to help digest fats from food. - To store glycogen (sugar), which the body uses for energy. See the following PDQ summaries for more information about liver (hepatocellular) cancer: - Liver (Hepatocellular) Cancer Prevention - Adult Primary Liver Cancer Treatment - Childhood Liver Cancer Treatment Liver cancer is less common in the United States than in other parts of the world. Liver cancer is uncommon in the United States, but is the fourth most common cancer in the world. In the United States, men, especially Chinese American men, have a greater risk of developing liver cancer.",Liver (Hepatocellular) Cancer,0000007_5,CancerGov,https://www.cancer.gov/types/liver/patient/liver-screening-pdq,C0006826,T191,Disorders Who is at risk for Liver (Hepatocellular) Cancer? ?,0000007_5-2,susceptibility,"Having hepatitis or cirrhosis can increase the risk of developing liver cancer. Anything that increases the chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. People who think they may be at risk should discuss this with their doctor. Risk factors for liver cancer include: - Having hepatitis B or hepatitis C; having both hepatitis B and hepatitis C increases the risk even more. - Having cirrhosis, which can be caused by: - hepatitis (especially hepatitis C); or - drinking large amounts of alcohol for many years or being an alcoholic. - Eating foods tainted with aflatoxin (poison from a fungus that can grow on foods, such as grains and nuts, that have not been stored properly).",Liver (Hepatocellular) Cancer,0000007_5,CancerGov,https://www.cancer.gov/types/liver/patient/liver-screening-pdq,C0006826,T191,Disorders What is (are) Ewing Sarcoma ?,0000009_1-1,information,"Key Points - Ewing sarcoma is a type of tumor that forms in bone or soft tissue. - Signs and symptoms of Ewing sarcoma include swelling and pain near the tumor. - Tests that examine the bone and soft tissue are used to diagnose and stage Ewing sarcoma. - A biopsy is done to diagnose Ewing sarcoma. - Certain factors affect prognosis (chance of recovery). Ewing sarcoma is a type of tumor that forms in bone or soft tissue. Ewing sarcoma is a type of tumor that forms from a certain kind of cell in bone or soft tissue. Ewing sarcoma may be found in the bones of the legs, arms, feet, hands, chest, pelvis, spine, or skull. Ewing sarcoma also may be found in the soft tissue of the trunk, arms, legs, head and neck, abdominal cavity, or other areas. Ewing sarcoma is most common in adolescents and young adults. Ewing sarcoma has also been called peripheral primitive neuroectodermal tumor, Askin tumor (Ewing sarcoma of the chest wall), extraosseous Ewing sarcoma (Ewing sarcoma in tissue other than bone), and Ewing sarcoma family of tumors.",Ewing Sarcoma,0000009_1,CancerGov,https://www.cancer.gov/types/bone/patient/ewing-treatment-pdq,C1261473,T191,Disorders How to diagnose Ewing Sarcoma ?,0000009_1-2,exams and tests,"Tests that examine the bone and soft tissue are used to diagnose and stage Ewing sarcoma. Procedures that make pictures of the bones and soft tissues and nearby areas help diagnose Ewing sarcoma and show how far the cancer has spread. The process used to find out if cancer cells have spread within and around the bones and soft tissues is called staging. In order to plan treatment, it is important to know if the cancer is in the area where it first formed or if it has spread to other parts of the body. Tests and procedures to detect, diagnose, and stage Ewing sarcoma are usually done at the same time. The following tests and procedures may be used to diagnose or stage Ewing sarcoma: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body, such as the area where the tumor formed. This procedure is also called nuclear magnetic resonance imaging (NMRI). - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the area where the tumor formed or the chest, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. A PET scan and a CT scan are often done at the same time. If there is any cancer, this increases the chance that it will be found. - Bone scan : A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones with cancer and is detected by a scanner. - Bone marrow aspiration and biopsy : The removal of bone marrow and a small piece of bone by inserting a hollow needle into the hipbone. Samples are removed from both hipbones. A pathologist views the bone marrow and bone under a microscope to see if the cancer has spread. - X-ray: An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body, such as the chest or the area where the tumor formed. - Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following: - The number of red blood cells, white blood cells, and platelets. - The amount of hemoglobin (the protein that carries oxygen) in the red blood cells. - The portion of the blood sample made up of red blood cells. - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances, such as lactate dehydrogenase (LDH), released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. A biopsy is done to diagnose Ewing sarcoma. Tissue samples are removed during a biopsy so they can be viewed under a microscope by a pathologist to check for signs of cancer. It is helpful if the biopsy is done at the same center where treatment will be given. - Needle biopsy : For a needle biopsy, tissue is removed using a needle. This type of needle biopsy may be done if its possible to remove tissue samples large enough to be used for testing. - Incisional biopsy : For an incisional biopsy, a sample of tissue is removed through an incision in the skin. - Excisional biopsy : The removal of an entire lump or area of tissue that doesnt look normal. The specialists (pathologist, radiation oncologist, and surgeon) who will treat the patient usually work together to decide where the needle should be placed or the biopsy incision should be made. This is done so that the biopsy doesn't affect later treatment such as surgery to remove the tumor or radiation therapy. If there is a chance that the cancer has spread to nearby lymph nodes, one or more lymph nodes may be removed and checked for signs of cancer. The following tests may be done on the tissue that is removed: - Cytogenetic analysis : A laboratory test in which cells in a sample of tissue are viewed under a microscope to look for certain changes in the chromosomes. - Immunohistochemistry : A test that uses antibodies to check for certain antigens in a sample of tissue. The antibody is usually linked to a radioactive substance or a dye that causes the tissue to light up under a microscope. This type of test may be used to tell the difference between different types of cancer. - Flow cytometry : A laboratory test that measures the number of cells in a sample, the percentage of live cells in a sample, and certain characteristics of cells, such as size, shape, and the presence of tumor markers on the cell surface. The cells are stained with a light-sensitive dye, placed in a fluid, and passed in a stream before a laser or other type of light. The measurements are based on how the light-sensitive dye reacts to the light.",Ewing Sarcoma,0000009_1,CancerGov,https://www.cancer.gov/types/bone/patient/ewing-treatment-pdq,C1261473,T191,Disorders What are the symptoms of Ewing Sarcoma ?,0000009_1-3,symptoms,"Signs and symptoms of Ewing sarcoma include swelling and pain near the tumor. These and other signs and symptoms may be caused by Ewing sarcoma or by other conditions. Check with your childs doctor if your child has any of the following: - Pain and/or swelling, usually in the arms, legs, chest, back, or pelvis. - A lump (which may feel soft and warm) in the arms, legs, chest, or pelvis. - Fever for no known reason. - A bone that breaks for no known reason.",Ewing Sarcoma,0000009_1,CancerGov,https://www.cancer.gov/types/bone/patient/ewing-treatment-pdq,C1261473,T191,Disorders What is the outlook for Ewing Sarcoma ?,0000009_1-4,outlook,"Certain factors affect prognosis (chance of recovery). The factors that affect prognosis (chance of recovery) are different before and after treatment. Before treatment, prognosis depends on: - Whether the tumor has spread to lymph nodes or distant parts of the body. - Where in the body the tumor started. - Whether the tumor formed in the bone or in soft tissue. - How large the tumor is at when the tumor is diagnosed. - Whether the LDH level in the blood is higher than normal. - Whether the tumor has certain gene changes. - Whether the child is younger than 15 years. - The patient's gender. - Whether the child has had treatment for a different cancer before Ewing sarcoma. - Whether the tumor has just been diagnosed or has recurred (come back). After treatment, prognosis is affected by: - Whether the tumor was completely removed by surgery. - Whether the tumor responds to chemotherapy or radiation therapy. If the cancer recurs after initial treatment, prognosis depends on: - Whether the cancer came back more than two years after the initial treatment. - Where in the body the tumor came back. - The type of initial treatment given.",Ewing Sarcoma,0000009_1,CancerGov,https://www.cancer.gov/types/bone/patient/ewing-treatment-pdq,C1261473,T191,Disorders What are the stages of Ewing Sarcoma ?,0000009_1-5,stages,"Key Points - The results of diagnostic and staging tests are used to find out if cancer cells have spread. - Ewing sarcoma is described based on whether the cancer has spread from the bone or soft tissue in which the cancer began. - Localized Ewing sarcoma - Metastatic Ewing sarcoma - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. The results of diagnostic and staging tests are used to find out if cancer cells have spread. The process used to find out if cancer has spread from where it began to other parts of the body is called staging. There is no standard staging system for Ewing sarcoma. The results of the tests and procedures done to diagnose and stage Ewing sarcoma are used to group the tumors into localized or metastatic. Ewing sarcoma is described based on whether the cancer has spread from the bone or soft tissue in which the cancer began. Ewing sarcoma is described as either localized or metastatic. Localized Ewing sarcoma The cancer is found in the bone or soft tissue in which it began and may have spread to nearby tissue, including nearby lymph nodes. Metastatic Ewing sarcoma The cancer has spread from the bone or soft tissue in which it began to other parts of the body. In Ewing tumor of bone, the cancer most often spreads to the lung, other bones, and bone marrow. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if Ewing sarcoma spreads to the lung, the cancer cells in the lung are actually Ewing sarcoma cells. The disease is metastatic Ewing sarcoma, not lung cancer.",Ewing Sarcoma,0000009_1,CancerGov,https://www.cancer.gov/types/bone/patient/ewing-treatment-pdq,C1261473,T191,Disorders What are the treatments for Ewing Sarcoma ?,0000009_1-6,treatment,"Key Points - There are different types of treatment for children with Ewing sarcoma. - Children with Ewing sarcoma should have their treatment planned by a team of health care providers who are experts in treating cancer in children. - Treatment for Ewing sarcoma may cause side effects. - Five types of standard treatment are used: - Chemotherapy - Radiation therapy - Surgery - Targeted therapy - High-dose chemotherapy with stem cell rescue - New types of treatment are being tested in clinical trials. - Chimeric antigen receptor (CAR) T-cell therapy - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for children with Ewing sarcoma. Different types of treatments are available for children with Ewing sarcoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment. Children with Ewing sarcoma should have their treatment planned by a team of health care providers who are experts in treating cancer in children. Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other health care providers who are experts in treating children with Ewing sarcoma and who specialize in certain areas of medicine. These may include the following specialists: - Pediatrician. - Surgical oncologist or orthopedic oncologist. - Radiation oncologist. - Pediatric nurse specialist. - Social worker. - Rehabilitation specialist. - Psychologist. Treatment for Ewing sarcoma may cause side effects. For information about side effects that begin during treatment for cancer, see our Side Effects page. Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include the following: - Physical problems. - Changes in mood, feelings, thinking, learning, or memory. - Second cancers (new types of cancer). Patients treated for Ewing sarcoma have an increased risk of acute myeloid leukemia and myelodysplastic syndrome. There is also an increased risk of sarcoma in the area treated with radiation therapy. Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information.) Five types of standard treatment are used: Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is treatment using more than one anticancer drug. Systemic chemotherapy is part of the treatment for all patients with Ewing tumors. It is often the first treatment given and lasts for about 6 to 12 months. Chemotherapy is often given to shrink the tumor before surgery or radiation therapy and to kill any tumor cells that may have spread to other parts of the body. See Drugs Approved for Soft Tissue Sarcoma for more information. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. External radiation therapy is used to treat Ewing sarcoma. Radiation therapy is used when the tumor cannot be removed by surgery or when surgery to remove the tumor will affect important body functions or the way the child will look. It may be used to make the tumor smaller and decrease the amount of tissue that needs to be removed during surgery. It may also be used to treat any tumor that remains after surgery and tumors that have spread to other parts of the body. Surgery Surgery is usually done to remove cancer that is left after chemotherapy or radiation therapy. When possible, the whole tumor is removed by surgery. Tissue and bone that are removed may be replaced with a graft, which uses tissue and bone taken from another part of the patient's body or a donor. Sometimes an implant, such as artificial bone, is used. Even if the doctor removes all of the cancer that can be seen at the time of the operation, chemotherapy or radiation therapy may be given after surgery to kill any cancer cells that are left. Chemotherapy or radiation therapy given after surgery to lower the risk that the cancer will come back is called adjuvant therapy. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Monoclonal antibody therapy is a type of targeted therapy used in the treatment of recurrent Ewing sarcoma. It is being studied for the treatment of metastatic Ewing sarcoma. Monoclonal antibodies are made in the laboratory from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. New types of targeted therapy are being studied. - Kinase inhibitor therapy is another type of targeted therapy. Kinase inhibitors are drugs that block a protein needed for cancer cells to divide. They are being studied in the treatment of recurrent Ewing sarcoma. - PARP inhibitor therapy is another type of targeted therapy. PARP inhibitors are drugs that block DNA repair and may cause cancer cells to die. They are being studied in the treatment of recurrent Ewing sarcoma. High-dose chemotherapy with stem cell rescue High-dose chemotherapy with stem cell rescue is a way of giving high doses of chemotherapy to treat Ewing sarcoma and then replacing blood -forming cells destroyed by cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient and are frozen and stored. After chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. Chemotherapy with stem cell rescue is used to treat recurrent Ewing sarcoma. New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Chimeric antigen receptor (CAR) T-cell therapy CAR T-cell therapy is a type of immunotherapy that changes the patient's T cells (a type of immune system cell) so they will attack certain proteins on the surface of cancer cells. T cells are taken from the patient and special receptors are added to their surface in the laboratory. The changed cells are called chimeric antigen receptor (CAR) T cells. The CAR T cells are grown in the laboratory and given to the patient by infusion. The CAR T cells multiply in the patient's blood and attack cancer cells. CAR T-cell therapy is being studied in the treatment of Ewing sarcoma that has recurred. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Ewing Sarcoma Localized Ewing Sarcoma Standard treatments for localized Ewing sarcoma include: - Chemotherapy. - Surgery and/or radiation therapy. These treatments and the order they are given depend on the following: - Where in the body the tumor started. - How large the tumor is when the cancer is diagnosed. - Whether the tumor was completely removed by surgery. - The child's age and general health. - Whether the treatment will affect important body functions or the way the child will look. Treatments being studied for localized Ewing sarcoma include: - High-dose chemotherapy with stem cell rescue. Check the list of NCI-supported cancer clinical trials that are now accepting patients with localized Ewing sarcoma/peripheral primitive neuroectodermal tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Metastatic Ewing Sarcoma Standard treatments for metastatic Ewing sarcoma include: - Chemotherapy. - Surgery. - Radiation therapy. These treatments and the order they are given depend on the following: - Where in the body the tumor started. - Where the tumor has spread. - How large the tumor is. - Whether the treatment will affect important body functions or the way the child will look. - The child's age and general health. Treatments being studied for metastatic Ewing sarcoma include the following: - Combination chemotherapy with or without targeted therapy. Radiation therapy is given to areas of bone where cancer has spread. - High-dose chemotherapy with stem cell rescue. Check the list of NCI-supported cancer clinical trials that are now accepting patients with metastatic Ewing sarcoma/peripheral primitive neuroectodermal tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Recurrent Ewing Sarcoma There is no standard treatment for recurrent Ewing sarcoma but treatment options may include the following: - Combination chemotherapy. - Radiation therapy to bone tumors, as palliative therapy to relieve symptoms and improve the quality of life. - Radiation therapy that may be followed by surgery to remove tumors that have spread to the lungs. - High-dose chemotherapy with stem cell rescue. - Targeted therapy with a monoclonal antibody. These treatments and the order they are given depend on the following: - Where in the body the tumor came back. - The initial treatment given. Treatment options being studied for recurrent Ewing sarcoma include the following: - Targeted therapy with a monoclonal antibody. - Chimeric antigen receptor (CAR) T-cell therapy. - Targeted therapy with a PARP inhibitor and chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent Ewing sarcoma/peripheral primitive neuroectodermal tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website.",Ewing Sarcoma,0000009_1,CancerGov,https://www.cancer.gov/types/bone/patient/ewing-treatment-pdq,C1261473,T191,Disorders what research (or clinical trials) is being done for Ewing Sarcoma ?,0000009_1-7,research,"New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Chimeric antigen receptor (CAR) T-cell therapy CAR T-cell therapy is a type of immunotherapy that changes the patient's T cells (a type of immune system cell) so they will attack certain proteins on the surface of cancer cells. T cells are taken from the patient and special receptors are added to their surface in the laboratory. The changed cells are called chimeric antigen receptor (CAR) T cells. The CAR T cells are grown in the laboratory and given to the patient by infusion. The CAR T cells multiply in the patient's blood and attack cancer cells. CAR T-cell therapy is being studied in the treatment of Ewing sarcoma that has recurred. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Ewing Sarcoma,0000009_1,CancerGov,https://www.cancer.gov/types/bone/patient/ewing-treatment-pdq,C1261473,T191,Disorders What is (are) Osteosarcoma and Malignant Fibrous Histiocytoma of Bone ?,0000009_2-1,information,"Key Points - Osteosarcoma and malignant fibrous histiocytoma (MFH) of the bone are diseases in which malignant (cancer) cells form in bone. - Having past treatment with radiation can increase the risk of osteosarcoma. - Signs and symptoms of osteosarcoma and MFH include swelling over a bone or a bony part of the body and joint pain. - Imaging tests are used to detect (find) osteosarcoma and MFH. - A biopsy is done to diagnose osteosarcoma. - Certain factors affect prognosis (chance of recovery) and treatment options. Osteosarcoma and malignant fibrous histiocytoma (MFH) of the bone are diseases in which malignant (cancer) cells form in bone. Osteosarcoma usually starts in osteoblasts, which are a type of bone cell that becomes new bone tissue. Osteosarcoma is most common in adolescents. It commonly forms in the ends of the long bones of the body, which include bones of the arms and legs. In children and adolescents, it often forms in the bones near the knee. Rarely, osteosarcoma may be found in soft tissue or organs in the chest or abdomen. Osteosarcoma is the most common type of bone cancer. Malignant fibrous histiocytoma (MFH) of bone is a rare tumor of the bone. It is treated like osteosarcoma. Ewing sarcoma is another kind of bone cancer, but it is not covered in this summary. See the PDQ summary about Ewing Sarcoma Treatment for more information.",Osteosarcoma and Malignant Fibrous Histiocytoma of Bone,0000009_2,CancerGov,https://www.cancer.gov/types/bone/patient/osteosarcoma-treatment-pdq,C0002991,T191,Disorders Who is at risk for Osteosarcoma and Malignant Fibrous Histiocytoma of Bone? ?,0000009_2-2,susceptibility,"Having past treatment with radiation can increase the risk of osteosarcoma. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesnt mean that you will not get cancer. Talk with your child's doctor if you think your child may be at risk. Risk factors for osteosarcoma include the following: - Past treatment with radiation therapy. - Past treatment with anticancer drugs called alkylating agents. - Having a certain change in the retinoblastoma gene. - Having certain conditions, such as the following: - Bloom syndrome. - Diamond-Blackfan anemia. - Li-Fraumeni syndrome. - Paget disease. - Hereditary retinoblastoma. - Rothmund-Thomson syndrome. - Werner syndrome.",Osteosarcoma and Malignant Fibrous Histiocytoma of Bone,0000009_2,CancerGov,https://www.cancer.gov/types/bone/patient/osteosarcoma-treatment-pdq,C0002991,T191,Disorders What are the symptoms of Osteosarcoma and Malignant Fibrous Histiocytoma of Bone ?,0000009_2-3,symptoms,Signs and symptoms of osteosarcoma and MFH include swelling over a bone or a bony part of the body and joint pain. These and other signs and symptoms may be caused by osteosarcoma or MFH or by other conditions. Check with a doctor if your child has any of the following: - Swelling over a bone or bony part of the body. - Pain in a bone or joint. - A bone that breaks for no known reason.,Osteosarcoma and Malignant Fibrous Histiocytoma of Bone,0000009_2,CancerGov,https://www.cancer.gov/types/bone/patient/osteosarcoma-treatment-pdq,C0002991,T191,Disorders How to diagnose Osteosarcoma and Malignant Fibrous Histiocytoma of Bone ?,0000009_2-4,exams and tests,"Imaging tests are used to detect (find) osteosarcoma and MFH. Imaging tests are done before the biopsy. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - X-ray : An x-ray of the organs and bones inside the body. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). A biopsy is done to diagnose osteosarcoma. Cells and tissues are removed during a biopsy so they can be viewed under a microscope by a pathologist to check for signs of cancer. It is important that the biopsy be done by a surgeon who is an expert in treating cancer of the bone. It is best if that surgeon is also the one who removes the tumor. The biopsy and the surgery to remove the tumor are planned together. The way the biopsy is done affects which type of surgery can be done later. The type of biopsy that is done will be based on the size of the tumor and where it is in the body. There are two types of biopsy that may be used: - Core biopsy : The removal of tissue using a wide needle. - Incisional biopsy : The removal of part of a lump or a sample of tissue that doesn't look normal. The following test may be done on the tissue that is removed: - Light and electron microscopy : A laboratory test in which cells in a sample of tissue are viewed under regular and high-powered microscopes to look for certain changes in the cells.",Osteosarcoma and Malignant Fibrous Histiocytoma of Bone,0000009_2,CancerGov,https://www.cancer.gov/types/bone/patient/osteosarcoma-treatment-pdq,C0002991,T191,Disorders What is the outlook for Osteosarcoma and Malignant Fibrous Histiocytoma of Bone ?,0000009_2-5,outlook,"Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) is affected by certain factors before and after treatment. The prognosis of untreated osteosarcoma and MFH depends on the following: - Where the tumor is in the body and whether tumors formed in more than one bone. - The size of the tumor. - Whether the cancer has spread to other parts of the body and where it has spread. - The type of tumor (based on how the cancer cells look under a microscope). - The patient's age and weight at diagnosis. - Whether the tumor has caused a break in the bone. - Whether the patient has certain genetic diseases. After osteosarcoma or MFH is treated, prognosis also depends on the following: - How much of the cancer was killed by chemotherapy. - How much of the tumor was taken out by surgery. - Whether chemotherapy is delayed for more than 3 weeks after surgery takes place. - Whether the cancer has recurred (come back) within 2 years of diagnosis. Treatment options for osteosarcoma and MFH depend on the following: - Where the tumor is in the body. - The size of the tumor. - The stage of the cancer. - Whether the bones are still growing. - The patient's age and general health. - The desire of the patient and family for the patient to be able to participate in activities such as sports or have a certain appearance. - Whether the cancer is newly diagnosed or has recurred after treatment.",Osteosarcoma and Malignant Fibrous Histiocytoma of Bone,0000009_2,CancerGov,https://www.cancer.gov/types/bone/patient/osteosarcoma-treatment-pdq,C0002991,T191,Disorders What are the stages of Osteosarcoma and Malignant Fibrous Histiocytoma of Bone ?,0000009_2-6,stages,"Key Points - After osteosarcoma or malignant fibrous histiocytoma (MFH) has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - Osteosarcoma and MFH are described as either localized or metastatic. After osteosarcoma or malignant fibrous histiocytoma (MFH) has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body. The process used to find out if cancer has spread to other parts of the body is called staging. For osteosarcoma and malignant fibrous histiocytoma (MFH), most patients are grouped according to whether cancer is found in only one part of the body or has spread. The following tests and procedures may be used: - X-ray : An x-ray of the organs, such as the chest, and bones inside the body. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. X-rays will be taken of the chest and the area where the tumor formed. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the chest, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. Pictures will be taken of the chest and the area where the tumor formed. - PET-CT scan : A procedure that combines the pictures from a positron emission tomography (PET) scan and a computed tomography (CT) scan. The PET and CT scans are done at the same time on the same machine. The pictures from both scans are combined to make a more detailed picture than either test would make by itself. A PET scan is a procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Bone scan : A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones and is detected by a scanner. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if osteosarcoma spreads to the lung, the cancer cells in the lung are actually osteosarcoma cells. The disease is metastatic osteosarcoma, not lung cancer. Osteosarcoma and MFH are described as either localized or metastatic. - Localized osteosarcoma or MFH has not spread out of the bone where the cancer started. There may be one or more areas of cancer in the bone that can be removed during surgery. - Metastatic osteosarcoma or MFH has spread from the bone in which the cancer began to other parts of the body. The cancer most often spreads to the lungs. It may also spread to other bones.",Osteosarcoma and Malignant Fibrous Histiocytoma of Bone,0000009_2,CancerGov,https://www.cancer.gov/types/bone/patient/osteosarcoma-treatment-pdq,C0002991,T191,Disorders What are the treatments for Osteosarcoma and Malignant Fibrous Histiocytoma of Bone ?,0000009_2-7,treatment,"Key Points - There are different types of treatment for patients with osteosarcoma or malignant fibrous histiocytoma (MFH) of bone. - Children with osteosarcoma or MFH should have their treatment planned by a team of health care providers who are experts in treating cancer in children. - Treatment for osteosarcoma or malignant fibrous histiocytoma may cause side effects. - Four types of standard treatment are used: - Surgery - Chemotherapy - Radiation therapy - Samarium - New types of treatment are being tested in clinical trials. - Targeted therapy - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with osteosarcoma or malignant fibrous histiocytoma (MFH) of bone. Different types of treatment are available for children with osteosarcoma or malignant fibrous histiocytoma (MFH) of bone. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment. Children with osteosarcoma or MFH should have their treatment planned by a team of health care providers who are experts in treating cancer in children. Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other pediatric health care providers who are experts in treating osteosarcoma and MFH and who specialize in certain areas of medicine. These may include the following specialists: - Pediatrician. - Orthopedic surgeon. - Radiation oncologist. - Rehabilitation specialist. - Pediatric nurse specialist. - Social worker. - Psychologist. Treatment for osteosarcoma or malignant fibrous histiocytoma may cause side effects. For information about side effects that begin during treatment for cancer, see our Side Effects page. Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include the following: - Physical problems. - Changes in mood, feelings, thinking, learning, or memory. - Second cancers (new types of cancer). Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information). Four types of standard treatment are used: Surgery Surgery to remove the entire tumor will be done when possible. Chemotherapy may be given before surgery to make the tumor smaller. This is called neoadjuvant chemotherapy. Chemotherapy is given so less bone tissue needs to be removed and there are fewer problems after surgery. The following types of surgery may be done: - Wide local excision: Surgery to remove the cancer and some healthy tissue around it. - Limb-sparing surgery: Removal of the tumor in a limb (arm or leg) without amputation, so the use and appearance of the limb is saved. Most patients with osteosarcoma in a limb can be treated with limb-sparing surgery. The tumor is removed by wide local excision. Tissue and bone that are removed may be replaced with a graft using tissue and bone taken from another part of the patient's body, or with an implant such as artificial bone. If a fracture is found at diagnosis or during chemotherapy before surgery, limb-sparing surgery may still be possible in some cases. If the surgeon is not able to remove all of the tumor and enough healthy tissue around it, an amputation may be done. - Amputation: Surgery to remove part or all of an arm or leg. This may be done when it is not possible to remove all of the tumor in limb-sparing surgery. The patient may be fitted with a prosthesis (artificial limb) after amputation. - Rotationplasty: Surgery to remove the tumor and the knee joint. The part of the leg that remains below the knee is then attached to the part of the leg that remains above the knee, with the foot facing backward and the ankle acting as a knee. A prosthesis may then be attached to the foot. Studies have shown that survival is the same whether the first surgery done is a limb-sparing surgery or an amputation. Even if the doctor removes all the cancer that can be seen at the time of the surgery, patients are also given chemotherapy after surgery to kill any cancer cells that are left in the area where the tumor was removed or that have spread to other parts of the body. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is the use of more than one anticancer drug. The way the chemotherapy is given depends on the type and stage of the cancer being treated. In the treatment of osteosarcoma and malignant fibrous histiocytosis of bone, chemotherapy is usually given before and after surgery to remove the primary tumor. See Drugs Approved for Bone Cancer for more information. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. Osteosarcoma and MFH cells are not killed easily by external radiation therapy. It may be used when a small amount of cancer is left after surgery or used together with other treatments. Samarium Samarium is a radioactive drug that targets areas where bone cells are growing, such as tumor cells in bone. It helps relieve pain caused by cancer in the bone and it also kills blood cells in the bone marrow. It also is used to treat osteosarcoma that has come back after treatment in a different bone. Treatment with samarium may be followed by stem cell transplant. Before treatment with samarium, stem cells (immature blood cells) are removed from the blood or bone marrow of the patient and are frozen and stored. After treatment with samarium is complete, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about ongoing clinical trials is available from the NCI website. Targeted therapy Targeted therapy is a treatment that uses drugs or other substances to find and attack specific cancer cells without harming normal cells. Kinase inhibitor therapy and monoclonal antibody therapy are types of targeted therapy being studied in clinical trials for osteosarcoma. Kinase inhibitor therapy blocks a protein needed for cancer cells to divide. Sorafenib is a type of kinase inhibitor therapy being studied for the treatment of recurrent osteosarcoma. Monoclonal antibody therapy is a cancer treatment that uses antibodies made in the laboratory, from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Denosumab, dinutuximab, and glembatumumab are monoclonal antibodies being studied for the treatment of recurrent osteosarcoma. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Osteosarcoma and Malignant Fibrous Histiocytoma of Bone Localized Osteosarcoma and Malignant Fibrous Histiocytoma of Bone Treatment may include the following: - Surgery. Combination chemotherapy is usually given before and after surgery. - Surgery followed by radiation therapy when the tumor cannot be completely removed by surgery. Check the list of NCI-supported cancer clinical trials that are now accepting patients with localized osteosarcoma and localized childhood malignant fibrous histiocytoma of bone. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Metastatic Osteosarcoma and Malignant Fibrous Histiocytoma of Bone Lung Metastasis When osteosarcoma or malignant fibrous histiocytoma (MFH) spreads, it usually spreads to the lung. Treatment of osteosarcoma and MFH with lung metastasis may include the following: - Combination chemotherapy followed by surgery to remove the primary cancer and the cancer that has spread to the lung. Bone Metastasis or Bone with Lung Metastasis Osteosarcoma and malignant fibrous histiocytoma may spread to a distant bone and/or the lung. Treatment may include the following: - Combination chemotherapy followed by surgery to remove the primary tumor and the cancer that has spread to other parts of the body. More chemotherapy is given after surgery. - Surgery to remove the primary tumor followed by chemotherapy and surgery to remove cancer that has spread to other parts of the body. Check the list of NCI-supported cancer clinical trials that are now accepting patients with metastatic osteosarcoma and metastatic childhood malignant fibrous histiocytoma of bone. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Recurrent Osteosarcoma and Malignant Fibrous Histiocytoma of Bone Treatment of recurrent osteosarcoma and malignant fibrous histiocytoma of bone may include the following: - Surgery to remove the tumor with or without chemotherapy. - Samarium with or without stem cell transplant using the patient's own stem cells, as palliative treatment to relieve pain and improve the quality of life. - A clinical trial of new types of treatment for patients whose cancer cannot be removed by surgery. These may include targeted therapy such as kinase inhibitor therapy or monoclonal antibody therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent osteosarcoma and recurrent childhood malignant fibrous histiocytoma of bone. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website.",Osteosarcoma and Malignant Fibrous Histiocytoma of Bone,0000009_2,CancerGov,https://www.cancer.gov/types/bone/patient/osteosarcoma-treatment-pdq,C0002991,T191,Disorders what research (or clinical trials) is being done for Osteosarcoma and Malignant Fibrous Histiocytoma of Bone ?,0000009_2-8,research,"New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about ongoing clinical trials is available from the NCI website. Targeted therapy Targeted therapy is a treatment that uses drugs or other substances to find and attack specific cancer cells without harming normal cells. Kinase inhibitor therapy and monoclonal antibody therapy are types of targeted therapy being studied in clinical trials for osteosarcoma. Kinase inhibitor therapy blocks a protein needed for cancer cells to divide. Sorafenib is a type of kinase inhibitor therapy being studied for the treatment of recurrent osteosarcoma. Monoclonal antibody therapy is a cancer treatment that uses antibodies made in the laboratory, from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Denosumab, dinutuximab, and glembatumumab are monoclonal antibodies being studied for the treatment of recurrent osteosarcoma. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.",Osteosarcoma and Malignant Fibrous Histiocytoma of Bone,0000009_2,CancerGov,https://www.cancer.gov/types/bone/patient/osteosarcoma-treatment-pdq,C0002991,T191,Disorders What is (are) Gastrointestinal Carcinoid Tumors ?,0000010_1-1,information,"Key Points - A gastrointestinal carcinoid tumor is cancer that forms in the lining of the gastrointestinal tract. - Health history can affect the risk of gastrointestinal carcinoid tumors. - Some gastrointestinal carcinoid tumors have no signs or symptoms in the early stages. - Carcinoid syndrome may occur if the tumor spreads to the liver or other parts of the body. - Imaging studies and tests that examine the blood and urine are used to detect (find) and diagnose gastrointestinal carcinoid tumors. - Certain factors affect prognosis (chance of recovery) and treatment options. A gastrointestinal carcinoid tumor is cancer that forms in the lining of the gastrointestinal tract. The gastrointestinal (GI) tract is part of the body's digestive system. It helps to digest food, takes nutrients (vitamins, minerals, carbohydrates, fats, proteins, and water) from food to be used by the body and helps pass waste material out of the body. The GI tract is made up of these and other organs: - Stomach. - Small intestine (duodenum, jejunum, and ileum). - Colon. - Rectum. Gastrointestinal carcinoid tumors form from a certain type of neuroendocrine cell (a type of cell that is like a nerve cell and a hormone -making cell). These cells are scattered throughout the chest and abdomen but most are found in the GI tract. Neuroendocrine cells make hormones that help control digestive juices and the muscles used in moving food through the stomach and intestines. A GI carcinoid tumor may also make hormones and release them into the body. GI carcinoid tumors are rare and most grow very slowly. Most of them occur in the small intestine, rectum, and appendix. Sometimes more than one tumor will form. See the following PDQ summaries for more information related to GI and other types of carcinoid tumors: - Non-Small Cell Lung Cancer Treatment. - Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment. - Rectal Cancer Treatment. - Small Intestine Cancer Treatment. - Unusual Cancers of Childhood Treatment Carcinoid syndrome may occur if the tumor spreads to the liver or other parts of the body. The hormones made by gastrointestinal carcinoid tumors are usually destroyed by liver enzymes in the blood. If the tumor has spread to the liver and the liver enzymes cannot destroy the extra hormones made by the tumor, high amounts of these hormones may remain in the body and cause carcinoid syndrome. This can also happen if tumor cells enter the blood. Signs and symptoms of carcinoid syndrome include the following: - Redness or a feeling of warmth in the face and neck. - Abdominal pain. - Feeling bloated. - Diarrhea. - Wheezing or other trouble breathing. - Fast heartbeat. These signs and symptoms may be caused by gastrointestinal carcinoid tumors or by other conditions. Talk to your doctor if you have any of these signs or symptoms.",Gastrointestinal Carcinoid Tumors,0000010_1,CancerGov,https://www.cancer.gov/types/gi-carcinoid-tumors/patient/gi-carcinoid-treatment-pdq,C0220620,T191,Disorders Who is at risk for Gastrointestinal Carcinoid Tumors? ?,0000010_1-2,susceptibility,"Health history can affect the risk of gastrointestinal carcinoid tumors. Anything that increases a person's chance of developing a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesnt mean that you will not get cancer. Talk to your doctor if you think you may be at risk. Risk factors for GI carcinoid tumors include the following: - Having a family history of multiple endocrine neoplasia type 1 (MEN1) syndrome or neurofibromatosis type 1 (NF1) syndrome. - Having certain conditions that affect the stomach's ability to make stomach acid, such as atrophic gastritis, pernicious anemia, or Zollinger-Ellison syndrome.",Gastrointestinal Carcinoid Tumors,0000010_1,CancerGov,https://www.cancer.gov/types/gi-carcinoid-tumors/patient/gi-carcinoid-treatment-pdq,C0220620,T191,Disorders What are the symptoms of Gastrointestinal Carcinoid Tumors ?,0000010_1-3,symptoms,"Some gastrointestinal carcinoid tumors have no signs or symptoms in the early stages.Signs and symptoms may be caused by the growth of the tumor and/or the hormones the tumor makes. Some tumors, especially tumors of the stomach or appendix, may not cause signs or symptoms. Carcinoid tumors are often found during tests or treatments for other conditions. Carcinoid tumors in the small intestine (duodenum, jejunum, and ileum), colon, and rectum sometimes cause signs or symptoms as they grow or because of the hormones they make. Other conditions may cause the same signs or symptoms. Check with your doctor if you have any of the following: - Duodenum Signs and symptoms of GI carcinoid tumors in the duodenum (first part of the small intestine, that connects to the stomach) may include the following: - Abdominal pain. - Constipation. - Diarrhea. - Change in stool color. - Nausea. - Vomiting. - Jaundice (yellowing of the skin and whites of the eyes). - Heartburn. - Jejunum and ileum Signs and symptoms of GI carcinoid tumors in the jejunum (middle part of the small intestine) and ileum (last part of the small intestine, that connects to the colon) may include the following: - Abdominal pain. - Weight loss for no known reason. - Feeling very tired. - Feeling bloated - Diarrhea. - Nausea. - Vomiting. - Colon Signs and symptoms of GI carcinoid tumors in the colon may include the following: - Abdominal pain. - Weight loss for no known reason. - Rectum Signs and symptoms of GI carcinoid tumors in the rectum may include the following: - Blood in the stool. - Pain in the rectum. - Constipation.",Gastrointestinal Carcinoid Tumors,0000010_1,CancerGov,https://www.cancer.gov/types/gi-carcinoid-tumors/patient/gi-carcinoid-treatment-pdq,C0220620,T191,Disorders How to diagnose Gastrointestinal Carcinoid Tumors ?,0000010_1-4,exams and tests,"Imaging studies and tests that examine the blood and urine are used to detect (find) and diagnose gastrointestinal carcinoid tumors. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances, such as hormones, released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. The blood sample is checked to see if it contains a hormone produced by carcinoid tumors. This test is used to help diagnose carcinoid syndrome. - Tumor marker test : A procedure in which a sample of blood, urine, or tissue is checked to measure the amounts of certain substances, such as chromogranin A, made by organs, tissues, or tumor cells in the body. Chromogranin A is a tumor marker. It has been linked to neuroendocrine tumors when found in increased levels in the body. - Twenty-four-hour urine test: A test in which urine is collected for 24 hours to measure the amounts of certain substances, such as 5-HIAA or serotonin (hormone). An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. This test is used to help diagnose carcinoid syndrome. - MIBG scan : A procedure used to find neuroendocrine tumors, such as carcinoid tumors. A very small amount of radioactive material called MIBG (metaiodobenzylguanidine) is injected into a vein and travels through the bloodstream. Carcinoid tumors take up the radioactive material and are detected by a device that measures radiation. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells. - Endoscopic ultrasound (EUS): A procedure in which an endoscope is inserted into the body, usually through the mouth or rectum. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. A probe at the end of the endoscope is used to bounce high-energy sound waves (ultrasound) off internal tissues or organs, such as the stomach, small intestine, colon, or rectum, and make echoes. The echoes form a picture of body tissues called a sonogram. This procedure is also called endosonography. - Upper endoscopy : A procedure to look at organs and tissues inside the body to check for abnormal areas. An endoscope is inserted through the mouth and passed through the esophagus into the stomach. Sometimes the endoscope also is passed from the stomach into the small intestine. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of disease. - Colonoscopy : A procedure to look inside the rectum and colon for polyps, abnormal areas, or cancer. A colonoscope is inserted through the rectum into the colon. A colonoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove polyps or tissue samples, which are checked under a microscope for signs of cancer. - Capsule endoscopy : A procedure used to see all of the small intestine. The patient swallows a capsule that contains a tiny camera. As the capsule moves through the gastrointestinal tract, the camera takes pictures and sends them to a receiver worn on the outside of the body. - Biopsy : The removal of cells or tissues so they can be viewed under a microscope to check for signs of cancer. Tissue samples may be taken during endoscopy and colonoscopy.",Gastrointestinal Carcinoid Tumors,0000010_1,CancerGov,https://www.cancer.gov/types/gi-carcinoid-tumors/patient/gi-carcinoid-treatment-pdq,C0220620,T191,Disorders What is the outlook for Gastrointestinal Carcinoid Tumors ?,0000010_1-5,outlook,"Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - Where the tumor is in the gastrointestinal tract. - The size of the tumor. - Whether the cancer has spread from the stomach and intestines to other parts of the body, such as the liver or lymph nodes. - Whether the patient has carcinoid syndrome or has carcinoid heart syndrome. - Whether the cancer can be completely removed by surgery. - Whether the cancer is newly diagnosed or has recurred.",Gastrointestinal Carcinoid Tumors,0000010_1,CancerGov,https://www.cancer.gov/types/gi-carcinoid-tumors/patient/gi-carcinoid-treatment-pdq,C0220620,T191,Disorders What are the stages of Gastrointestinal Carcinoid Tumors ?,0000010_1-6,stages,"Key Points - After a gastrointestinal carcinoid tumor has been diagnosed, tests are done to find out if cancer cells have spread within the stomach and intestines or to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - The plan for cancer treatment depends on where the carcinoid tumor is found and whether it can be removed by surgery. After a gastrointestinal carcinoid tumor has been diagnosed, tests are done to find out if cancer cells have spread within the stomach and intestines or to other parts of the body. Staging is the process used to find out how far the cancer has spread. The information gathered from the staging process determines the stage of the disease. The results of tests and procedures used to diagnose gastrointestinal (GI) carcinoid tumors may also be used for staging. See the General Information section for a description of these tests and procedures. A bone scan may be done to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones and is detected by a scanner. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of tumor as the primary tumor. For example, if a gastrointestinal (GI) carcinoid tumor spreads to the liver, the tumor cells in the liver are actually GI carcinoid tumor cells. The disease is metastatic GI carcinoid tumor, not liver cancer. The plan for cancer treatment depends on where the carcinoid tumor is found and whether it can be removed by surgery. For many cancers it is important to know the stage of the cancer in order to plan treatment. However, the treatment of gastrointestinal carcinoid tumors is not based on the stage of the cancer. Treatment depends mainly on whether the tumor can be removed by surgery and if the tumor has spread. Treatment is based on whether the tumor: - Can be completely removed by surgery. - Has spread to other parts of the body. - Has come back after treatment. The tumor may come back in the stomach or intestines or in other parts of the body. - Has not gotten better with treatment.",Gastrointestinal Carcinoid Tumors,0000010_1,CancerGov,https://www.cancer.gov/types/gi-carcinoid-tumors/patient/gi-carcinoid-treatment-pdq,C0220620,T191,Disorders What are the treatments for Gastrointestinal Carcinoid Tumors ?,0000010_1-7,treatment,"Key Points - There are different types of treatment for patients with gastrointestinal carcinoid tumors. - Four types of standard treatment are used: - Surgery - Radiation therapy - Chemotherapy - Hormone therapy - Treatment for carcinoid syndrome may also be needed. - New types of treatment are being tested in clinical trials. - Targeted therapy - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with gastrointestinal carcinoid tumors. Different types of treatment are available for patients with gastrointestinal carcinoid tumor. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Four types of standard treatment are used: Surgery Treatment of GI carcinoid tumors usually includes surgery. One of the following surgical procedures may be used: - Endoscopic resection: Surgery to remove a small tumor that is on the inside lining of the GI tract. An endoscope is inserted through the mouth and passed through the esophagus to the stomach and sometimes, the duodenum. An endoscope is a thin, tube-like instrument with a light, a lens for viewing, and a tool for removing tumor tissue. - Local excision: Surgery to remove the tumor and a small amount of normal tissue around it. - Resection: Surgery to remove part or all of the organ that contains cancer. Nearby lymph nodes may also be removed. - Cryosurgery: A treatment that uses an instrument to freeze and destroy carcinoid tumor tissue. This type of treatment is also called cryotherapy. The doctor may use ultrasound to guide the instrument. - Radiofrequency ablation: The use of a special probe with tiny electrodes that release high-energy radio waves (similar to microwaves) that kill cancer cells. The probe may be inserted through the skin or through an incision (cut) in the abdomen. - Liver transplant: Surgery to remove the whole liver and replace it with a healthy donated liver. - Hepatic artery embolization: A procedure to embolize (block) the hepatic artery, which is the main blood vessel that brings blood into the liver. Blocking the flow of blood to the liver helps kill cancer cells growing there. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. Radiopharmaceutical therapy is a type of internal radiation therapy. Radiation is given to the tumor using a drug that has a radioactive substance, such as iodine I 131, attached to it. The radioactive substance kills the tumor cells. External and internal radiation therapy are used to treat gastrointestinal carcinoid tumors that have spread to other parts of the body. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Chemoembolization of the hepatic artery is a type of regional chemotherapy that may be used to treat a gastrointestinal carcinoid tumor that has spread to the liver. The anticancer drug is injected into the hepatic artery through a catheter (thin tube). The drug is mixed with a substance that embolizes (blocks) the artery, and cuts off blood flow to the tumor. Most of the anticancer drug is trapped near the tumor and only a small amount of the drug reaches other parts of the body. The blockage may be temporary or permanent, depending on the substance used to block the artery. The tumor is prevented from getting the oxygen and nutrients it needs to grow. The liver continues to receive blood from the hepatic portal vein, which carries blood from the stomach and intestine. The way the chemotherapy is given depends on the type and stage of the cancer being treated. Hormone therapy Hormone therapy with a somatostatin analogue is a treatment that stops extra hormones from being made. GI carcinoid tumors are treated with octreotide or lanreotide which are injected under the skin or into the muscle. Octreotide and lanreotide may also have a small effect on stopping tumor growth. Treatment for carcinoid syndrome may also be needed. Treatment of carcinoid syndrome may include the following: - Hormone therapy with a somatostatin analogue stops extra hormones from being made. Carcinoid syndrome is treated with octreotide or lanreotide to lessen flushing and diarrhea. Octreotide and lanreotide may also help slow tumor growth. - Interferon therapy stimulates the bodys immune system to work better and lessens flushing and diarrhea. Interferon may also help slow tumor growth. - Taking medicine for diarrhea. - Taking medicine for skin rashes. - Taking medicine to breathe easier. - Taking medicine before having anesthesia for a medical procedure. Other ways to help treat carcinoid syndrome include avoiding things that cause flushing or difficulty breathing such as alcohol, nuts, certain cheeses and foods with capsaicin, such as chili peppers. Avoiding stressful situations and certain types of physical activity can also help treat carcinoid syndrome. For some patients with carcinoid heart syndrome, a heart valve replacement may be done. New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Several types of targeted therapy are being studied in the treatment of GI carcinoid tumors. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Gastrointestinal Carcinoid Tumors Carcinoid Tumors in the Stomach Treatment of gastrointestinal (GI) carcinoid tumors in the stomach may include the following: - Endoscopic surgery (resection) for small tumors. - Surgery (resection) to remove part or all of the stomach. Nearby lymph nodes for larger tumors, tumors that grow deep into the stomach wall, or tumors that are growing and spreading quickly may also be removed. For patients with GI carcinoid tumors in the stomach and MEN1 syndrome, treatment may also include: - Surgery (resection) to remove tumors in the duodenum (first part of the small intestine, that connects to the stomach). - Hormone therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with localized gastrointestinal carcinoid tumor and regional gastrointestinal carcinoid tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Carcinoid Tumors in the Small Intestine It is not clear what the best treatment is for GI carcinoid tumors in the duodenum (first part of the small intestine, that connects to the stomach). Treatment may include the following: - Endoscopic surgery (resection) for small tumors. - Surgery (local excision) to remove slightly larger tumors. - Surgery (resection) to remove the tumor and nearby lymph nodes. Treatment of GI carcinoid tumors in the jejunum (middle part of the small intestine) and ileum (last part of the small intestine, that connects to the colon) may include the following: - Surgery (resection) to remove the tumor and the membrane that connects the intestines to the back of the abdominal wall. Nearby lymph nodes are also removed. - A second surgery to remove the membrane that connects the intestines to the back of the abdominal wall, if any tumor remains or the tumor continues to grow. - Hormone therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with localized gastrointestinal carcinoid tumor and regional gastrointestinal carcinoid tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Carcinoid Tumors in the Appendix Treatment of GI carcinoid tumors in the appendix may include the following: - Surgery (resection) to remove the appendix. - Surgery (resection) to remove the right side of the colon including the appendix. Nearby lymph nodes are also removed. Check the list of NCI-supported cancer clinical trials that are now accepting patients with localized gastrointestinal carcinoid tumor and regional gastrointestinal carcinoid tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Carcinoid Tumors in the Colon Treatment of GI carcinoid tumors in the colon may include the following: - Surgery (resection) to remove part of the colon and nearby lymph nodes, in order to remove as much of the cancer as possible. Check the list of NCI-supported cancer clinical trials that are now accepting patients with localized gastrointestinal carcinoid tumor and regional gastrointestinal carcinoid tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Carcinoid Tumors in the Rectum Treatment of GI carcinoid tumors in the rectum may include the following: - Endoscopic surgery (resection) for tumors that are smaller than 1 centimeter. - Surgery (resection) for tumors that are larger than 2 centimeters or that have spread to the muscle layer of the rectal wall. This may be either: - surgery to remove part of the rectum; or - surgery to remove the anus, the rectum, and part of the colon through an incision made in the abdomen. It is not clear what the best treatment is for tumors that are 1 to 2 centimeters. Treatment may include the following: - Endoscopic surgery (resection). - Surgery (resection) to remove part of the rectum. - Surgery (resection) to remove the anus, the rectum, and part of the colon through an incision made in the abdomen. Check the list of NCI-supported cancer clinical trials that are now accepting patients with localized gastrointestinal carcinoid tumor and regional gastrointestinal carcinoid tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Metastatic Gastrointestinal Carcinoid Tumors Distant metastases Treatment of distant metastases of GI carcinoid tumors is usually palliative therapy to relieve symptoms and improve quality of life. Treatment may include the following: - Surgery (resection) to remove as much of the tumor as possible. - Hormone therapy. - Radiopharmaceutical therapy. - External radiation therapy for cancer that has spread to the bone, brain, or spinal cord. - A clinical trial of a new treatment. Liver metastases Treatment of cancer that has spread to the liver may include the following: - Surgery (local excision) to remove the tumor from the liver. - Hepatic artery embolization. - Cryosurgery. - Radiofrequency ablation. - Liver transplant. Check the list of NCI-supported cancer clinical trials that are now accepting patients with metastatic gastrointestinal carcinoid tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Recurrent Gastrointestinal Carcinoid Tumors Treatment of recurrent GI carcinoid tumors may include the following: - Surgery (local excision) to remove part or all of the tumor. - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent gastrointestinal carcinoid tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Gastrointestinal Carcinoid Tumors,0000010_1,CancerGov,https://www.cancer.gov/types/gi-carcinoid-tumors/patient/gi-carcinoid-treatment-pdq,C0220620,T191,Disorders what research (or clinical trials) is being done for Gastrointestinal Carcinoid Tumors ?,0000010_1-8,research,"New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Several types of targeted therapy are being studied in the treatment of GI carcinoid tumors. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Gastrointestinal Carcinoid Tumors,0000010_1,CancerGov,https://www.cancer.gov/types/gi-carcinoid-tumors/patient/gi-carcinoid-treatment-pdq,C0220620,T191,Disorders What is (are) Myelodysplastic Syndromes ?,0000013_1-1,information,"Key Points - Myelodysplastic syndromes are a group of cancers in which immature blood cells in the bone marrow do not mature or become healthy blood cells. - The different types of myelodysplastic syndromes are diagnosed based on certain changes in the blood cells and bone marrow. - Age and past treatment with chemotherapy or radiation therapy affect the risk of a myelodysplastic syndrome. - Signs and symptoms of a myelodysplastic syndrome include shortness of breath and feeling tired. - Tests that examine the blood and bone marrow are used to detect (find) and diagnose myelodysplastic syndromes. - Certain factors affect prognosis and treatment options. Myelodysplastic syndromes are a group of cancers in which immature blood cells in the bone marrow do not mature or become healthy blood cells. In a healthy person, the bone marrow makes blood stem cells (immature cells) that become mature blood cells over time. A blood stem cell may become a lymphoid stem cell or a myeloid stem cell. A lymphoid stem cell becomes a white blood cell. A myeloid stem cell becomes one of three types of mature blood cells: - Red blood cells that carry oxygen and other substances to all tissues of the body. - Platelets that form blood clots to stop bleeding. - White blood cells that fight infection and disease. In a patient with a myelodysplastic syndrome, the blood stem cells (immature cells) do not become mature red blood cells, white blood cells, or platelets in the bone marrow. These immature blood cells, called blasts, do not work the way they should and either die in the bone marrow or soon after they go into the blood. This leaves less room for healthy white blood cells, red blood cells, and platelets to form in the bone marrow. When there are fewer healthy blood cells, infection, anemia, or easy bleeding may occur. The different types of myelodysplastic syndromes are diagnosed based on certain changes in the blood cells and bone marrow. - Refractory anemia: There are too few red blood cells in the blood and the patient has anemia. The number of white blood cells and platelets is normal. - Refractory anemia with ring sideroblasts: There are too few red blood cells in the blood and the patient has anemia. The red blood cells have too much iron inside the cell. The number of white blood cells and platelets is normal. - Refractory anemia with excess blasts: There are too few red blood cells in the blood and the patient has anemia. Five percent to 19% of the cells in the bone marrow are blasts. There also may be changes to the white blood cells and platelets. Refractory anemia with excess blasts may progress to acute myeloid leukemia (AML). See the PDQ Adult Acute Myeloid Leukemia Treatment summary for more information. - Refractory cytopenia with multilineage dysplasia: There are too few of at least two types of blood cells (red blood cells, platelets, or white blood cells). Less than 5% of the cells in the bone marrow are blasts and less than 1% of the cells in the blood are blasts. If red blood cells are affected, they may have extra iron. Refractory cytopenia may progress to acute myeloid leukemia (AML). - Refractory cytopenia with unilineage dysplasia: There are too few of one type of blood cell (red blood cells, platelets, or white blood cells). There are changes in 10% or more of two other types of blood cells. Less than 5% of the cells in the bone marrow are blasts and less than 1% of the cells in the blood are blasts. - Unclassifiable myelodysplastic syndrome: The numbers of blasts in the bone marrow and blood are normal, and the disease is not one of the other myelodysplastic syndromes. - Myelodysplastic syndrome associated with an isolated del(5q) chromosome abnormality: There are too few red blood cells in the blood and the patient has anemia. Less than 5% of the cells in the bone marrow and blood are blasts. There is a specific change in the chromosome. - Chronic myelomonocytic leukemia (CMML): See the PDQ summary on Myelodysplastic/ Myeloproliferative Neoplasms Treatment for more information.",Myelodysplastic Syndromes,0000013_1,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/myelodysplastic-treatment-pdq,C0026986,T191,Disorders What are the treatments for Myelodysplastic Syndromes ?,0000013_1-6,treatment,"Key Points - There are different types of treatment for patients with myelodysplastic syndromes. - Treatment for myelodysplastic syndromes includes supportive care, drug therapy, and stem cell transplantation. - Three types of standard treatment are used: - Supportive care - Drug therapy - Chemotherapy with stem cell transplant - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their treatment. - Follow-up tests may be needed. There are different types of treatment for patients with myelodysplastic syndromes. Different types of treatment are available for patients with myelodysplastic syndromes. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Treatment for myelodysplastic syndromes includes supportive care, drug therapy, and stem cell transplantation. Patients with a myelodysplastic syndrome who have symptoms caused by low blood counts are given supportive care to relieve symptoms and improve quality of life. Drug therapy may be used to slow progression of the disease. Certain patients can be cured with aggressive treatment with chemotherapy followed by stem cell transplant using stem cells from a donor. Three types of standard treatment are used: Supportive care Supportive care is given to lessen the problems caused by the disease or its treatment. Supportive care may include the following: - Transfusion therapy Transfusion therapy (blood transfusion) is a method of giving red blood cells, white blood cells, or platelets to replace blood cells destroyed by disease or treatment. A red blood cell transfusion is given when the red blood cell count is low and signs or symptoms of anemia, such as shortness of breath or feeling very tired, occur. A platelet transfusion is usually given when the patient is bleeding, is having a procedure that may cause bleeding, or when the platelet count is very low. Patients who receive many blood cell transfusions may have tissue and organ damage caused by the buildup of extra iron. These patients may be treated with iron chelation therapy to remove the extra iron from the blood. - Erythropoiesis-stimulating agents Erythropoiesis-stimulating agents (ESAs) may be given to increase the number of mature red blood cells made by the body and to lessen the effects of anemia. Sometimes granulocyte colony-stimulating factor (G-CSF) is given with ESAs to help the treatment work better. - Antibiotic therapy Antibiotics may be given to fight infection. Drug therapy - Lenalidomide Patients with myelodysplastic syndrome associated with an isolated del(5q) chromosome abnormality who need frequent red blood cell transfusions may be treated with lenalidomide. Lenalidomide is used to lessen the need for red blood cell transfusions. - Immunosuppressive therapy Antithymocyte globulin (ATG) works to suppress or weaken the immune system. It is used to lessen the need for red blood cell transfusions. - Azacitidine and decitabine Azacitidine and decitabine are used to treat myelodysplastic syndromes by killing cells that are dividing rapidly. They also help genes that are involved in cell growth to work the way they should. Treatment with azacitidine and decitabine may slow the progression of myelodysplastic syndromes to acute myeloid leukemia. - Chemotherapy used in acute myeloid leukemia (AML) Patients with a myelodysplastic syndrome and a high number of blasts in their bone marrow have a high risk of acute leukemia. They may be treated with the same chemotherapy regimen used in patients with acute myeloid leukemia. Chemotherapy with stem cell transplant Stem cell transplant is a method of giving chemotherapy and replacing blood-forming cells destroyed by the treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of a donor and are frozen for storage. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. This treatment may not work as well in patients whose myelodysplastic syndrome was caused by past treatment for cancer. New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.",Myelodysplastic Syndromes,0000013_1,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/myelodysplastic-treatment-pdq,C0026986,T191,Disorders What is (are) Chronic Myeloproliferative Neoplasms ?,0000013_2-1,information,"Key Points - Myeloproliferative neoplasms are a group of diseases in which the bone marrow makes too many red blood cells, white blood cells, or platelets. - There are 6 types of chronic myeloproliferative neoplasms. - Tests that examine the blood and bone marrow are used to detect (find) and diagnose chronic myeloproliferative neoplasms. Myeloproliferative neoplasms are a group of diseases in which the bone marrow makes too many red blood cells, white blood cells, or platelets. Normally, the bone marrow makes blood stem cells (immature cells) that become mature blood cells over time. A blood stem cell may become a myeloid stem cell or a lymphoid stem cell. A lymphoid stem cell becomes a white blood cell. A myeloid stem cell becomes one of three types of mature blood cells: - Red blood cells that carry oxygen and other substances to all tissues of the body. - White blood cells that fight infection and disease. - Platelets that form blood clots to stop bleeding. In myeloproliferative neoplasms, too many blood stem cells become one or more types of blood cells. The neoplasms usually get worse slowly as the number of extra blood cells increases. There are 6 types of chronic myeloproliferative neoplasms. The type of myeloproliferative neoplasm is based on whether too many red blood cells, white blood cells, or platelets are being made. Sometimes the body will make too many of more than one type of blood cell, but usually one type of blood cell is affected more than the others are. Chronic myeloproliferative neoplasms include the following 6 types: - Chronic myelogenous leukemia. - Polycythemia vera. - Primary myelofibrosis (also called chronic idiopathic myelofibrosis). - Essential thrombocythemia. - Chronic neutrophilic leukemia. - Chronic eosinophilic leukemia. These types are described below. Chronic myeloproliferative neoplasms sometimes become acute leukemia, in which too many abnormal white blood cells are made.",Chronic Myeloproliferative Neoplasms,0000013_2,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/chronic-treatment-pdq,C0027651,T191,Disorders How to diagnose Chronic Myeloproliferative Neoplasms ?,0000013_2-2,exams and tests,"Tests that examine the blood and bone marrow are used to detect (find) and diagnose chronic myeloproliferative neoplasms. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Complete blood count (CBC) with differential : A procedure in which a sample of blood is drawn and checked for the following: - The number of red blood cells and platelets. - The number and type of white blood cells. - The amount of hemoglobin (the protein that carries oxygen) in the red blood cells. - The portion of the blood sample made up of red blood cells. - Peripheral blood smear : A procedure in which a sample of blood is checked for the following: - Whether there are red blood cells shaped like teardrops. - The number and kinds of white blood cells. - The number of platelets. - Whether there are blast cells. - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. - Bone marrow aspiration and biopsy : The removal of bone marrow, blood, and a small piece of bone by inserting a hollow needle into the hipbone or breastbone. A pathologist views the bone marrow, blood, and bone under a microscope to look for abnormal cells. - Cytogenetic analysis : A test in which cells in a sample of blood or bone marrow are viewed under a microscope to look for certain changes in the chromosomes. Certain diseases or disorders may be diagnosed or ruled out based on the chromosomal changes. - Gene mutation test: A laboratory test done on a bone marrow or blood sample to check for mutations in JAK2 , MPL , or CALR genes. A JAK2 gene mutation is often found in patients with polycythemia vera, essential thrombocythemia, or primary myelofibrosis. MPL or CALR gene mutations are found in patients with essential thrombocythemia or primary myelofibrosis.",Chronic Myeloproliferative Neoplasms,0000013_2,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/chronic-treatment-pdq,C0027651,T191,Disorders What are the stages of Chronic Myeloproliferative Neoplasms ?,0000013_2-3,stages,Key Points - There is no standard staging system for chronic myeloproliferative neoplasms. There is no standard staging system for chronic myeloproliferative neoplasms. Staging is the process used to find out how far the cancer has spread. There is no standard staging system for chronic myeloproliferative neoplasms. Treatment is based on the type of myeloproliferative neoplasm the patient has. It is important to know the type in order to plan treatment.,Chronic Myeloproliferative Neoplasms,0000013_2,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/chronic-treatment-pdq,C0027651,T191,Disorders What are the treatments for Chronic Myeloproliferative Neoplasms ?,0000013_2-4,treatment,"Key Points - There are different types of treatment for patients with chronic myeloproliferative neoplasms. - Eleven types of standard treatment are used: - Watchful waiting - Phlebotomy - Platelet apheresis - Transfusion therapy - Chemotherapy - Radiation therapy - Other drug therapy - Surgery - Biologic therapy - Targeted therapy - High-dose chemotherapy with stem cell transplant - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with chronic myeloproliferative neoplasms. Different types of treatments are available for patients with chronic myeloproliferative neoplasms. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Eleven types of standard treatment are used: Watchful waiting Watchful waiting is closely monitoring a patients condition without giving any treatment until signs or symptoms appear or change. Phlebotomy Phlebotomy is a procedure in which blood is taken from a vein. A sample of blood may be taken for tests such as a CBC or blood chemistry. Sometimes phlebotomy is used as a treatment and blood is taken from the body to remove extra red blood cells. Phlebotomy is used in this way to treat some chronic myeloproliferative neoplasms. Platelet apheresis Platelet apheresis is a treatment that uses a special machine to remove platelets from the blood. Blood is taken from the patient and put through a blood cell separator where the platelets are removed. The rest of the blood is then returned to the patients bloodstream. Transfusion therapy Transfusion therapy (blood transfusion) is a method of giving red blood cells, white blood cells, or platelets to replace blood cells destroyed by disease or cancer treatment. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. See Drugs Approved for Myeloproliferative Neoplasms for more information. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type of cancer being treated. External radiation therapy is used to treat chronic myeloproliferative neoplasms, and is usually directed at the spleen. Other drug therapy Prednisone and danazol are drugs that may be used to treat anemia in patients with primary myelofibrosis. Anagrelide therapy is used to reduce the risk of blood clots in patients who have too many platelets in their blood. Low-dose aspirin may also be used to reduce the risk of blood clots. Thalidomide, lenalidomide, and pomalidomide are drugs that prevent blood vessels from growing into areas of tumor cells. See Drugs Approved for Myeloproliferative Neoplasms for more information. Surgery Splenectomy (surgery to remove the spleen) may be done if the spleen is enlarged. Biologic therapy Biologic therapy is a treatment that uses the patient's immune system to fight cancer or other diseases. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against disease. This type of treatment is also called biotherapy or immunotherapy. Interferon alfa and pegylated interferon alpha are biologic agents commonly used to treat some chronic myeloproliferative neoplasms. Erythropoietic growth factors are also biologic agents. They are used to stimulate the bone marrow to make red blood cells. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Tyrosine kinase inhibitors are targeted therapy drugs that block signals needed for tumors to grow. Ruxolitinib is a tyrosine kinase inhibitor used to treat certain types of myelofibrosis. See Drugs Approved for Myeloproliferative Neoplasms for more information. Other types of targeted therapies are being studied in clinical trials. High-dose chemotherapy with stem cell transplant High-dose chemotherapy with stem cell transplant is a method of giving high doses of chemotherapy and replacing blood-forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Chronic Myeloproliferative Neoplasms Chronic Myelogenous Leukemia See the PDQ summary about Chronic Myelogenous Leukemia Treatment for information. Polycythemia Vera The purpose of treatment for polycythemia vera is to reduce the number of extra blood cells. Treatment of polycythemia vera may include the following: - Phlebotomy. - Chemotherapy with or without phlebotomy. - Biologic therapy using interferon alfa or pegylated interferon alpha. - Low-dose aspirin. Check the list of NCI-supported cancer clinical trials that are now accepting patients with polycythemia vera. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Primary Myelofibrosis Treatment of primary myelofibrosis in patients without signs or symptoms is usually watchful waiting. Patients with primary myelofibrosis may have signs or symptoms of anemia. Anemia is usually treated with transfusion of red blood cells to relieve symptoms and improve quality of life. In addition, anemia may be treated with: - Erythropoietic growth factors. - Prednisone. - Danazol. - Thalidomide, lenalidomide, or pomalidomide, with or without prednisone. Treatment of primary myelofibrosis in patients with other signs or symptoms may include the following: - Targeted therapy with ruxolitinib. - Chemotherapy. - Donor stem cell transplant. - Thalidomide, lenalidomide, or pomalidomide. - Splenectomy. - Radiation therapy to the spleen, lymph nodes, or other areas outside the bone marrow where blood cells are forming. - Biologic therapy using interferon alfa or erythropoietic growth factors. - A clinical trial of other targeted therapy drugs. Check the list of NCI-supported cancer clinical trials that are now accepting patients with primary myelofibrosis. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Essential Thrombocythemia Treatment of essential thrombocythemia in patients younger than 60 years who have no signs or symptoms and an acceptable platelet count is usually watchful waiting. Treatment of other patients may include the following: - Chemotherapy. - Anagrelide therapy. - Biologic therapy using interferon alfa or pegylated interferon alpha. - Platelet apheresis. - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with essential thrombocythemia. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Chronic Neutrophilic Leukemia Treatment of chronic neutrophilic leukemia may include the following: - Donor bone marrow transplant. - Chemotherapy. - Biologic therapy using interferon alfa. - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with chronic neutrophilic leukemia. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Chronic Eosinophilic Leukemia Treatment of chronic eosinophilic leukemia may include the following: - Bone marrow transplant. - Biologic therapy using interferon alfa. - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with chronic eosinophilic leukemia. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Chronic Myeloproliferative Neoplasms,0000013_2,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/chronic-treatment-pdq,C0027651,T191,Disorders What is (are) Polycythemia Vera ?,0000013_2-1,information,"Key Points - Polycythemia vera is a disease in which too many red blood cells are made in the bone marrow. - Symptoms of polycythemia vera include headaches and a feeling of fullness below the ribs on the left side. - Special blood tests are used to diagnose polycythemia vera. Polycythemia vera is a disease in which too many red blood cells are made in the bone marrow. In polycythemia vera, the blood becomes thickened with too many red blood cells. The number of white blood cells and platelets may also increase. These extra blood cells may collect in the spleen and cause it to swell. The increased number of red blood cells, white blood cells, or platelets in the blood can cause bleeding problems and make clots form in blood vessels. This can increase the risk of stroke or heart attack. In patients who are older than 65 years or who have a history of blood clots, the risk of stroke or heart attack is higher. Patients also have an increased risk of acute myeloid leukemia or primary myelofibrosis.",Polycythemia Vera,0000013_2,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/chronic-treatment-pdq,C0032463,T191,Disorders What are the symptoms of Polycythemia Vera ?,0000013_2-2,symptoms,"Symptoms of polycythemia vera include headaches and a feeling of fullness below the ribs on the left side. Polycythemia vera often does not cause early signs or symptoms. It may be found during a routine blood test. Signs and symptoms may occur as the number of blood cells increases. Other conditions may cause the same signs and symptoms. Check with your doctor if you have any of the following: - A feeling of pressure or fullness below the ribs on the left side. - Headaches. - Double vision or seeing dark or blind spots that come and go. - Itching all over the body, especially after being in warm or hot water. - Reddened face that looks like a blush or sunburn. - Weakness. - Dizziness. - Weight loss for no known reason.",Polycythemia Vera,0000013_2,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/chronic-treatment-pdq,C0032463,T191,Disorders How to diagnose Polycythemia Vera ?,0000013_2-3,exams and tests,"Special blood tests are used to diagnose polycythemia vera. In addition to a complete blood count, bone marrow aspiration and biopsy, and cytogenetic analysis, a serum erythropoietin test is used to diagnose polycythemia vera. In this test, a sample of blood is checked for the level of erythropoietin (a hormone that stimulates new red blood cells to be made). In polycythemia vera, the erythropoietin level would be lower than normal because the body does not need to make more red blood cells.",Polycythemia Vera,0000013_2,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/chronic-treatment-pdq,C0032463,T191,Disorders What are the treatments for Polycythemia Vera ?,0000013_2-4,treatment,"The purpose of treatment for polycythemia vera is to reduce the number of extra blood cells. Treatment of polycythemia vera may include the following: - Phlebotomy. - Chemotherapy with or without phlebotomy. - Biologic therapy using interferon alfa or pegylated interferon alpha. - Low-dose aspirin. Check the list of NCI-supported cancer clinical trials that are now accepting patients with polycythemia vera. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Polycythemia Vera,0000013_2,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/chronic-treatment-pdq,C0032463,T191,Disorders What is (are) Primary Myelofibrosis ?,0000013_2-1,information,"Key Points - Primary myelofibrosis is a disease in which abnormal blood cells and fibers build up inside the bone marrow. - Symptoms of primary myelofibrosis include pain below the ribs on the left side and feeling very tired. - Certain factors affect prognosis (chance of recovery) and treatment options for primary myelofibrosis. Primary myelofibrosis is a disease in which abnormal blood cells and fibers build up inside the bone marrow. The bone marrow is made of tissues that make blood cells (red blood cells, white blood cells, and platelets) and a web of fibers that support the blood-forming tissues. In primary myelofibrosis (also called chronic idiopathic myelofibrosis), large numbers of blood stem cells become blood cells that do not mature properly (blasts). The web of fibers inside the bone marrow also becomes very thick (like scar tissue) and slows the blood-forming tissues ability to make blood cells. This causes the blood-forming tissues to make fewer and fewer blood cells. In order to make up for the low number of blood cells made in the bone marrow, the liver and spleen begin to make the blood cells.",Primary Myelofibrosis,0000013_2,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/chronic-treatment-pdq,C0001815,T191,Disorders What are the symptoms of Primary Myelofibrosis ?,0000013_2-2,symptoms,"Symptoms of primary myelofibrosis include pain below the ribs on the left side and feeling very tired. Primary myelofibrosis often does not cause early signs or symptoms. It may be found during a routine blood test. Signs and symptoms may be caused by primary myelofibrosis or by other conditions. Check with your doctor if you have any of the following: - Feeling pain or fullness below the ribs on the left side. - Feeling full sooner than normal when eating. - Feeling very tired. - Shortness of breath. - Easy bruising or bleeding. - Petechiae (flat, red, pinpoint spots under the skin that are caused by bleeding). - Fever. - Night sweats. - Weight loss.",Primary Myelofibrosis,0000013_2,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/chronic-treatment-pdq,C0001815,T191,Disorders What is the outlook for Primary Myelofibrosis ?,0000013_2-3,outlook,"Certain factors affect prognosis (chance of recovery) and treatment options for primary myelofibrosis. Prognosis (chance of recovery) depends on the following: - The age of the patient. - The number of abnormal red blood cells and white blood cells. - The number of blasts in the blood. - Whether there are certain changes in the chromosomes. - Whether the patient has signs such as fever, night sweats, or weight loss.",Primary Myelofibrosis,0000013_2,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/chronic-treatment-pdq,C0001815,T191,Disorders What are the treatments for Primary Myelofibrosis ?,0000013_2-4,treatment,"Treatment of primary myelofibrosis in patients without signs or symptoms is usually watchful waiting. Patients with primary myelofibrosis may have signs or symptoms of anemia. Anemia is usually treated with transfusion of red blood cells to relieve symptoms and improve quality of life. In addition, anemia may be treated with: - Erythropoietic growth factors. - Prednisone. - Danazol. - Thalidomide, lenalidomide, or pomalidomide, with or without prednisone. Treatment of primary myelofibrosis in patients with other signs or symptoms may include the following: - Targeted therapy with ruxolitinib. - Chemotherapy. - Donor stem cell transplant. - Thalidomide, lenalidomide, or pomalidomide. - Splenectomy. - Radiation therapy to the spleen, lymph nodes, or other areas outside the bone marrow where blood cells are forming. - Biologic therapy using interferon alfa or erythropoietic growth factors. - A clinical trial of other targeted therapy drugs. Check the list of NCI-supported cancer clinical trials that are now accepting patients with primary myelofibrosis. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Primary Myelofibrosis,0000013_2,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/chronic-treatment-pdq,C0001815,T191,Disorders What is (are) Essential Thrombocythemia ?,0000013_2-1,information,Key Points - Essential thrombocythemia is a disease in which too many platelets are made in the bone marrow. - Patients with essential thrombocythemia may have no signs or symptoms. - Certain factors affect prognosis (chance of recovery) and treatment options for essential thrombocythemia. Essential thrombocythemia is a disease in which too many platelets are made in the bone marrow. Essential thrombocythemia causes an abnormal increase in the number of platelets made in the blood and bone marrow.,Essential Thrombocythemia,0000013_2,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/chronic-treatment-pdq,C0040028,T047,Disorders What are the symptoms of Essential Thrombocythemia ?,0000013_2-2,symptoms,"Patients with essential thrombocythemia may have no signs or symptoms. Essential thrombocythemia often does not cause early signs or symptoms. It may be found during a routine blood test. Signs and symptoms may be caused by essential thrombocytopenia or by other conditions. Check with your doctor if you have any of the following: - Headache. - Burning or tingling in the hands or feet. - Redness and warmth of the hands or feet. - Vision or hearing problems. Platelets are sticky. When there are too many platelets, they may clump together and make it hard for the blood to flow. Clots may form in blood vessels and there may also be increased bleeding. These can cause serious health problems such as stroke or heart attack.",Essential Thrombocythemia,0000013_2,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/chronic-treatment-pdq,C0040028,T047,Disorders What is the outlook for Essential Thrombocythemia ?,0000013_2-3,outlook,Certain factors affect prognosis (chance of recovery) and treatment options for essential thrombocythemia. Prognosis (chance of recovery) and treatment options depend on the following: - The age of the patient. - Whether the patient has signs or symptoms or other problems related to essential thrombocythemia.,Essential Thrombocythemia,0000013_2,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/chronic-treatment-pdq,C0040028,T047,Disorders What are the treatments for Essential Thrombocythemia ?,0000013_2-4,treatment,"Treatment of essential thrombocythemia in patients younger than 60 years who have no signs or symptoms and an acceptable platelet count is usually watchful waiting. Treatment of other patients may include the following: - Chemotherapy. - Anagrelide therapy. - Biologic therapy using interferon alfa or pegylated interferon alpha. - Platelet apheresis. - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with essential thrombocythemia. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Essential Thrombocythemia,0000013_2,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/chronic-treatment-pdq,C0040028,T047,Disorders What is (are) Chronic Neutrophilic Leukemia ?,0000013_2-1,information,Chronic neutrophilic leukemia is a disease in which too many blood stem cells become a type of white blood cell called neutrophils. Neutrophils are infection -fighting blood cells that surround and destroy dead cells and foreign substances (such as bacteria). The spleen and liver may swell because of the extra neutrophils. Chronic neutrophilic leukemia may stay the same or it may progress quickly to acute leukemia.,Chronic Neutrophilic Leukemia,0000013_2,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/chronic-treatment-pdq,C0023481,T191,Disorders What are the treatments for Chronic Neutrophilic Leukemia ?,0000013_2-2,treatment,"Treatment of chronic neutrophilic leukemia may include the following: - Donor bone marrow transplant. - Chemotherapy. - Biologic therapy using interferon alfa. - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with chronic neutrophilic leukemia. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Chronic Neutrophilic Leukemia,0000013_2,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/chronic-treatment-pdq,C0023481,T191,Disorders What is (are) Chronic Eosinophilic Leukemia ?,0000013_2-1,information,"Key Points - Chronic eosinophilic leukemia is a disease in which too many white blood cells (eosinophils) are made in the bone marrow. - Signs and symptoms of chronic eosinophilic leukemia include fever and feeling very tired. Chronic eosinophilic leukemia is a disease in which too many white blood cells (eosinophils) are made in the bone marrow. Eosinophils are white blood cells that react to allergens (substances that cause an allergic response) and help fight infections caused by certain parasites. In chronic eosinophilic leukemia, there are too many eosinophils in the blood, bone marrow, and other tissues. Chronic eosinophilic leukemia may stay the same for many years or it may progress quickly to acute leukemia.",Chronic Eosinophilic Leukemia,0000013_2,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/chronic-treatment-pdq,C0346421,T191,Disorders What are the symptoms of Chronic Eosinophilic Leukemia ?,0000013_2-2,symptoms,"Signs and symptoms of chronic eosinophilic leukemia include fever and feeling very tired. Chronic eosinophilic leukemia may not cause early signs or symptoms. It may be found during a routine blood test. Signs and symptoms may be caused by chronic eosinophilic leukemia or by other conditions. Check with your doctor if you have any of the following: - Fever. - Feeling very tired. - Cough. - Swelling under the skin around the eyes and lips, in the throat, or on the hands and feet. - Muscle pain. - Itching. - Diarrhea.",Chronic Eosinophilic Leukemia,0000013_2,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/chronic-treatment-pdq,C0346421,T191,Disorders What are the treatments for Chronic Eosinophilic Leukemia ?,0000013_2-3,treatment,"Treatment of chronic eosinophilic leukemia may include the following: - Bone marrow transplant. - Biologic therapy using interferon alfa. - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with chronic eosinophilic leukemia. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Chronic Eosinophilic Leukemia,0000013_2,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/chronic-treatment-pdq,C0346421,T191,Disorders What is (are) Chronic Myelogenous Leukemia ?,0000013_2-1,information,"Chronic myelogenous leukemia is a disease in which too many white blood cells are made in the bone marrow. See the PDQ summary on Chronic Myelogenous Leukemia Treatment for information on diagnosis, staging, and treatment.",Chronic Myelogenous Leukemia,0000013_2,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/chronic-treatment-pdq,C0023473,T191,Disorders What are the treatments for Chronic Myelogenous Leukemia ?,0000013_2-2,treatment,See the PDQ summary about Chronic Myelogenous Leukemia Treatment for information.,Chronic Myelogenous Leukemia,0000013_2,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/chronic-treatment-pdq,C0023473,T191,Disorders What is (are) Myelodysplastic/ Myeloproliferative Neoplasms ?,0000013_3-1,information,"Key Points - Myelodysplastic/myeloproliferative neoplasms are a group of diseases in which the bone marrow makes too many white blood cells. - Myelodysplastic/myeloproliferative neoplasms have features of both myelodysplastic syndromes and myeloproliferative neoplasms. - There are different types of myelodysplastic/myeloproliferative neoplasms. - Tests that examine the blood and bone marrow are used to detect (find) and diagnose myelodysplastic/myeloproliferative neoplasms. Myelodysplastic/myeloproliferative neoplasms are a group of diseases in which the bone marrow makes too many white blood cells. Myelodysplastic /myeloproliferative neoplasms are diseases of the blood and bone marrow. Normally, the bone marrow makes blood stem cells (immature cells) that become mature blood cells over time. A blood stem cell may become a myeloid stem cell or a lymphoid stem cell. A lymphoid stem cell becomes a white blood cell. A myeloid stem cell becomes one of three types of mature blood cells: - Red blood cells that carry oxygen and other substances to all tissues of the body. - White blood cells that fight infection and disease. - Platelets that form blood clots to stop bleeding. Myelodysplastic/myeloproliferative neoplasms have features of both myelodysplastic syndromes and myeloproliferative neoplasms. In myelodysplastic diseases, the blood stem cells do not mature into healthy red blood cells, white blood cells, or platelets. The immature blood cells, called blasts, do not work the way they should and die in the bone marrow or soon after they enter the blood. As a result, there are fewer healthy red blood cells, white blood cells, and platelets. In myeloproliferative diseases, a greater than normal number of blood stem cells become one or more types of blood cells and the total number of blood cells slowly increases. This summary is about neoplasms that have features of both myelodysplastic and myeloproliferative diseases. See the following PDQ summaries for more information about related diseases: - Myelodysplastic Syndromes Treatment - Chronic Myeloproliferative Neoplasms Treatment - Chronic Myelogenous Leukemia Treatment There are different types of myelodysplastic/myeloproliferative neoplasms. The 3 main types of myelodysplastic/myeloproliferative neoplasms include the following: - Chronic myelomonocytic leukemia (CMML). - Juvenile myelomonocytic leukemia (JMML). - Atypical chronic myelogenous leukemia (CML). When a myelodysplastic/myeloproliferative neoplasm does not match any of these types, it is called myelodysplastic/myeloproliferative neoplasm, unclassifiable (MDS/MPN-UC). Myelodysplastic/myeloproliferative neoplasms may progress to acute leukemia.",Myelodysplastic/ Myeloproliferative Neoplasms,0000013_3,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/mds-mpd-treatment-pdq,C0027651,T191,Disorders How to diagnose Myelodysplastic/ Myeloproliferative Neoplasms ?,0000013_3-2,exams and tests,"Tests that examine the blood and bone marrow are used to detect (find) and diagnose myelodysplastic/myeloproliferative neoplasms. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease such as an enlarged spleen and liver. A history of the patients health habits and past illnesses and treatments will also be taken. - Complete blood count (CBC) with differential : A procedure in which a sample of blood is drawn and checked for the following: - The number of red blood cells and platelets. - The number and type of white blood cells. - The amount of hemoglobin (the protein that carries oxygen) in the red blood cells. - The portion of the sample made up of red blood cells. - Peripheral blood smear : A procedure in which a sample of blood is checked for blast cells, the number and kinds of white blood cells, the number of platelets, and changes in the shape of blood cells. - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. - Bone marrow aspiration and biopsy : The removal of a small piece of bone and bone marrow by inserting a needle into the hipbone or breastbone. A pathologist views both the bone and bone marrow samples under a microscope to look for abnormal cells. The following tests may be done on the sample of tissue that is removed: - Cytogenetic analysis : A test in which cells in a sample of blood or bone marrow are viewed under a microscope to look for certain changes in the chromosomes. The cancer cells in myelodysplastic/myeloproliferative neoplasms do not contain the Philadelphia chromosome that is present in chronic myelogenous leukemia. - Immunocytochemistry : A test that uses antibodies to check for certain antigens in a sample of bone marrow. The antibody is usually linked to a radioactive substance or a dye that causes the cells in the sample to light up under a microscope. This type of test is used to tell the difference between myelodysplastic/myeloproliferative neoplasms, leukemia, and other conditions.",Myelodysplastic/ Myeloproliferative Neoplasms,0000013_3,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/mds-mpd-treatment-pdq,C0027651,T191,Disorders What are the stages of Myelodysplastic/ Myeloproliferative Neoplasms ?,0000013_3-3,stages,Key Points - There is no standard staging system for myelodysplastic/myeloproliferative neoplasms. There is no standard staging system for myelodysplastic/myeloproliferative neoplasms. Staging is the process used to find out how far the cancer has spread. There is no standard staging system for myelodysplastic /myeloproliferative neoplasms. Treatment is based on the type of myelodysplastic/myeloproliferative neoplasm the patient has. It is important to know the type in order to plan treatment.,Myelodysplastic/ Myeloproliferative Neoplasms,0000013_3,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/mds-mpd-treatment-pdq,C0027651,T191,Disorders What are the treatments for Myelodysplastic/ Myeloproliferative Neoplasms ?,0000013_3-4,treatment,"Key Points - There are different types of treatment for patients with myelodysplastic/myeloproliferative neoplasms. - Five types of standard treatment are used: - Chemotherapy - Other drug therapy - Stem cell transplant - Supportive care - Targeted therapy - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with myelodysplastic/myeloproliferative neoplasms. Different types of treatments are available for patients with myelodysplastic /myeloproliferative neoplasms. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Five types of standard treatment are used: Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. Combination chemotherapy is treatment using more than one anticancer drug. See Drugs Approved for Myeloproliferative Neoplasms for more information. Other drug therapy 13-cis retinoic acid is a vitamin -like drug that slows the cancer's ability to make more cancer cells and changes the way these cells look and act. Stem cell transplant Stem cell transplant is a method of replacing blood -forming cells that are destroyed by chemotherapy. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. Supportive care Supportive care is given to lessen the problems caused by the disease or its treatment. Supportive care may include transfusion therapy or drug therapy, such as antibiotics to fight infection. Targeted therapy Targeted therapy is a cancer treatment that uses drugs or other substances to attack cancer cells without harming normal cells. Targeted therapy drugs called tyrosine kinase inhibitors (TKIs) are used to treat myelodysplastic/myeloproliferative neoplasm, unclassifiable. TKIs block the enzyme, tyrosine kinase, that causes stem cells to become more blood cells (blasts) than the body needs. Imatinib mesylate (Gleevec) is a TKI that may be used. Other targeted therapy drugs are being studied in the treatment of JMML. See Drugs Approved for Myeloproliferative Neoplasms for more information. New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Myelodysplastic/ Myeloproliferative Neoplasms Chronic Myelomonocytic Leukemia Treatment of chronic myelomonocytic leukemia (CMML) may include the following: - Chemotherapy with one or more agents. - Stem cell transplant. - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with chronic myelomonocytic leukemia. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Juvenile Myelomonocytic Leukemia Treatment of juvenile myelomonocytic leukemia (JMML) may include the following: - Combination chemotherapy. - Stem cell transplant. - 13-cis-retinoic acid therapy. - A clinical trial of a new treatment, such as targeted therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with juvenile myelomonocytic leukemia. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Atypical Chronic Myelogenous Leukemia Treatment of atypical chronic myelogenous leukemia (CML) may include chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with atypical chronic myeloid leukemia, BCR-ABL1 negative. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Myelodysplastic/ Myeloproliferative Neoplasm, Unclassifiable Because myelodysplastic /myeloproliferative neoplasm, unclassifiable (MDS/MPN-UC) is a rare disease, little is known about its treatment. Treatment may include the following: - Supportive care treatments to manage problems caused by the disease such as infection, bleeding, and anemia. - Targeted therapy (imatinib mesylate). Check the list of NCI-supported cancer clinical trials that are now accepting patients with myelodysplastic/myeloproliferative neoplasm, unclassifiable. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Myelodysplastic/ Myeloproliferative Neoplasms,0000013_3,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/mds-mpd-treatment-pdq,C0027651,T191,Disorders What is (are) Chronic Myelomonocytic Leukemia ?,0000013_3-1,information,"Key Points - Chronic myelomonocytic leukemia is a disease in which too many myelocytes and monocytes (immature white blood cells) are made in the bone marrow. - Older age and being male increase the risk of chronic myelomonocytic leukemia. - Signs and symptoms of chronic myelomonocytic leukemia include fever, weight loss, and feeling very tired. - Certain factors affect prognosis (chance of recovery) and treatment options. Chronic myelomonocytic leukemia is a disease in which too many myelocytes and monocytes (immature white blood cells) are made in the bone marrow. In chronic myelomonocytic leukemia (CMML), the body tells too many blood stem cells to become two types of white blood cells called myelocytes and monocytes. Some of these blood stem cells never become mature white blood cells. These immature white blood cells are called blasts. Over time, the myelocytes, monocytes, and blasts crowd out the red blood cells and platelets in the bone marrow. When this happens, infection, anemia, or easy bleeding may occur.",Chronic Myelomonocytic Leukemia,0000013_3,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/mds-mpd-treatment-pdq,C0023480,T191,Disorders Who is at risk for Chronic Myelomonocytic Leukemia? ?,0000013_3-2,susceptibility,Older age and being male increase the risk of chronic myelomonocytic leukemia. Anything that increases your chance of getting a disease is called a risk factor. Possible risk factors for CMML include the following: - Older age. - Being male. - Being exposed to certain substances at work or in the environment. - Being exposed to radiation. - Past treatment with certain anticancer drugs.,Chronic Myelomonocytic Leukemia,0000013_3,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/mds-mpd-treatment-pdq,C0023480,T191,Disorders What are the symptoms of Chronic Myelomonocytic Leukemia ?,0000013_3-3,symptoms,"Signs and symptoms of chronic myelomonocytic leukemia include fever, weight loss, and feeling very tired. These and other signs and symptoms may be caused by CMML or by other conditions. Check with your doctor if you have any of the following: - Fever for no known reason. - Infection. - Feeling very tired. - Weight loss for no known reason. - Easy bruising or bleeding. - Pain or a feeling of fullness below the ribs.",Chronic Myelomonocytic Leukemia,0000013_3,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/mds-mpd-treatment-pdq,C0023480,T191,Disorders What is the outlook for Chronic Myelomonocytic Leukemia ?,0000013_3-4,outlook,Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options for CMML depend on the following: - The number of white blood cells or platelets in the blood or bone marrow. - Whether the patient is anemic. - The amount of blasts in the blood or bone marrow. - The amount of hemoglobin in red blood cells. - Whether there are certain changes in the chromosomes.,Chronic Myelomonocytic Leukemia,0000013_3,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/mds-mpd-treatment-pdq,C0023480,T191,Disorders What are the treatments for Chronic Myelomonocytic Leukemia ?,0000013_3-5,treatment,"Treatment of chronic myelomonocytic leukemia (CMML) may include the following: - Chemotherapy with one or more agents. - Stem cell transplant. - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with chronic myelomonocytic leukemia. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Chronic Myelomonocytic Leukemia,0000013_3,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/mds-mpd-treatment-pdq,C0023480,T191,Disorders What is (are) Juvenile Myelomonocytic Leukemia ?,0000013_3-1,information,"Key Points - Juvenile myelomonocytic leukemia is a childhood disease in which too many myelocytes and monocytes (immature white blood cells) are made in the bone marrow. - Signs and symptoms of juvenile myelomonocytic leukemia include fever, weight loss, and feeling very tired. - Certain factors affect prognosis (chance of recovery) and treatment options. Juvenile myelomonocytic leukemia is a childhood disease in which too many myelocytes and monocytes (immature white blood cells) are made in the bone marrow. Juvenile myelomonocytic leukemia (JMML) is a rare childhood cancer that occurs more often in children younger than 2 years. Children who have neurofibromatosis type 1 and males have an increased risk of juvenile myelomonocytic leukemia. In JMML, the body tells too many blood stem cells to become two types of white blood cells called myelocytes and monocytes. Some of these blood stem cells never become mature white blood cells. These immature white blood cells are called blasts. Over time, the myelocytes, monocytes, and blasts crowd out the red blood cells and platelets in the bone marrow. When this happens, infection, anemia, or easy bleeding may occur.",Juvenile Myelomonocytic Leukemia,0000013_3,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/mds-mpd-treatment-pdq,C0349639,T191,Disorders What are the symptoms of Juvenile Myelomonocytic Leukemia ?,0000013_3-2,symptoms,"Signs and symptoms of juvenile myelomonocytic leukemia include fever, weight loss, and feeling very tired. These and other signs and symptoms may be caused by JMML or by other conditions. Check with your doctor if you have any of the following: - Fever for no known reason. - Having infections, such as bronchitis or tonsillitis. - Feeling very tired. - Easy bruising or bleeding. - Skin rash. - Painless swelling of the lymph nodes in the neck, underarm, stomach, or groin. - Pain or a feeling of fullness below the ribs.",Juvenile Myelomonocytic Leukemia,0000013_3,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/mds-mpd-treatment-pdq,C0349639,T191,Disorders What is the outlook for Juvenile Myelomonocytic Leukemia ?,0000013_3-3,outlook,Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options for JMML depend on the following: - The age of the child at diagnosis. - The number of platelets in the blood. - The amount of a certain type of hemoglobin in red blood cells.,Juvenile Myelomonocytic Leukemia,0000013_3,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/mds-mpd-treatment-pdq,C0349639,T191,Disorders What are the treatments for Juvenile Myelomonocytic Leukemia ?,0000013_3-4,treatment,"Treatment of juvenile myelomonocytic leukemia (JMML) may include the following: - Combination chemotherapy. - Stem cell transplant. - 13-cis-retinoic acid therapy. - A clinical trial of a new treatment, such as targeted therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with juvenile myelomonocytic leukemia. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Juvenile Myelomonocytic Leukemia,0000013_3,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/mds-mpd-treatment-pdq,C0349639,T191,Disorders What is (are) Atypical Chronic Myelogenous Leukemia ?,0000013_3-1,information,"Key Points - Atypical chronic myelogenous leukemia is a disease in which too many granulocytes (immature white blood cells) are made in the bone marrow. - Signs and symptoms of atypical chronic myelogenous leukemia include easy bruising or bleeding and feeling tired and weak. - Certain factors affect prognosis (chance of recovery). Atypical chronic myelogenous leukemia is a disease in which too many granulocytes (immature white blood cells) are made in the bone marrow. In atypical chronic myelogenous leukemia (CML), the body tells too many blood stem cells to become a type of white blood cell called granulocytes. Some of these blood stem cells never become mature white blood cells. These immature white blood cells are called blasts. Over time, the granulocytes and blasts crowd out the red blood cells and platelets in the bone marrow. The leukemia cells in atypical CML and CML look alike under a microscope. However, in atypical CML a certain chromosome change, called the ""Philadelphia chromosome"" is not there.",Atypical Chronic Myelogenous Leukemia,0000013_3,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/mds-mpd-treatment-pdq,C1292772,T191,Disorders What are the symptoms of Atypical Chronic Myelogenous Leukemia ?,0000013_3-2,symptoms,"Signs and symptoms of atypical chronic myelogenous leukemia include easy bruising or bleeding and feeling tired and weak. These and other signs and symptoms may be caused by atypical CML or by other conditions. Check with your doctor if you have any of the following: - Shortness of breath. - Pale skin. - Feeling very tired and weak. - Easy bruising or bleeding. - Petechiae (flat, pinpoint spots under the skin caused by bleeding). - Pain or a feeling of fullness below the ribs on the left side.",Atypical Chronic Myelogenous Leukemia,0000013_3,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/mds-mpd-treatment-pdq,C1292772,T191,Disorders What is the outlook for Atypical Chronic Myelogenous Leukemia ?,0000013_3-3,outlook,Certain factors affect prognosis (chance of recovery). The prognosis (chance of recovery) for atypical CML depends on the number of red blood cells and platelets in the blood.,Atypical Chronic Myelogenous Leukemia,0000013_3,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/mds-mpd-treatment-pdq,C1292772,T191,Disorders What are the treatments for Atypical Chronic Myelogenous Leukemia ?,0000013_3-4,treatment,"Treatment of atypical chronic myelogenous leukemia (CML) may include chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with atypical chronic myeloid leukemia, BCR-ABL1 negative. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Atypical Chronic Myelogenous Leukemia,0000013_3,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/mds-mpd-treatment-pdq,C1292772,T191,Disorders "What is (are) Myelodysplastic/ Myeloproliferative Neoplasm, Unclassifiable ?",0000013_3-1,information,"Key Points - Myelodysplastic/myeloproliferative neoplasm, unclassifiable, is a disease that has features of both myelodysplastic and myeloproliferative diseases but is not chronic myelomonocytic leukemia, juvenile myelomonocytic leukemia, or atypical chronic myelogenous leukemia. - Signs and symptoms of myelodysplastic/myeloproliferative neoplasm, unclassifiable, include fever, weight loss, and feeling very tired. Myelodysplastic/myeloproliferative neoplasm, unclassifiable, is a disease that has features of both myelodysplastic and myeloproliferative diseases but is not chronic myelomonocytic leukemia, juvenile myelomonocytic leukemia, or atypical chronic myelogenous leukemia. In myelodysplastic /myeloproliferative neoplasm, unclassifiable (MDS/MPD-UC), the body tells too many blood stem cells to become red blood cells, white blood cells, or platelets. Some of these blood stem cells never become mature blood cells. These immature blood cells are called blasts. Over time, the abnormal blood cells and blasts in the bone marrow crowd out the healthy red blood cells, white blood cells, and platelets. MDS/MPN-UC is a very rare disease. Because it is so rare, the factors that affect risk and prognosis are not known.","Myelodysplastic/ Myeloproliferative Neoplasm, Unclassifiable",0000013_3,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/mds-mpd-treatment-pdq,C1333046,T191,Disorders "What are the symptoms of Myelodysplastic/ Myeloproliferative Neoplasm, Unclassifiable ?",0000013_3-2,symptoms,"Signs and symptoms of myelodysplastic/myeloproliferative neoplasm, unclassifiable, include fever, weight loss, and feeling very tired. These and other signs and symptoms may be caused by MDS/MPN-UC or by other conditions. Check with your doctor if you have any of the following: - Fever or frequent infections. - Shortness of breath. - Feeling very tired and weak. - Pale skin. - Easy bruising or bleeding. - Petechiae (flat, pinpoint spots under the skin caused by bleeding). - Pain or a feeling of fullness below the ribs.","Myelodysplastic/ Myeloproliferative Neoplasm, Unclassifiable",0000013_3,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/mds-mpd-treatment-pdq,C1333046,T191,Disorders "What are the treatments for Myelodysplastic/ Myeloproliferative Neoplasm, Unclassifiable ?",0000013_3-3,treatment,"Because myelodysplastic /myeloproliferative neoplasm, unclassifiable (MDS/MPN-UC) is a rare disease, little is known about its treatment. Treatment may include the following: - Supportive care treatments to manage problems caused by the disease such as infection, bleeding, and anemia. - Targeted therapy (imatinib mesylate). Check the list of NCI-supported cancer clinical trials that are now accepting patients with myelodysplastic/myeloproliferative neoplasm, unclassifiable. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.","Myelodysplastic/ Myeloproliferative Neoplasm, Unclassifiable",0000013_3,CancerGov,https://www.cancer.gov/types/myeloproliferative/patient/mds-mpd-treatment-pdq,C1333046,T191,Disorders What is (are) Endometrial Cancer ?,0000014_1-1,information,"Key Points - Endometrial cancer is a disease in which malignant (cancer) cells form in the tissues of the endometrium. - Obesity and having metabolic syndrome may increase the risk of endometrial cancer. - Taking tamoxifen for breast cancer or taking estrogen alone (without progesterone) can increase the risk of endometrial cancer. - Signs and symptoms of endometrial cancer include unusual vaginal bleeding or pain in the pelvis. - Tests that examine the endometrium are used to detect (find) and diagnose endometrial cancer. - Certain factors affect prognosis (chance of recovery) and treatment options. Endometrial cancer is a disease in which malignant (cancer) cells form in the tissues of the endometrium. The endometrium is the lining of the uterus, a hollow, muscular organ in a womans pelvis. The uterus is where a fetus grows. In most nonpregnant women, the uterus is about 3 inches long. The lower, narrow end of the uterus is the cervix, which leads to the vagina. Cancer of the endometrium is different from cancer of the muscle of the uterus, which is called sarcoma of the uterus. See the PDQ summary on Uterine Sarcoma Treatment for more information about uterine sarcoma. Obesity and having metabolic syndrome may increase the risk of endometrial cancer. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for endometrial cancer include the following: - Having endometrial hyperplasia. - Being obese. - Having metabolic syndrome, a set of conditions that occur together, including extra fat around the abdomen, high blood sugar, high blood pressure, high levels of triglycerides and low levels of high-density lipoproteins in the blood. - Never giving birth. - Beginning menstruation at an early age. - Reaching menopause at an older age. - Having polycystic ovarian syndrome (PCOS). - Having a mother, sister, or daughter with uterine cancer. - Having a certain gene change that is linked to Lynch syndrome (hereditary non-polyposis colon cancer). - Having hyperinsulinemia (high levels of insulin in the blood). Taking tamoxifen for breast cancer or taking estrogen alone (without progesterone) can increase the risk of endometrial cancer. Endometrial cancer may develop in breast cancer patients who have been treated with tamoxifen. A patient who takes this drug and has abnormal vaginal bleeding should have a follow-up exam and a biopsy of the endometrial lining if needed. Women taking estrogen (a hormone that can affect the growth of some cancers) alone also have an increased risk of endometrial cancer. Taking estrogen combined with progesterone (another hormone) does not increase a womans risk of endometrial cancer.",Endometrial Cancer,0000014_1,CancerGov,https://www.cancer.gov/types/uterine/patient/endometrial-treatment-pdq,C1883486,T191,Disorders Who is at risk for Endometrial Cancer? ?,0000014_1-2,susceptibility,"Obesity and having metabolic syndrome may increase the risk of endometrial cancer. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for endometrial cancer include the following: - Having endometrial hyperplasia. - Being obese. - Having metabolic syndrome, a set of conditions that occur together, including extra fat around the abdomen, high blood sugar, high blood pressure, high levels of triglycerides and low levels of high-density lipoproteins in the blood. - Never giving birth. - Beginning menstruation at an early age. - Reaching menopause at an older age. - Having polycystic ovarian syndrome (PCOS). - Having a mother, sister, or daughter with uterine cancer. - Having a certain gene change that is linked to Lynch syndrome (hereditary non-polyposis colon cancer). - Having hyperinsulinemia (high levels of insulin in the blood). Taking tamoxifen for breast cancer or taking estrogen alone (without progesterone) can increase the risk of endometrial cancer. Endometrial cancer may develop in breast cancer patients who have been treated with tamoxifen. A patient who takes this drug and has abnormal vaginal bleeding should have a follow-up exam and a biopsy of the endometrial lining if needed. Women taking estrogen (a hormone that can affect the growth of some cancers) alone also have an increased risk of endometrial cancer. Taking estrogen combined with progesterone (another hormone) does not increase a womans risk of endometrial cancer.",Endometrial Cancer,0000014_1,CancerGov,https://www.cancer.gov/types/uterine/patient/endometrial-treatment-pdq,C1883486,T191,Disorders What are the symptoms of Endometrial Cancer ?,0000014_1-3,symptoms,Signs and symptoms of endometrial cancer include unusual vaginal bleeding or pain in the pelvis. These and other signs and symptoms may be caused by endometrial cancer or by other conditions. Check with your doctor if you have any of the following: - Vaginal bleeding or discharge not related to menstruation (periods). - Vaginal bleeding after menopause. - Difficult or painful urination. - Pain during sexual intercourse. - Pain in the pelvic area.,Endometrial Cancer,0000014_1,CancerGov,https://www.cancer.gov/types/uterine/patient/endometrial-treatment-pdq,C1883486,T191,Disorders How to diagnose Endometrial Cancer ?,0000014_1-4,exams and tests,"Tests that examine the endometrium are used to detect (find) and diagnose endometrial cancer. Because endometrial cancer begins inside the uterus, it does not usually show up in the results of a Pap test. For this reason, a sample of endometrial tissue must be removed and checked under a microscope to look for cancer cells. One of the following procedures may be used: - Endometrial biopsy : The removal of tissue from the endometrium (inner lining of the uterus) by inserting a thin, flexible tube through the cervix and into the uterus. The tube is used to gently scrape a small amount of tissue from the endometrium and then remove the tissue samples. A pathologist views the tissue under a microscope to look for cancer cells. - Dilatation and curettage : A procedure to remove samples of tissue from the inner lining of the uterus. The cervix is dilated and a curette (spoon-shaped instrument) is inserted into the uterus to remove tissue. The tissue samples are checked under a microscope for signs of disease. This procedure is also called a D&C. - Hysteroscopy: A procedure to look inside the uterus for abnormal areas. A hysteroscope is inserted through the vagina and cervix into the uterus. A hysteroscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer. Other tests and procedures used to diagnose endometrial cancer include the following: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Transvaginal ultrasound exam: A procedure used to examine the vagina, uterus, fallopian tubes, and bladder. An ultrasound transducer (probe) is inserted into the vagina and used to bounce high-energy sound waves (ultrasound) off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The doctor can identify tumors by looking at the sonogram.",Endometrial Cancer,0000014_1,CancerGov,https://www.cancer.gov/types/uterine/patient/endometrial-treatment-pdq,C1883486,T191,Disorders What is the outlook for Endometrial Cancer ?,0000014_1-5,outlook,"Certain factors affect prognosis (chance of recovery) and treatment options.The prognosis (chance of recovery) and treatment options depend on the following: - The stage of the cancer (whether it is in the endometrium only, involves the uterus wall, or has spread to other places in the body). - How the cancer cells look under a microscope. - Whether the cancer cells are affected by progesterone. Endometrial cancer can usually be cured because it is usually diagnosed early.",Endometrial Cancer,0000014_1,CancerGov,https://www.cancer.gov/types/uterine/patient/endometrial-treatment-pdq,C1883486,T191,Disorders What are the stages of Endometrial Cancer ?,0000014_1-6,stages,"Key Points - After endometrial cancer has been diagnosed, tests are done to find out if cancer cells have spread within the uterus or to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - The following stages are used for endometrial cancer: - Stage I - Stage II - Stage III - Stage IV - Endometrial cancer may be grouped for treatment as follows: - Low-risk endometrial cancer - High-risk endometrial cancer After endometrial cancer has been diagnosed, tests are done to find out if cancer cells have spread within the uterus or to other parts of the body. The process used to find out whether the cancer has spread within the uterus or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. Certain tests and procedures are used in the staging process. A hysterectomy (an operation in which the uterus is removed) will usually be done to treat endometrial cancer. Tissue samples are taken from the area around the uterus and checked under a microscope for signs of cancer to help find out whether the cancer has spread. The following procedures may be used in the staging process: - Pelvic exam : An exam of the vagina, cervix, uterus, fallopian tubes, ovaries, and rectum. A speculum is inserted into the vagina and the doctor or nurse looks at the vagina and cervix for signs of disease. A Pap test of the cervix is usually done. The doctor or nurse also inserts one or two lubricated, gloved fingers of one hand into the vagina and places the other hand over the lower abdomen to feel the size, shape, and position of the uterus and ovaries. The doctor or nurse also inserts a lubricated, gloved finger into the rectum to feel for lumps or abnormal areas. - Chest x-ray : An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. - Lymph node dissection : A surgical procedure in which the lymph nodes are removed from the pelvic area and a sample of tissue is checked under a microscope for signs of cancer. This procedure is also called lymphadenectomy. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if endometrial cancer spreads to the lung, the cancer cells in the lung are actually endometrial cancer cells. The disease is metastatic endometrial cancer, not lung cancer. The following stages are used for endometrial cancer: Stage I In stage I, cancer is found in the uterus only. Stage I is divided into stages IA and IB, based on how far the cancer has spread. - Stage IA: Cancer is in the endometrium only or less than halfway through the myometrium (muscle layer of the uterus). - Stage IB: Cancer has spread halfway or more into the myometrium. Stage II In stage II, cancer has spread into connective tissue of the cervix, but has not spread outside the uterus. Stage III In stage III, cancer has spread beyond the uterus and cervix, but has not spread beyond the pelvis. Stage III is divided into stages IIIA, IIIB, and IIIC, based on how far the cancer has spread within the pelvis. - Stage IIIA: Cancer has spread to the outer layer of the uterus and/or to the fallopian tubes, ovaries, and ligaments of the uterus. - Stage IIIB: Cancer has spread to the vagina and/or to the parametrium (connective tissue and fat around the uterus). - Stage IIIC: Cancer has spread to lymph nodes in the pelvis and/or around the aorta (largest artery in the body, which carries blood away from the heart). Stage IV In stage IV, cancer has spread beyond the pelvis. Stage IV is divided into stages IVA and IVB, based on how far the cancer has spread. - Stage IVA: Cancer has spread to the bladder and/or bowel wall. - Stage IVB: Cancer has spread to other parts of the body beyond the pelvis, including the abdomen and/or lymph nodes in the groin. Endometrial cancer may be grouped for treatment as follows: Low-risk endometrial cancer Grades 1 and 2 tumors are usually considered low-risk. They usually do not spread to other parts of the body. High-risk endometrial cancer Grade 3 tumors are considered high-risk. They often spread to other parts of the body. Uterine papillary serous, clear cell, and carcinosarcoma are three subtypes of endometrial cancer that are considered grade 3.",Endometrial Cancer,0000014_1,CancerGov,https://www.cancer.gov/types/uterine/patient/endometrial-treatment-pdq,C1883486,T191,Disorders What are the treatments for Endometrial Cancer ?,0000014_1-7,treatment,"Key Points - There are different types of treatment for patients with endometrial cancer. - Five types of standard treatment are used: - Surgery - Radiation therapy - Chemotherapy - Hormone therapy - Targeted therapy - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with endometrial cancer. Different types of treatment are available for patients with endometrial cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Five types of standard treatment are used: Surgery Surgery (removing the cancer in an operation) is the most common treatment for endometrial cancer. The following surgical procedures may be used: - Total hysterectomy: Surgery to remove the uterus, including the cervix. If the uterus and cervix are taken out through the vagina, the operation is called a vaginal hysterectomy. If the uterus and cervix are taken out through a large incision (cut) in the abdomen, the operation is called a total abdominal hysterectomy. If the uterus and cervix are taken out through a small incision (cut) in the abdomen using a laparoscope, the operation is called a total laparoscopic hysterectomy. - Bilateral salpingo-oophorectomy: Surgery to remove both ovaries and both fallopian tubes. - Radical hysterectomy: Surgery to remove the uterus, cervix, and part of the vagina. The ovaries, fallopian tubes, or nearby lymph nodes may also be removed. - Lymph node dissection: A surgical procedure in which the lymph nodes are removed from the pelvic area and a sample of tissue is checked under a microscope for signs of cancer. This procedure is also called lymphadenectomy. Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given radiation therapy or hormone treatment after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External and internal radiation therapy are used to treat endometrial cancer, and may also be used as palliative therapy to relieve symptoms and improve quality of life. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. Hormone therapy Hormone therapy is a cancer treatment that removes hormones or blocks their action and stops cancer cells from growing. Hormones are substances made by glands in the body and circulated in the bloodstream. Some hormones can cause certain cancers to grow. If tests show that the cancer cells have places where hormones can attach (receptors), drugs, surgery, or radiation therapy is used to reduce the production of hormones or block them from working. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Monoclonal antibodies, mTOR inhibitors, and signal transduction inhibitors are three types of targeted therapy used to treat endometrial cancer. - Monoclonal antibody therapy is a cancer treatment that uses antibodies made in the laboratory from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Bevacizumab is used to treat stage III, stage IV, and recurrent endometrial cancer. - mTOR inhibitors block a protein called mTOR, which helps control cell division. mTOR inhibitors may keep cancer cells from growing and prevent the growth of new blood vessels that tumors need to grow. Everolimus and ridaforalimus are used to treat stage III, stage IV, and recurrent endometrial cancer. - Signal transduction inhibitors block signals that are passed from one molecule to another inside a cell. Blocking these signals may kill cancer cells. Metformin is being studied to treat stage III, stage IV, and recurrent endometrial cancer. New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options by Stage Stage I and Stage II Endometrial Cancer Low-risk endometrial cancer (grade 1 or grade 2) Treatment of low-risk stage I endometrial cancer and stage II endometrial cancer may include the following: - Surgery (total hysterectomy and bilateral salpingo-oophorectomy). Lymph nodes in the pelvis and abdomen may also be removed and viewed under a microscope to check for cancer cells. - Surgery (total hysterectomy and bilateral salpingo-oophorectomy, with or without removal of lymph nodes in the pelvis and abdomen) followed by internal radiation therapy. In certain cases, external radiation therapy to the pelvis may be used in place of internal radiation therapy. - Radiation therapy alone for patients who cannot have surgery. - A clinical trial of a new chemotherapy regimen. If cancer has spread to the cervix, a radical hysterectomy with bilateral salpingo-oophorectomy may be done. High-risk endometrial cancer (grade 3) Treatment of high-risk stage I endometrial cancer and stage II endometrial cancer may include the following: - Surgery (radical hysterectomy and bilateral salpingo-oophorectomy). Lymph nodes in the pelvis and abdomen may also be removed and viewed under a microscope to check for cancer cells. - Surgery (radical hysterectomy and bilateral salpingo-oophorectomy) followed by chemotherapy and sometimes radiation therapy. - A clinical trial of a new chemotherapy regimen. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I endometrial carcinoma and stage II endometrial carcinoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage III, Stage IV, and Recurrent Endometrial Cancer Treatment of stage III endometrial cancer, stage IV endometrial cancer, and recurrent endometrial cancer may include the following: - Surgery (radical hysterectomy and removal of lymph nodes in the pelvis so they can be viewed under a microscope to check for cancer cells) followed by adjuvant chemotherapy and/or radiation therapy. - Chemotherapy and internal and external radiation therapy for patients who cannot have surgery. - Hormone therapy for patients who cannot have surgery or radiation therapy. - Targeted therapy with mTOR inhibitors (everolimus or ridaforolimus) or a monoclonal antibody (bevacizumab). - A clinical trial of a new treatment regimen that may include combination chemotherapy, targeted therapy, such as an mTOR inhibitor (everolimus) or signal transduction inhibitor (metformin), and/or hormone therapy, for patients with advanced or recurrent endometrial cancer. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III endometrial carcinoma, stage IV endometrial carcinoma and recurrent endometrial carcinoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Endometrial Cancer,0000014_1,CancerGov,https://www.cancer.gov/types/uterine/patient/endometrial-treatment-pdq,C1883486,T191,Disorders what research (or clinical trials) is being done for Endometrial Cancer ?,0000014_1-8,research,"New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Endometrial Cancer,0000014_1,CancerGov,https://www.cancer.gov/types/uterine/patient/endometrial-treatment-pdq,C1883486,T191,Disorders What is (are) Uterine Sarcoma ?,0000014_2-1,information,"Key Points - Uterine sarcoma is a disease in which malignant (cancer) cells form in the muscles of the uterus or other tissues that support the uterus. - Being exposed to x-rays can increase the risk of uterine sarcoma. - Signs of uterine sarcoma include abnormal bleeding. - Tests that examine the uterus are used to detect (find) and diagnose uterine sarcoma. - Certain factors affect prognosis (chance of recovery) and treatment options. Uterine sarcoma is a disease in which malignant (cancer) cells form in the muscles of the uterus or other tissues that support the uterus. The uterus is part of the female reproductive system. The uterus is the hollow, pear-shaped organ in the pelvis, where a fetus grows. The cervix is at the lower, narrow end of the uterus, and leads to the vagina. Uterine sarcoma is a very rare kind of cancer that forms in the uterine muscles or in tissues that support the uterus. (Information about other types of sarcomas can be found in the PDQ summary on Adult Soft Tissue Sarcoma Treatment.) Uterine sarcoma is different from cancer of the endometrium, a disease in which cancer cells start growing inside the lining of the uterus. (See the PDQ summary on Endometrial Cancer Treatment for information).",Uterine Sarcoma,0000014_2,CancerGov,https://www.cancer.gov/types/uterine/patient/uterine-sarcoma-treatment-pdq,C0338113,T191,Disorders Who is at risk for Uterine Sarcoma? ?,0000014_2-2,susceptibility,"Being exposed to x-rays can increase the risk of uterine sarcoma. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesnt mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for uterine sarcoma include the following: - Past treatment with radiation therapy to the pelvis. - Treatment with tamoxifen for breast cancer. If you are taking this drug, have a pelvic exam every year and report any vaginal bleeding (other than menstrual bleeding) as soon as possible.",Uterine Sarcoma,0000014_2,CancerGov,https://www.cancer.gov/types/uterine/patient/uterine-sarcoma-treatment-pdq,C0338113,T191,Disorders What are the symptoms of Uterine Sarcoma ?,0000014_2-3,symptoms,Signs of uterine sarcoma include abnormal bleeding. Abnormal bleeding from the vagina and other signs and symptoms may be caused by uterine sarcoma or by other conditions. Check with your doctor if you have any of the following: - Bleeding that is not part of menstrual periods. - Bleeding after menopause. - A mass in the vagina. - Pain or a feeling of fullness in the abdomen. - Frequent urination.,Uterine Sarcoma,0000014_2,CancerGov,https://www.cancer.gov/types/uterine/patient/uterine-sarcoma-treatment-pdq,C0338113,T191,Disorders How to diagnose Uterine Sarcoma ?,0000014_2-4,exams and tests,"Tests that examine the uterus are used to detect (find) and diagnose uterine sarcoma. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Pelvic exam: An exam of the vagina, cervix, uterus, fallopian tubes, ovaries, and rectum. A speculum is inserted into the vagina and the doctor or nurse looks at the vagina and cervix for signs of disease. A Pap test of the cervix is usually done. The doctor or nurse also inserts one or two lubricated, gloved fingers of one hand into the vagina and places the other hand over the lower abdomen to feel the size, shape, and position of the uterus and ovaries. The doctor or nurse also inserts a lubricated, gloved finger into the rectum to feel for lumps or abnormal areas. - Pap test: A procedure to collect cells from the surface of the cervix and vagina. A piece of cotton, a brush, or a small wooden stick is used to gently scrape cells from the cervix and vagina. The cells are viewed under a microscope to find out if they are abnormal. This procedure is also called a Pap smear. Because uterine sarcoma begins inside the uterus, this cancer may not show up on the Pap test. - Transvaginal ultrasound exam: A procedure used to examine the vagina, uterus, fallopian tubes, and bladder. An ultrasound transducer (probe) is inserted into the vagina and used to bounce high-energy sound waves (ultrasound) off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The doctor can identify tumors by looking at the sonogram. - Dilatation and curettage : A procedure to remove samples of tissue from the inner lining of the uterus. The cervix is dilated and a curette (spoon-shaped instrument) is inserted into the uterus to remove tissue. The tissue samples are checked under a microscope for signs of disease. This procedure is also called a D&C. - Endometrial biopsy : The removal of tissue from the endometrium (inner lining of the uterus) by inserting a thin, flexible tube through the cervix and into the uterus. The tube is used to gently scrape a small amount of tissue from the endometrium and then remove the tissue samples. A pathologist views the tissue under a microscope to look for cancer cells.",Uterine Sarcoma,0000014_2,CancerGov,https://www.cancer.gov/types/uterine/patient/uterine-sarcoma-treatment-pdq,C0338113,T191,Disorders What is the outlook for Uterine Sarcoma ?,0000014_2-5,outlook,Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - The stage of the cancer. - The type and size of the tumor. - The patient's general health. - Whether the cancer has just been diagnosed or has recurred (come back).,Uterine Sarcoma,0000014_2,CancerGov,https://www.cancer.gov/types/uterine/patient/uterine-sarcoma-treatment-pdq,C0338113,T191,Disorders What are the stages of Uterine Sarcoma ?,0000014_2-6,stages,"Key Points - After uterine sarcoma has been diagnosed, tests are done to find out if cancer cells have spread within the uterus or to other parts of the body. - Uterine sarcoma may be diagnosed, staged, and treated in the same surgery. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - The following stages are used for uterine sarcoma: - Stage I - Stage II - Stage III - Stage IV After uterine sarcoma has been diagnosed, tests are done to find out if cancer cells have spread within the uterus or to other parts of the body. The process used to find out if cancer has spread within the uterus or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following procedures may be used in the staging process: - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. - CA 125 assay : A test that measures the level of CA 125 in the blood. CA 125 is a substance released by cells into the bloodstream. An increased CA 125 level is sometimes a sign of cancer or other condition. - Chest x-ray : An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. - Transvaginal ultrasound exam: A procedure used to examine the vagina, uterus, fallopian tubes, and bladder. An ultrasound transducer (probe) is inserted into the vagina and used to bounce high-energy sound waves (ultrasound) off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The doctor can identify tumors by looking at the sonogram. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the abdomen and pelvis, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues to show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - Cystoscopy : A procedure to look inside the bladder and urethra to check for abnormal areas. A cystoscope is inserted through the urethra into the bladder. A cystoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer. Uterine sarcoma may be diagnosed, staged, and treated in the same surgery. Surgery is used to diagnose, stage, and treat uterine sarcoma. During this surgery, the doctor removes as much of the cancer as possible. The following procedures may be used to diagnose, stage, and treat uterine sarcoma: - Laparotomy: A surgical procedure in which an incision (cut) is made in the wall of the abdomen to check the inside of the abdomen for signs of disease. The size of the incision depends on the reason the laparotomy is being done. Sometimes organs are removed or tissue samples are taken and checked under a microscope for signs of disease. - Abdominal and pelvic washings: A procedure in which a saline solution is placed into the abdominal and pelvic body cavities. After a short time, the fluid is removed and viewed under a microscope to check for cancer cells. - Total abdominal hysterectomy: A surgical procedure to remove the uterus and cervix through a large incision (cut) in the abdomen. - Bilateral salpingo-oophorectomy: Surgery to remove both ovaries and both fallopian tubes. - Lymphadenectomy: A surgical procedure in which lymph nodes are removed and checked under a microscope for signs of cancer. For a regional lymphadenectomy, some of the lymph nodes in the tumor area are removed. For a radical lymphadenectomy, most or all of the lymph nodes in the tumor area are removed. This procedure is also called lymph node dissection. Treatment in addition to surgery may be given, as described in the Treatment Option Overview section of this summary. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if uterine sarcoma spreads to the lung, the cancer cells in the lung are actually uterine sarcoma cells. The disease is metastatic uterine sarcoma, not lung cancer. The following stages are used for uterine sarcoma: Stage I In stage I, cancer is found in the uterus only. Stage I is divided into stages IA and IB, based on how far the cancer has spread. - Stage IA: Cancer is in the endometrium only or less than halfway through the myometrium (muscle layer of the uterus). - Stage IB: Cancer has spread halfway or more into the myometrium. Stage II In stage II, cancer has spread into connective tissue of the cervix, but has not spread outside the uterus. Stage III In stage III, cancer has spread beyond the uterus and cervix, but has not spread beyond the pelvis. Stage III is divided into stages IIIA, IIIB, and IIIC, based on how far the cancer has spread within the pelvis. - Stage IIIA: Cancer has spread to the outer layer of the uterus and/or to the fallopian tubes, ovaries, and ligaments of the uterus. - Stage IIIB: Cancer has spread to the vagina or to the parametrium (connective tissue and fat around the uterus). - Stage IIIC: Cancer has spread to lymph nodes in the pelvis and/or around the aorta (largest artery in the body, which carries blood away from the heart). Stage IV In stage IV, cancer has spread beyond the pelvis. Stage IV is divided into stages IVA and IVB, based on how far the cancer has spread. - Stage IVA: Cancer has spread to the bladder and/or bowel wall. - Stage IVB: Cancer has spread to other parts of the body beyond the pelvis, including the abdomen and/or lymph nodes in the groin.",Uterine Sarcoma,0000014_2,CancerGov,https://www.cancer.gov/types/uterine/patient/uterine-sarcoma-treatment-pdq,C0338113,T191,Disorders What are the treatments for Uterine Sarcoma ?,0000014_2-7,treatment,"Key Points - There are different types of treatment for patients with uterine sarcoma. - Four types of standard treatment are used: - Surgery - Radiation therapy - Chemotherapy - Hormone therapy - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with uterine sarcoma. Different types of treatments are available for patients with uterine sarcoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Four types of standard treatment are used: Surgery Surgery is the most common treatment for uterine sarcoma, as described in the Stages of Uterine Sarcoma section of this summary. Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy Radiation therapy is a cancer treatment that uses high energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External and internal radiation therapy are used to treat uterine sarcoma, and may also be used as palliative therapy to relieve symptoms and improve quality of life. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. Hormone therapy Hormone therapy is a cancer treatment that removes hormones or blocks their action and stops cancer cells from growing. Hormones are substances produced by glands in the body and circulated in the bloodstream. Some hormones can cause certain cancers to grow. If tests show the cancer cells have places where hormones can attach (receptors), drugs, surgery, or radiation therapy is used to reduce the production of hormones or block them from working. New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options by Stage Stage I Uterine Sarcoma Treatment of stage I uterine sarcoma may include the following: - Surgery (total abdominal hysterectomy, bilateral salpingo-oophorectomy, and lymphadenectomy). - Surgery followed by radiation therapy to the pelvis. - Surgery followed by chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I uterine sarcoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage II Uterine Sarcoma Treatment of stage II uterine sarcoma may include the following: - Surgery (total abdominal hysterectomy, bilateral salpingo-oophorectomy, and lymphadenectomy). - Surgery followed by radiation therapy to the pelvis. - Surgery followed by chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage II uterine sarcoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage III Uterine Sarcoma Treatment of stage III uterine sarcoma may include the following: - Surgery (total abdominal hysterectomy, bilateral salpingo-oophorectomy, and lymphadenectomy). - A clinical trial of surgery followed by radiation therapy to the pelvis. - A clinical trial of surgery followed by chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III uterine sarcoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage IV Uterine Sarcoma There is no standard treatment for patients with stage IV uterine sarcoma. Treatment may include a clinical trial using chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage IV uterine sarcoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Uterine Sarcoma,0000014_2,CancerGov,https://www.cancer.gov/types/uterine/patient/uterine-sarcoma-treatment-pdq,C0338113,T191,Disorders what research (or clinical trials) is being done for Uterine Sarcoma ?,0000014_2-8,research,"New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Uterine Sarcoma,0000014_2,CancerGov,https://www.cancer.gov/types/uterine/patient/uterine-sarcoma-treatment-pdq,C0338113,T191,Disorders What is (are) Endometrial Cancer ?,0000014_3-1,information,"Key Points - Endometrial cancer is a disease in which malignant (cancer) cells form in the tissues of the endometrium. - Endometrial cancer is the most common invasive cancer of the female reproductive system. Endometrial cancer is a disease in which malignant (cancer) cells form in the tissues of the endometrium. The endometrium is the lining of the uterus. The uterus is part of the female reproductive system. It is a hollow, pear-shaped, muscular organ in the pelvis, where a fetus grows. Cancer of the endometrium is different from cancer of the muscle of the uterus, which is called sarcoma of the uterus. See the PDQ summary on Uterine Sarcoma Treatment for more information. See the following PDQ summaries for more information about endometrial cancer: - Endometrial Cancer Screening - Endometrial Cancer Treatment Endometrial cancer is the most common invasive cancer of the female reproductive system. Endometrial cancer is diagnosed most often in postmenopausal women at an average age of 60 years . From 2004 to 2013, the number of new cases of endometrial cancer increased slightly in white and African American women. From 2005 to 2014, the number of deaths from endometrial cancer also increased slightly in white and African American women. Compared with white women, rates of endometrial cancer are lower in Japanese Americans and in Latinas. The rates of endometrial cancer in white women are about the same as in African Americans or in native Hawaiians. The number of deaths from endometrial cancer is higher in African American women compared with women of other races.",Endometrial Cancer,0000014_3,CancerGov,https://www.cancer.gov/types/uterine/patient/endometrial-prevention-pdq,C1883486,T191,Disorders How to prevent Endometrial Cancer ?,0000014_3-2,prevention,"Key Points - Avoiding risk factors and increasing protective factors may help prevent cancer. - The following risk factors increase the risk of endometrial cancer: - Endometrial hyperplasia - Estrogen - Tamoxifen - Obesity, weight gain, metabolic syndrome, and diabetes - Genetic factors - The following protective factors decrease the risk of endometrial cancer: - Pregnancy and breast-feeding - Combination oral contraceptives - Physical activity - Cigarette smoking - It is not known if the following factors affect the risk of endometrial cancer: - Weight loss - Fruits, vegetables, and vitamins - Cancer prevention clinical trials are used to study ways to prevent cancer. - New ways to prevent endometrial cancer are being studied in clinical trials. Avoiding risk factors and increasing protective factors may help prevent cancer. Avoiding cancer risk factors may help prevent certain cancers. Risk factors include smoking, being overweight, and not getting enough exercise. Increasing protective factors such as quitting smoking and exercising may also help prevent some cancers. Talk to your doctor or other health care professional about how you might lower your risk of cancer. The following risk factors increase the risk of endometrial cancer: Endometrial hyperplasia Endometrial hyperplasia is an abnormal thickening of the endometrium (lining of the uterus). It is not cancer, but in some cases, it may lead to endometrial cancer. Estrogen Estrogen is a hormone made by the body. It helps the body develop and maintain female sex characteristics. Estrogen can affect the growth of some cancers, including endometrial cancer. A woman's risk of developing endometrial cancer is increased by being exposed to estrogen in the following ways: - Estrogen-only hormone replacement therapy: Estrogen may be given to replace the estrogen no longer produced by the ovaries in postmenopausal women or women whose ovaries have been removed. This is called hormone replacement therapy (HRT), or hormone therapy (HT). The use of HRT that contains only estrogen increases the risk of endometrial cancer and endometrial hyperplasia. For this reason, estrogen therapy alone is usually prescribed only for women who do not have a uterus. HRT that contains only estrogen also increases the risk of stroke and blood clots. When estrogen is combined with progestin (another hormone), it is called combination estrogen-progestin replacement therapy. For postmenopausal women, taking estrogen in combination with progestin does not increase the risk of endometrial cancer, but it does increase the risk of breast cancer. (See the Breast Cancer Prevention summary for more information.) - Early menstruation: Beginning to have menstrual periods at an early age increases the number of years the body is exposed to estrogen and increases a woman's risk of endometrial cancer. - Late menopause: Women who reach menopause at an older age are exposed to estrogen for a longer time and have an increased risk of endometrial cancer. - Never being pregnant: Because estrogen levels are lower during pregnancy, women who have never been pregnant are exposed to estrogen for a longer time than women who have been pregnant. This increases the risk of endometrial cancer. Tamoxifen Tamoxifen is one of a group of drugs called selective estrogen receptor modulators, or SERMs. Tamoxifen acts like estrogen on some tissues in the body, such as the uterus, but blocks the effects of estrogen on other tissues, such as the breast. Tamoxifen is used to prevent breast cancer in women who are at high risk for the disease. However, using tamoxifen for more than 2 years increases the risk of endometrial cancer. This risk is greater in postmenopausal women. Raloxifene is a SERM that is used to prevent bone weakness in postmenopausal women. However, it does not have estrogen-like effects on the uterus and has not been shown to increase the risk of endometrial cancer. Obesity, weight gain, metabolic syndrome, and diabetes Obesity, gaining weight as an adult, or having metabolic syndrome increases the risk of endometrial cancer. Obesity is related to other risk factors such as high estrogen levels, having extra fat around the waist, polycystic ovary syndrome, and lack of physical activity. Having metabolic syndrome increases the risk of endometrial cancer. Metabolic syndrome is a condition that includes extra fat around the waist, high blood sugar, high blood pressure, and high levels of triglycerides (a type of fat) in the blood. Genetic factors Hereditary nonpolyposis colon cancer (HNPCC) syndrome (also known as Lynch Syndrome) is an inherited disorder caused by changes in certain genes. Women who have HNPCC syndrome have a much higher risk of developing endometrial cancer than women who do not have HNPCC syndrome. Polycystic ovary syndrome (a disorder of the hormones made by the ovaries), and Cowden syndrome are inherited conditions that are linked to an increased risk of endometrial cancer. Women with a family history of endometrial cancer in a first-degree relative (mother, sister, or daughter) are also at increased risk of endometrial cancer. The following protective factors decrease the risk of endometrial cancer: Pregnancy and breast-feeding Estrogen levels are lower during pregnancy and when breast-feeding. The risk of endometrial cancer is lower in women who have had children. Breastfeeding for more than 18 months also decreases the risk of endometrial cancer. Combination oral contraceptives Taking contraceptives that combine estrogen and progestin (combination oral contraceptives) decreases the risk of endometrial cancer. The protective effect of combination oral contraceptives increases with the length of time they are used, and can last for many years after oral contraceptive use has been stopped. While taking oral contraceptives, women have a higher risk of blood clots, stroke, and heart attack, especially women who smoke and are older than 35 years. Physical activity Physical activity at home (exercise) or on the job may lower the risk of endometrial cancer. Cigarette smoking Smoking at least 20 cigarettes a day may lower the risk of endometrial cancer. The risk of endometrial cancer is even lower in postmenopausal women who smoke. However, there are many proven harms of smoking. Cigarette smokers live about 10 years less than nonsmokers. Cigarette smokers also have an increased risk of the following: - Heart disease. - Head and neck cancers. - Lung cancer. - Bladder cancer. - Pancreatic cancer. It is not known if the following factors affect the risk of endometrial cancer: Weight loss It is not known if losing weight decreases the risk of endometrial cancer. Fruits, vegetables, and vitamins A diet that includes, fruits, vegetables, phytoestrogen, soy, and vitamin D has not been found to affect the risk of endometrial cancer. Taking multivitamins has little or no effect on the risk of common cancers, including endometrial cancer. Cancer prevention clinical trials are used to study ways to prevent cancer. Cancer prevention clinical trials are used to study ways to lower the risk of developing certain types of cancer. Some cancer prevention trials are conducted with healthy people who have not had cancer but who have an increased risk for cancer. Other prevention trials are conducted with people who have had cancer and are trying to prevent another cancer of the same type or to lower their chance of developing a new type of cancer. Other trials are done with healthy volunteers who are not known to have any risk factors for cancer. The purpose of some cancer prevention clinical trials is to find out whether actions people take can prevent cancer. These may include eating fruits and vegetables, exercising, quitting smoking, or taking certain medicines, vitamins, minerals, or food supplements. New ways to prevent endometrial cancer are being studied in clinical trials. Clinical trials are taking place in many parts of the country. Information about clinical trials can be found in the Clinical Trials section of the NCI website. Check NCI's list of cancer clinical trials for endometrial cancer prevention trials that are now accepting patients.",Endometrial Cancer,0000014_3,CancerGov,https://www.cancer.gov/types/uterine/patient/endometrial-prevention-pdq,C1883486,T191,Disorders Who is at risk for Endometrial Cancer? ?,0000014_3-3,susceptibility,"Key Points - Avoiding risk factors and increasing protective factors may help prevent cancer. - The following risk factors increase the risk of endometrial cancer: - Endometrial hyperplasia - Estrogen - Tamoxifen - Obesity, weight gain, metabolic syndrome, and diabetes - Genetic factors - The following protective factors decrease the risk of endometrial cancer: - Pregnancy and breast-feeding - Combination oral contraceptives - Physical activity - Cigarette smoking - It is not known if the following factors affect the risk of endometrial cancer: - Weight loss - Fruits, vegetables, and vitamins - Cancer prevention clinical trials are used to study ways to prevent cancer. - New ways to prevent endometrial cancer are being studied in clinical trials. Avoiding risk factors and increasing protective factors may help prevent cancer. Avoiding cancer risk factors may help prevent certain cancers. Risk factors include smoking, being overweight, and not getting enough exercise. Increasing protective factors such as quitting smoking and exercising may also help prevent some cancers. Talk to your doctor or other health care professional about how you might lower your risk of cancer. The following risk factors increase the risk of endometrial cancer: Endometrial hyperplasia Endometrial hyperplasia is an abnormal thickening of the endometrium (lining of the uterus). It is not cancer, but in some cases, it may lead to endometrial cancer. Estrogen Estrogen is a hormone made by the body. It helps the body develop and maintain female sex characteristics. Estrogen can affect the growth of some cancers, including endometrial cancer. A woman's risk of developing endometrial cancer is increased by being exposed to estrogen in the following ways: - Estrogen-only hormone replacement therapy: Estrogen may be given to replace the estrogen no longer produced by the ovaries in postmenopausal women or women whose ovaries have been removed. This is called hormone replacement therapy (HRT), or hormone therapy (HT). The use of HRT that contains only estrogen increases the risk of endometrial cancer and endometrial hyperplasia. For this reason, estrogen therapy alone is usually prescribed only for women who do not have a uterus. HRT that contains only estrogen also increases the risk of stroke and blood clots. When estrogen is combined with progestin (another hormone), it is called combination estrogen-progestin replacement therapy. For postmenopausal women, taking estrogen in combination with progestin does not increase the risk of endometrial cancer, but it does increase the risk of breast cancer. (See the Breast Cancer Prevention summary for more information.) - Early menstruation: Beginning to have menstrual periods at an early age increases the number of years the body is exposed to estrogen and increases a woman's risk of endometrial cancer. - Late menopause: Women who reach menopause at an older age are exposed to estrogen for a longer time and have an increased risk of endometrial cancer. - Never being pregnant: Because estrogen levels are lower during pregnancy, women who have never been pregnant are exposed to estrogen for a longer time than women who have been pregnant. This increases the risk of endometrial cancer. Tamoxifen Tamoxifen is one of a group of drugs called selective estrogen receptor modulators, or SERMs. Tamoxifen acts like estrogen on some tissues in the body, such as the uterus, but blocks the effects of estrogen on other tissues, such as the breast. Tamoxifen is used to prevent breast cancer in women who are at high risk for the disease. However, using tamoxifen for more than 2 years increases the risk of endometrial cancer. This risk is greater in postmenopausal women. Raloxifene is a SERM that is used to prevent bone weakness in postmenopausal women. However, it does not have estrogen-like effects on the uterus and has not been shown to increase the risk of endometrial cancer. Obesity, weight gain, metabolic syndrome, and diabetes Obesity, gaining weight as an adult, or having metabolic syndrome increases the risk of endometrial cancer. Obesity is related to other risk factors such as high estrogen levels, having extra fat around the waist, polycystic ovary syndrome, and lack of physical activity. Having metabolic syndrome increases the risk of endometrial cancer. Metabolic syndrome is a condition that includes extra fat around the waist, high blood sugar, high blood pressure, and high levels of triglycerides (a type of fat) in the blood. Genetic factors Hereditary nonpolyposis colon cancer (HNPCC) syndrome (also known as Lynch Syndrome) is an inherited disorder caused by changes in certain genes. Women who have HNPCC syndrome have a much higher risk of developing endometrial cancer than women who do not have HNPCC syndrome. Polycystic ovary syndrome (a disorder of the hormones made by the ovaries), and Cowden syndrome are inherited conditions that are linked to an increased risk of endometrial cancer. Women with a family history of endometrial cancer in a first-degree relative (mother, sister, or daughter) are also at increased risk of endometrial cancer. It is not known if the following factors affect the risk of endometrial cancer: Weight loss It is not known if losing weight decreases the risk of endometrial cancer. Fruits, vegetables, and vitamins A diet that includes, fruits, vegetables, phytoestrogen, soy, and vitamin D has not been found to affect the risk of endometrial cancer. Taking multivitamins has little or no effect on the risk of common cancers, including endometrial cancer.",Endometrial Cancer,0000014_3,CancerGov,https://www.cancer.gov/types/uterine/patient/endometrial-prevention-pdq,C1883486,T191,Disorders what research (or clinical trials) is being done for Endometrial Cancer ?,0000014_3-4,research,"Cancer prevention clinical trials are used to study ways to prevent cancer. Cancer prevention clinical trials are used to study ways to lower the risk of developing certain types of cancer. Some cancer prevention trials are conducted with healthy people who have not had cancer but who have an increased risk for cancer. Other prevention trials are conducted with people who have had cancer and are trying to prevent another cancer of the same type or to lower their chance of developing a new type of cancer. Other trials are done with healthy volunteers who are not known to have any risk factors for cancer. The purpose of some cancer prevention clinical trials is to find out whether actions people take can prevent cancer. These may include eating fruits and vegetables, exercising, quitting smoking, or taking certain medicines, vitamins, minerals, or food supplements. New ways to prevent endometrial cancer are being studied in clinical trials. Clinical trials are taking place in many parts of the country. Information about clinical trials can be found in the Clinical Trials section of the NCI website. Check NCI's list of cancer clinical trials for endometrial cancer prevention trials that are now accepting patients.",Endometrial Cancer,0000014_3,CancerGov,https://www.cancer.gov/types/uterine/patient/endometrial-prevention-pdq,C1883486,T191,Disorders What is (are) Endometrial Cancer ?,0000014_4-1,information,"Key Points - Endometrial cancer is a disease in which malignant (cancer) cells form in the tissues of the endometrium. - In the United States, endometrial cancer is the most common invasive cancer of the female reproductive system. - Health history and certain medicines can affect the risk of developing endometrial cancer. Endometrial cancer is a disease in which malignant (cancer) cells form in the tissues of the endometrium. The endometrium is the innermost lining of the uterus. The uterus is a hollow, muscular organ in a woman's pelvis. The uterus is where a fetus grows. In most nonpregnant women, the uterus is about 3 inches long. Cancer of the endometrium is different from cancer of the muscle of the uterus, which is called uterine sarcoma. See the PDQ summary on Uterine Sarcoma Treatment for more information. See the following PDQ summaries for more information about endometrial cancer: - Endometrial Cancer Treatment - Endometrial Cancer Prevention In the United States, endometrial cancer is the most common invasive cancer of the female reproductive system. Endometrial cancer is diagnosed most often in postmenopausal women at an average age of 60 years. From 2004 to 2013, the number of new cases of endometrial cancer increased slightly in white and black women. From 2005 to 2014, the number of deaths from endometrial cancer increased slightly in white and black women. When endometrial cancer is diagnosed in black women, it is usually more advanced and less likely to be cured.",Endometrial Cancer,0000014_4,CancerGov,https://www.cancer.gov/types/uterine/patient/endometrial-screening-pdq,C1883486,T191,Disorders Who is at risk for Endometrial Cancer? ?,0000014_4-2,susceptibility,Health history and certain medicines can affect the risk of developing endometrial cancer. Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesnt mean that you will not get cancer. People who think they may be at risk should discuss this with their doctor. Risk factors for endometrial cancer include the following: - Taking tamoxifen for treatment or prevention of breast cancer. - Taking estrogen alone. (Taking estrogen in combination with progestin does not appear to increase the risk of endometrial cancer.) - Being overweight. - Eating a high-fat diet. - Never giving birth. - Beginning menstruation at an early age. - Reaching menopause at an older age. - Having the gene for hereditary non-polyposis colon cancer (HNPCC). - Being white.,Endometrial Cancer,0000014_4,CancerGov,https://www.cancer.gov/types/uterine/patient/endometrial-screening-pdq,C1883486,T191,Disorders What is (are) Childhood Extracranial Germ Cell Tumors ?,0000015_1-1,information,"Key Points - Childhood extracranial germ cell tumors form from germ cells in parts of the body other than the brain. - Childhood extracranial germ cell tumors may be benign or malignant. - There are three types of extracranial germ cell tumors. - Mature Teratomas - Immature Teratomas - Malignant Germ Cell Tumors - Childhood extracranial germ cell tumors are grouped as gonadal or extragonadal. - Gonadal Germ Cell Tumors - Extragonadal Extracranial Germ Cell Tumors - The cause of most childhood extracranial germ cell tumors is unknown. - Having certain inherited disorders can increase the risk of an extracranial germ cell tumor. - Signs of childhood extracranial germ cell tumors depend on the type of tumor and where it is in the body. - Imaging studies and blood tests are used to detect (find) and diagnose childhood extracranial germ cell tumors. - Certain factors affect prognosis (chance of recovery) and treatment options. Childhood extracranial germ cell tumors form from germ cells in parts of the body other than the brain. A germ cell is a type of cell that forms as a fetus (unborn baby) develops. These cells later become sperm in the testicles or eggs in the ovaries. Sometimes while the fetus is forming, germ cells travel to parts of the body where they should not be and grow into a germ cell tumor. The tumor may form before or after birth. This summary is about germ cell tumors that form in parts of the body that are extracranial (outside the brain). Extracranial germ cell tumors usually form in the following areas of the body: - Testicles. - Ovaries. - Sacrum or coccyx (bottom part of the spine). - Retroperitoneum (the back wall of the abdomen). - Mediastinum (area between the lungs). Extracranial germ cell tumors are most common in adolescents 15 to 19 years of age. See the PDQ summary on Childhood Central Nervous System Germ Cell Tumors Treatment for information on intracranial (inside the brain) germ cell tumors. Childhood extracranial germ cell tumors may be benign or malignant. Extracranial germ cell tumors may be benign (noncancer) or malignant (cancer). There are three types of extracranial germ cell tumors. Extracranial germ cell tumors are grouped into mature teratomas, immature teratomas, and malignant germ cell tumors: Mature Teratomas Mature teratomas are the most common type of extracranial germ cell tumor. Mature teratomas are benign tumors and not likely to become cancer. They usually occur in the sacrum or coccyx (bottom part of the spine) in newborns or in the ovaries of girls at the start of puberty. The cells of mature teratomas look almost like normal cells under a microscope. Some mature teratomas release enzymes or hormones that cause signs and symptoms of disease. Immature Teratomas Immature teratomas also usually occur in the sacrum or coccyx (bottom part of the spine) in newborns or the ovaries of girls at the start of puberty. Immature teratomas have cells that look very different from normal cells under a microscope. Immature teratomas may be cancer. They often have several different types of tissue in them, such as hair, muscle, and bone. Some immature teratomas release enzymes or hormones that cause signs and symptoms of disease. Malignant Germ Cell Tumors Malignant germ cell tumors are cancer. There are two main types of malignant germ cell tumors: - Germinomas: Tumors that make a hormone called beta-human chorionic gonadotropin (-hCG). There are three types of germinomas. - Dysgerminomas form in the ovary in girls. - Seminomas form in the testicle in boys. - Germinomas form in areas of the body that are not the ovary or testicle. - Nongerminomas: There are four types of nongerminomas. - Yolk sac tumors make a hormone called alpha-fetoprotein (AFP). They can form in the ovary, testicle, or other areas of the body. - Choriocarcinomas make a hormone called beta-human chorionic gonadotropin (-hCG). They can form in the ovary, testicle, or other areas of the body. - Embryonal carcinomas may make a hormone called -hCG and/or a hormone called AFP. They can form in the testicle or other parts of the body, but not in the ovary. - Mixed germ cell tumors are made up of both malignant germ cell tumor and teratoma. They can form in the ovary, testicle, or other areas of the body. Childhood extracranial germ cell tumors are grouped as gonadal or extragonadal. Malignant extracranial germ cell tumors are gonadal or extragonadal. Gonadal Germ Cell Tumors Gonadal germ cell tumors form in the testicles in boys or ovaries in girls. Testicular Germ Cell Tumors Testicular germ cell tumors are divided into two main types, seminoma and nonseminoma. - Seminomas make a hormone called beta-human chorionic gonadotropin (-hCG). - Nonseminomas are usually large and cause signs or symptoms. They tend to grow and spread more quickly than seminomas. Testicular germ cell tumors usually occur before the age of 4 years or in adolescents and young adults. Testicular germ cell tumors in adolescents and young adults are different from those that form in early childhood. Boys older than 14 years with testicular germ cell tumors are treated in pediatric cancer centers, but the treatment is much like the treatment used in adults. (See the PDQ summary on Testicular Cancer Treatment for more information.) Ovarian Germ Cell Tumors Ovarian germ cell tumors are more common in adolescent girls and young women. Most ovarian germ cell tumors are benign teratomas. Sometimes immature teratomas, dysgerminomas, yolk sac tumors, and mixed germ cell tumors (cancer) occur. (See the PDQ summary on Ovarian Germ Cell Tumors Treatment for more information.) Extragonadal Extracranial Germ Cell Tumors Extragonadal extracranial germ cell tumors form in areas other than the brain, testicles, or ovaries. Most extragonadal extracranial germ cell tumors form along the midline of the body. This includes the following: - Sacrum (the large, triangle-shaped bone in the lower spine that forms part of the pelvis). - Coccyx (the small bone at the bottom of the spine, also called the tailbone). - Mediastinum (the area between the lungs). - Back of the abdomen. - Neck. In younger children, extragonadal extracranial germ cell tumors usually occur at birth or in early childhood. Most of these tumors are teratomas in the sacrum or coccyx. In older children, adolescents, and young adults, extragonadal extracranial germ cell tumors are often in the mediastinum.",Childhood Extracranial Germ Cell Tumors,0000015_1,CancerGov,https://www.cancer.gov/types/extracranial-germ-cell/patient/germ-cell-treatment-pdq,C0205851,T191,Disorders What causes Childhood Extracranial Germ Cell Tumors ?,0000015_1-2,causes,The cause of most childhood extracranial germ cell tumors is unknown.,Childhood Extracranial Germ Cell Tumors,0000015_1,CancerGov,https://www.cancer.gov/types/extracranial-germ-cell/patient/germ-cell-treatment-pdq,C0205851,T191,Disorders Who is at risk for Childhood Extracranial Germ Cell Tumors? ?,0000015_1-3,susceptibility,Having certain inherited disorders can increase the risk of an extracranial germ cell tumor. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesnt mean that you will not get cancer. Talk with your childs doctor if you think your child may be at risk. Possible risk factors for extracranial germ cell tumors include the following: - Having certain genetic syndromes: - Klinefelter syndrome may increase the risk of germ cell tumors in the mediastinum. - Swyer syndrome may increase the risk of germ cell tumors in the testicles or ovaries. - Turner syndrome may increase the risk of germ cell tumors in the ovaries. - Having an undescended testicle may increase the risk of developing a testicular germ cell tumor.,Childhood Extracranial Germ Cell Tumors,0000015_1,CancerGov,https://www.cancer.gov/types/extracranial-germ-cell/patient/germ-cell-treatment-pdq,C0205851,T191,Disorders What are the symptoms of Childhood Extracranial Germ Cell Tumors ?,0000015_1-4,symptoms,"Signs of childhood extracranial germ cell tumors depend on the type of tumor and where it is in the body. Different tumors may cause the following signs and symptoms. Other conditions may cause these same signs and symptoms. Check with a doctor if your child has any of the following: - A lump in the abdomen or lower back. - A painless lump in the testicle. - Pain in the abdomen. - Fever. - Constipation. - In females, no menstrual periods. - In females, unusual vaginal bleeding.",Childhood Extracranial Germ Cell Tumors,0000015_1,CancerGov,https://www.cancer.gov/types/extracranial-germ-cell/patient/germ-cell-treatment-pdq,C0205851,T191,Disorders How to diagnose Childhood Extracranial Germ Cell Tumors ?,0000015_1-5,exams and tests,"Imaging studies and blood tests are used to detect (find) and diagnose childhood extracranial germ cell tumors. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. The testicles may be checked for lumps, swelling, or pain. A history of the patient's health habits and past illnesses and treatments will also be taken. - Serum tumor marker test : A procedure in which a sample of blood is checked to measure the amounts of certain substances released into the blood by organs, tissues, or tumor cells in the body. Certain substances are linked to specific types of cancer when found in increased levels in the blood. These are called tumor markers. Most malignant germ cell tumors release tumor markers. The following tumor markers are used to detect extracranial germ cell tumors: - Alpha-fetoprotein (AFP). - Beta-human chorionic gonadotropin (-hCG). For testicular germ cell tumors, blood levels of the tumor markers help show if the tumor is a seminoma or nonseminoma. - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. - Chest x-ray : An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later. - Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. In some cases, the tumor is removed during surgery and then a biopsy is done. The following tests may be done on the sample of tissue that is removed: - Cytogenetic analysis : A laboratory test in which cells in a sample of tissue are viewed under a microscope to look for certain changes in the chromosomes. - Immunohistochemistry : A test that uses antibodies to check for certain antigens in a sample of tissue. The antibody is usually linked to a radioactive substance or a dye that causes the tissue to light up under a microscope. This type of test may be used to tell the difference between different types of cancer.",Childhood Extracranial Germ Cell Tumors,0000015_1,CancerGov,https://www.cancer.gov/types/extracranial-germ-cell/patient/germ-cell-treatment-pdq,C0205851,T191,Disorders What is the outlook for Childhood Extracranial Germ Cell Tumors ?,0000015_1-6,outlook,"Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - The type of germ cell tumor. - Where the tumor first began to grow. - The stage of the cancer (whether it has spread to nearby areas or to other places in the body). - How well the tumor responds to treatment (lower AFP and -hCG levels). - Whether the tumor can be completely removed by surgery. - The patient's age and general health. - Whether the cancer has just been diagnosed or has recurred (come back). The prognosis for childhood extracranial germ cell tumors, especially ovarian germ cell tumors, is good.",Childhood Extracranial Germ Cell Tumors,0000015_1,CancerGov,https://www.cancer.gov/types/extracranial-germ-cell/patient/germ-cell-treatment-pdq,C0205851,T191,Disorders What are the stages of Childhood Extracranial Germ Cell Tumors ?,0000015_1-7,stages,"Key Points - After a childhood extracranial germ cell tumor has been diagnosed, tests are done to find out if cancer cells have spread from where the tumor started to nearby areas or to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - Stages are used to describe the different types of extracranial germ cell tumors. - Childhood nonseminoma testicular germ cell tumors - Childhood ovarian germ cell tumors - Childhood extragonadal extracranial germ cell tumors After a childhood extracranial germ cell tumor has been diagnosed, tests are done to find out if cancer cells have spread from where the tumor started to nearby areas or to other parts of the body. The process used to find out if cancer has spread from where the tumor started to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. In some cases, staging may follow surgery to remove the tumor. The following procedures may be used: - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body, such as the lymph nodes. This procedure is also called nuclear magnetic resonance imaging. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the chest or lymph nodes, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - Bone scan : A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones with cancer and is detected by a scanner. - Thoracentesis : The removal of fluid from the space between the lining of the chest and the lung, using a needle. A pathologist views the fluid under a microscope to look for cancer cells. - Paracentesis : The removal of fluid from the space between the lining of the abdomen and the organs in the abdomen, using a needle. A pathologist views the fluid under a microscope to look for cancer cells. The results from tests and procedures used to detect and diagnose childhood extracranial germ cell tumors may also be used in staging. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if an extracranial germ cell tumor spreads to the liver, the cancer cells in the liver are actually cancerous germ cells. The disease is metastatic extracranial germ cell tumor, not liver cancer. Stages are used to describe the different types of extracranial germ cell tumors. Childhood nonseminoma testicular germ cell tumors - Stage I: In stage I, the cancer is found in the testicle only and is completely removed by surgery. - Stage II: In stage II, the cancer is removed by surgery and some cancer cells remain in the scrotum or cancer that can be seen with a microscope only has spread to the scrotum or spermatic cord. Tumor marker levels do not return to normal after surgery or the tumor marker levels increase. - Stage III: In stage III, the cancer has spread to one or more lymph nodes in the abdomen and is not completely removed by surgery. The cancer that remains after surgery can be seen without a microscope. - Stage IV: In stage IV, the cancer has spread to distant parts of the body such as the liver, brain, bone, or lung. Childhood ovarian germ cell tumors There are two types of stages used for childhood ovarian germ cell tumors. The following stages are from the Children's Oncology Group: - Stage I: In stage I, the cancer is in the ovary and can be completely removed by surgery and the capsule (outer covering) of the ovary has not ruptured (broken open). - Stage II: In stage II, one of the following is true: - The cancer is not completely removed by surgery. The remaining cancer can be seen with a microscope only. - The cancer has spread to the lymph nodes and can be seen with a microscope only. - The cancer has spread to the capsule (outer covering) of the ovary. - Stage III: In stage III, one of the following is true: - The cancer is not completely removed by surgery. The remaining cancer can be seen without a microscope. - The cancer has spread to lymph nodes and the lymph nodes are 2 centimeters or larger. Cancer in the lymph nodes can be seen without a microscope. - The cancer is found in fluid in the abdomen. - Stage IV: In stage IV, the cancer has spread to the lung, liver, brain, or bone. The following stages are from the International Federation of Gynecology and Obstetrics (FIGO): - Stage I: In stage I, cancer is found in one or both of the ovaries and has not spread. Stage I is divided into stage IA, stage IB, and stage IC. - Stage IA: Cancer is found in one ovary. - Stage IB: Cancer is found in both ovaries. - Stage IC: Cancer is found in one or both ovaries and one of the following is true: - cancer is found on the outside surface of one or both ovaries; or - the capsule (outer covering) of the tumor has ruptured (broken open); or - cancer cells are found in fluid that has collected in the abdomen; or - cancer cells are found in washings of the peritoneal cavity (the body cavity that contains most of the organs in the abdomen). - Stage II: In stage II, cancer is found in one or both ovaries and has spread into other areas of the pelvis. Stage II is divided into stage IIA, stage IIB, and stage IIC. - Stage IIA: Cancer has spread to the uterus and/or the fallopian tubes (the long slender tubes through which eggs pass from the ovaries to the uterus). - Stage IIB: Cancer has spread to other tissue within the pelvis such as the bladder, rectum, or vagina. - Stage IIC: Cancer has spread to the uterus and/or fallopian tubes and/or other tissue within the pelvis and one of the following is true: - cancer is found on the outside surface of one or both ovaries; or - the capsule (outer covering) of the tumor has ruptured (broken open); or - cancer cells are found in fluid that has collected in the abdomen; or - cancer cells are found in washings of the peritoneal cavity (the body cavity that contains most of the organs in the abdomen). - Stage III: In stage III, cancer is found in one or both ovaries and has spread to other parts of the abdomen. Cancer that has spread to the surface of the liver is also stage III disease. Stage III is divided into stage IIIA, stage IIIB, and stage IIIC: - Stage IIIA: The tumor is found in the pelvis only, but cancer cells that can only be seen with a microscope have spread to the surface of the peritoneum (tissue that lines the abdominal wall and covers most of the organs in the abdomen) or to the small bowel. - Stage IIIB: Cancer has spread to the peritoneum and is 2 centimeters or smaller in diameter. - Stage IIIC: Cancer has spread to the peritoneum and is larger than 2 centimeters in diameter and/or has spread to lymph nodes in the abdomen. - Stage IV: In stage IV, cancer is found in one or both ovaries and has metastasized (spread) beyond the abdomen to other parts of the body. Cancer that has spread to tissues in the liver is also stage IV disease. Childhood extragonadal extracranial germ cell tumors - Stage I: In stage I, the cancer is in one place and can be completely removed by surgery. For tumors in the sacrum or coccyx (bottom part of the spine), the sacrum and coccyx are completely removed by surgery. Tumor marker levels return to normal after surgery. - Stage II: In stage II, the cancer has spread to the capsule (outer covering) and/or lymph nodes. The cancer is not completely removed by surgery and the cancer remaining after surgery can be seen with a microscope only. Tumor marker levels do not return to normal after surgery or increase. - Stage III: In stage III, one of the following is true: - The cancer is not completely removed by surgery. The cancer remaining after surgery can be seen without a microscope. - The cancer has spread to lymph nodes and is larger than 2 centimeters in diameter. - Stage IV: In stage IV, the cancer has spread to distant parts of the body, including the liver, brain, bone, or lung.",Childhood Extracranial Germ Cell Tumors,0000015_1,CancerGov,https://www.cancer.gov/types/extracranial-germ-cell/patient/germ-cell-treatment-pdq,C0205851,T191,Disorders What are the treatments for Childhood Extracranial Germ Cell Tumors ?,0000015_1-8,treatment,"Key Points - There are different types of treatment for children with extracranial germ cell tumors. - Children with extracranial germ cell tumors should have their treatment planned by a team of health care providers who are experts in treating cancer in children. - Some cancer treatments cause side effects months or years after treatment has ended. - Three types of standard treatment are used: - Surgery - Observation - Chemotherapy - New types of treatment are being tested in clinical trials. - High-dose chemotherapy with stem cell transplant - Radiation therapy - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for children with extracranial germ cell tumors. Different types of treatments are available for children with extracranial germ cell tumors. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment. Children with extracranial germ cell tumors should have their treatment planned by a team of health care providers who are experts in treating cancer in children. Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other health care providers who are experts in treating children with extracranial germ cell tumors and who specialize in certain areas of medicine. These may include the following specialists: - Pediatrician. - Pediatric surgeon. - Pediatric hematologist. - Radiation oncologist. - Endocrinologist. - Pediatric nurse specialist. - Rehabilitation specialist. - Child life professional. - Psychologist. - Social worker. - Geneticist. Some cancer treatments cause side effects months or years after treatment has ended. Side effects from cancer treatment that begin during or after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include the following: - Physical problems. - Changes in mood, feelings, thinking, learning, or memory. - Second cancers (new types of cancer). For example, late effects of surgery to remove tumors in the sacrum or coccyx include constipation, loss of bowel and bladder control, and scars. Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information). Three types of standard treatment are used: Surgery Surgery to completely remove the tumor is done whenever possible. If the tumor is very large, chemotherapy may be given first, to make the tumor smaller and decrease the amount of tissue that needs to be removed during surgery. A goal of surgery is to keep reproductive function. The following types of surgery may be used: - Resection: Surgery to remove tissue or part or all of an organ. - Radical inguinal orchiectomy: Surgery to remove one or both testicles through an incision (cut) in the groin. - Unilateral salpingo-oophorectomy: Surgery to remove one ovary and one fallopian tube on the same side. Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Observation Observation is closely monitoring a patients condition without giving any treatment until signs or symptoms appear or change. For childhood extracranial germ cell tumors, this includes physical exams, imaging tests, and tumor marker tests. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is treatment using more than one anticancer drug. The way the chemotherapy is given depends on the type and stage of the cancer being treated. New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. High-dose chemotherapy with stem cell transplant High-dose chemotherapy with stem cell transplant is a way of giving high doses of chemotherapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type of cancer and whether it has come back. External radiation therapy is being studied for the treatment of childhood extracranial germ cell tumors that have come back. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For childhood extracranial germ cell tumors, alpha-fetoprotein (AFP) tests and beta-human chorionic gonadotropin (-hCG) tests are done to see if treatment is working. Continued high levels of AFP or -hCG may mean the cancer is still growing. For at least 3 years after surgery, follow-up will include regular physical exams, imaging tests, and tumor marker tests. Treatment Options for Childhood Extracranial Germ Cell Tumors Mature and Immature Teratomas Treatment of mature teratomas that are not in the sacrum or coccyx (bottom part of the spine) includes the following: - Surgery to remove the tumor followed by observation. Treatment of immature teratomas that are not in the sacrum or coccyx includes the following: - Surgery to remove the tumor followed by observation for stage I tumors. - Surgery to remove the tumor for stage IIIV tumors. Treatment of immature teratomas that are in the sacrum or coccyx includes the following: - Surgery (removal of the sacrum and coccyx) followed by observation. Sometimes a mature or immature teratoma also has malignant cells. The teratoma and malignant cells may need to be treated differently. Regular follow-up exams with imaging tests and the alpha-fetoprotein (AFP) tumor marker test will be done for at least 3 years. Check the list of NCI-supported cancer clinical trials that are now accepting patients with childhood teratoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Malignant Gonadal Germ Cell Tumors Malignant Testicular Germ Cell Tumors Treatment of malignant testicular germ cell tumors may include the following: For boys younger than 15 years: - Surgery (radical inguinal orchiectomy) followed by observation for stage I tumors. - Surgery (radical inguinal orchiectomy) followed by combination chemotherapy for stage II-IV tumors. A second surgery may be done to remove any tumor remaining after chemotherapy. For boys 15 years and older: Malignant testicular germ cell tumors in boys 15 years and older are treated differently than they are in young boys. Surgery may include removal of lymph nodes in the abdomen. (See the PDQ summary on Testicular Cancer Treatment for more information.) Check the list of NCI-supported cancer clinical trials that are now accepting patients with childhood malignant testicular germ cell tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Malignant Ovarian Germ Cell Tumors Dysgerminomas Treatment of stage I dysgerminomas in young girls may include the following: - Surgery (unilateral salpingo-oophorectomy) followed by observation. Combination chemotherapy may be given if the tumor comes back. Treatment of stages IIIV dysgerminomas in young girls may include the following: - Surgery (unilateral salpingo-oophorectomy) followed by combination chemotherapy. - Combination chemotherapy to shrink the tumor, followed by surgery (unilateral salpingo-oophorectomy). Nongerminomas Treatment of stage I nongerminomas in young girls may include the following: - Surgery followed by observation. - Surgery followed by combination chemotherapy. Treatment of stages IIIV nongerminomas in young girls may include the following: - Surgery followed by combination chemotherapy. A second surgery may be done to remove any remaining cancer. - Biopsy followed by combination chemotherapy to shrink the tumor and sometimes surgery for tumors that cannot be removed by surgery when cancer is diagnosed. The treatment for adolescents and young adults with ovarian germ cell tumor is much like the treatment for adults. (See the PDQ treatment summary on Ovarian Germ Cell Tumors for more information.) Check the list of NCI-supported cancer clinical trials that are now accepting patients with childhood malignant ovarian germ cell tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Malignant Extragonadal Extracranial Germ Cell Tumors Treatment of childhood malignant extragonadal extracranial germ cell tumors may include the following: - Combination chemotherapy to shrink the tumor followed by surgery to remove the sacrum and coccyx (bottom part of the spine) for tumors that are in the sacrum or coccyx. - Combination chemotherapy to shrink the tumor followed by surgery to remove tumors that are in the mediastinum. - Biopsy followed by combination chemotherapy to shrink the tumor and surgery to remove tumors that are in the abdomen. - Surgery to remove the tumor followed by combination chemotherapy for tumors of the head and neck. Treatment of malignant extragonadal extracranial germ cell tumors in places not already described includes the following: - Surgery followed by combination chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with childhood extragonadal germ cell tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Recurrent Childhood Malignant Extracranial Germ Cell Tumors There is no standard treatment for recurrent childhood malignant extracranial germ cell tumors. Treatment depends on the following: - The type of treatment given when the cancer was diagnosed. - How the tumor responded to the initial treatment. Treatment is usually within a clinical trial and may include the following: - Surgery. - Surgery followed by combination chemotherapy, for most malignant extracranial germ cell tumors including immature teratomas, malignant testicular germ cell tumors, and malignant ovarian germ cell tumors. - Surgery for tumors that come back in the sacrum or coccyx (bottom part of the spine), if surgery to remove the sacrum and coccyx was not done when the cancer was diagnosed. Chemotherapy may be given before surgery, to shrink the tumor. If any tumor remains after surgery, radiation therapy may also be given. - Combination chemotherapy for stage I malignant testicular germ cell tumors and stage I ovarian dysgerminomas. - High-dose chemotherapy and stem cell transplant. - Radiation therapy followed by surgery to remove the tumor in the brain for cancer that has spread to the brain. - A clinical trial of combination chemotherapy alone compared with high-dose chemotherapy followed by stem cell transplant. Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent childhood malignant germ cell tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website.",Childhood Extracranial Germ Cell Tumors,0000015_1,CancerGov,https://www.cancer.gov/types/extracranial-germ-cell/patient/germ-cell-treatment-pdq,C0205851,T191,Disorders what research (or clinical trials) is being done for Childhood Extracranial Germ Cell Tumors ?,0000015_1-9,research,"New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. High-dose chemotherapy with stem cell transplant High-dose chemotherapy with stem cell transplant is a way of giving high doses of chemotherapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type of cancer and whether it has come back. External radiation therapy is being studied for the treatment of childhood extracranial germ cell tumors that have come back. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Childhood Extracranial Germ Cell Tumors,0000015_1,CancerGov,https://www.cancer.gov/types/extracranial-germ-cell/patient/germ-cell-treatment-pdq,C0205851,T191,Disorders What is (are) Extragonadal Germ Cell Tumors ?,0000016_1-1,information,"Key Points - Extragonadal germ cell tumors form from developing sperm or egg cells that travel from the gonads to other parts of the body. - Age and gender can affect the risk of extragonadal germ cell tumors. - Signs and symptoms of extragonadal germ cell tumors include breathing problems and chest pain. - Imaging and blood tests are used to detect (find) and diagnose extragonadal germ cell tumors. - Certain factors affect prognosis (chance of recovery) and treatment options. Extragonadal germ cell tumors form from developing sperm or egg cells that travel from the gonads to other parts of the body. "" Extragonadal"" means outside of the gonads (sex organs). When cells that are meant to form sperm in the testicles or eggs in the ovaries travel to other parts of the body, they may grow into extragonadal germ cell tumors. These tumors may begin to grow anywhere in the body but usually begin in organs such as the pineal gland in the brain, in the mediastinum (area between the lungs), or in the retroperitoneum (the back wall of the abdomen). Extragonadal germ cell tumors can be benign (noncancer) or malignant (cancer). Benign extragonadal germ cell tumors are called benign teratomas. These are more common than malignant extragonadal germ cell tumors and often are very large. Malignant extragonadal germ cell tumors are divided into two types, nonseminoma and seminoma. Nonseminomas tend to grow and spread more quickly than seminomas. They usually are large and cause signs and symptoms. If untreated, malignant extragonadal germ cell tumors may spread to the lungs, lymph nodes, bones, liver, or other parts of the body. For information about germ cell tumors in the ovaries and testicles, see the following PDQ summaries: - Ovarian Germ Cell Tumors Treatment - Testicular Cancer Treatment",Extragonadal Germ Cell Tumors,0000016_1,CancerGov,https://www.cancer.gov/types/extragonadal-germ-cell/patient/extragonadal-treatment-pdq,C0262963,T191,Disorders Who is at risk for Extragonadal Germ Cell Tumors? ?,0000016_1-2,susceptibility,Age and gender can affect the risk of extragonadal germ cell tumors. Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesnt mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for malignant extragonadal germ cell tumors include the following: - Being male. - Being age 20 or older. - Having Klinefelter syndrome.,Extragonadal Germ Cell Tumors,0000016_1,CancerGov,https://www.cancer.gov/types/extragonadal-germ-cell/patient/extragonadal-treatment-pdq,C0262963,T191,Disorders What are the symptoms of Extragonadal Germ Cell Tumors ?,0000016_1-3,symptoms,Signs and symptoms of extragonadal germ cell tumors include breathing problems and chest pain. Malignant extragonadal germ cell tumors may cause signs and symptoms as they grow into nearby areas. Other conditions may cause the same signs and symptoms. Check with your doctor if you have any of the following: - Chest pain. - Breathing problems. - Cough. - Fever. - Headache. - Change in bowel habits. - Feeling very tired. - Trouble walking. - Trouble in seeing or moving the eyes.,Extragonadal Germ Cell Tumors,0000016_1,CancerGov,https://www.cancer.gov/types/extragonadal-germ-cell/patient/extragonadal-treatment-pdq,C0262963,T191,Disorders How to diagnose Extragonadal Germ Cell Tumors ?,0000016_1-4,exams and tests,"Imaging and blood tests are used to detect (find) and diagnose extragonadal germ cell tumors. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. The testicles may be checked for lumps, swelling, or pain. A history of the patient's health habits and past illnesses and treatments will also be taken. - Chest x-ray : An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. - Serum tumor marker test : A procedure in which a sample of blood is examined to measure the amounts of certain substances released into the blood by organs, tissues, or tumor cells in the body. Certain substances are linked to specific types of cancer when found in increased levels in the blood. These are called tumor markers. The following three tumor markers are used to detect extragonadal germ cell tumor: - Alpha-fetoprotein (AFP). - Beta-human chorionic gonadotropin (-hCG). - Lactate dehydrogenase (LDH). Blood levels of the tumor markers help determine if the tumor is a seminoma or nonseminoma. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. Sometimes a CT scan and a PET scan are done at the same time. A PET scan is a procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. When a PET scan and CT scan are done at the same time, it is called a PET-CT. - Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. The type of biopsy used depends on where the extragonadal germ cell tumor is found. - Excisional biopsy : The removal of an entire lump of tissue. - Incisional biopsy : The removal of part of a lump or sample of tissue. - Core biopsy : The removal of tissue using a wide needle. - Fine-needle aspiration (FNA) biopsy : The removal of tissue or fluid using a thin needle.",Extragonadal Germ Cell Tumors,0000016_1,CancerGov,https://www.cancer.gov/types/extragonadal-germ-cell/patient/extragonadal-treatment-pdq,C0262963,T191,Disorders What is the outlook for Extragonadal Germ Cell Tumors ?,0000016_1-5,outlook,"Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - Whether the tumor is nonseminoma or seminoma. - The size of the tumor and where it is in the body. - The blood levels of AFP, -hCG, and LDH. - Whether the tumor has spread to other parts of the body. - The way the tumor responds to initial treatment. - Whether the tumor has just been diagnosed or has recurred (come back).",Extragonadal Germ Cell Tumors,0000016_1,CancerGov,https://www.cancer.gov/types/extragonadal-germ-cell/patient/extragonadal-treatment-pdq,C0262963,T191,Disorders What are the stages of Extragonadal Germ Cell Tumors ?,0000016_1-6,stages,"Key Points - After an extragonadal germ cell tumor has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - The following prognostic groups are used for extragonadal germ cell tumors: - Good prognosis - Intermediate prognosis - Poor prognosis After an extragonadal germ cell tumor has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body. The extent or spread of cancer is usually described as stages. For extragonadal germ cell tumors, prognostic groups are used instead of stages. The tumors are grouped according to how well the cancer is expected to respond to treatment. It is important to know the prognostic group in order to plan treatment. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of tumor as the primary tumor. For example, if an extragonadal germ cell tumor spreads to the lung, the tumor cells in the lung are actually cancerous germ cells. The disease is metastatic extragonadal germ cell tumor, not lung cancer. The following prognostic groups are used for extragonadal germ cell tumors: Good prognosis A nonseminoma extragonadal germ cell tumor is in the good prognosis group if: - the tumor is in the back of the abdomen; and - the tumor has not spread to organs other than the lungs; and - the levels of tumor markers AFP and -hCG are normal and LDH is slightly above normal. A seminoma extragonadal germ cell tumor is in the good prognosis group if: - the tumor has not spread to organs other than the lungs; and - the level of AFP is normal; -hCG and LDH may be at any level. Intermediate prognosis A nonseminoma extragonadal germ cell tumor is in the intermediate prognosis group if: - the tumor is in the back of the abdomen; and - the tumor has not spread to organs other than the lungs; and - the level of any one of the tumor markers (AFP, -hCG, or LDH) is more than slightly above normal. A seminoma extragonadal germ cell tumor is in the intermediate prognosis group if: - the tumor has spread to organs other than the lungs; and - the level of AFP is normal; -hCG and LDH may be at any level. Poor prognosis A nonseminoma extragonadal germ cell tumor is in the poor prognosis group if: - the tumor is in the chest; or - the tumor has spread to organs other than the lungs; or - the level of any one of the tumor markers (AFP, -hCG, or LDH) is high. Seminoma extragonadal germ cell tumor does not have a poor prognosis group.",Extragonadal Germ Cell Tumors,0000016_1,CancerGov,https://www.cancer.gov/types/extragonadal-germ-cell/patient/extragonadal-treatment-pdq,C0262963,T191,Disorders What are the treatments for Extragonadal Germ Cell Tumors ?,0000016_1-7,treatment,"Key Points - There are different types of treatment for patients with extragonadal germ cell tumors. - Three types of standard treatment are used: - Radiation therapy - Chemotherapy - Surgery - New types of treatment are being tested in clinical trials. - High-dose chemotherapy with stem cell transplant - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with extragonadal germ cell tumors. Different types of treatments are available for patients with extragonadal germ cell tumors. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Three types of standard treatment are used: Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External radiation therapy is used to treat seminoma. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly in the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. Surgery Patients who have benign tumors or tumor remaining after chemotherapy or radiation therapy may need to have surgery. New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. High-dose chemotherapy with stem cell transplant High-dose chemotherapy with stem cell transplant is a method of giving high doses of chemotherapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. After initial treatment for extragonadal germ cell tumors, blood levels of AFP and other tumor markers continue to be checked to find out how well the treatment is working. Treatment Options for Extragonadal Germ Cell Tumors Benign Teratoma Treatment of benign teratomas is surgery. Check the list of NCI-supported cancer clinical trials that are now accepting patients with benign teratoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Seminoma Treatment of seminoma extragonadal germ cell tumors may include the following: - Radiation therapy for small tumors in one area, followed by watchful waiting if there is tumor remaining after treatment. - Chemotherapy for larger tumors or tumors that have spread. If a tumor smaller than 3 centimeters remains after chemotherapy, watchful waiting follows. If a larger tumor remains after treatment, surgery or watchful waiting follow. Check the list of NCI-supported cancer clinical trials that are now accepting patients with extragonadal seminoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Nonseminoma Treatment of nonseminoma extragonadal germ cell tumors may include the following: - Combination chemotherapy followed by surgery to remove any remaining tumor. - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with malignant extragonadal non-seminomatous germ cell tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Recurrent or Refractory Extragonadal Germ Cell Tumors Treatment of extragonadal germ cell tumors that are recurrent (come back after being treated) or refractory (do not get better during treatment) may include the following: - Chemotherapy. - A clinical trial of high-dose chemotherapy with stem cell transplant. - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent extragonadal germ cell tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Extragonadal Germ Cell Tumors,0000016_1,CancerGov,https://www.cancer.gov/types/extragonadal-germ-cell/patient/extragonadal-treatment-pdq,C0262963,T191,Disorders what research (or clinical trials) is being done for Extragonadal Germ Cell Tumors ?,0000016_1-8,research,"New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. High-dose chemotherapy with stem cell transplant High-dose chemotherapy with stem cell transplant is a method of giving high doses of chemotherapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Extragonadal Germ Cell Tumors,0000016_1,CancerGov,https://www.cancer.gov/types/extragonadal-germ-cell/patient/extragonadal-treatment-pdq,C0262963,T191,Disorders What is (are) Intraocular (Uveal) Melanoma ?,0000017_1-1,information,"Key Points - Intraocular melanoma is a disease in which malignant (cancer) cells form in the tissues of the eye. - Being older and having fair skin may increase the risk of intraocular melanoma. - Signs of intraocular melanoma include blurred vision or a dark spot on the iris. - Tests that examine the eye are used to help detect (find) and diagnose intraocular melanoma. - A biopsy of the tumor is rarely needed to diagnose intraocular melanoma. - Certain factors affect prognosis (chance of recovery) and treatment options. Intraocular melanoma is a disease in which malignant (cancer) cells form in the tissues of the eye. Intraocular melanoma begins in the middle of three layers of the wall of the eye. The outer layer includes the white sclera (the ""white of the eye"") and the clear cornea at the front of the eye. The inner layer has a lining of nerve tissue, called the retina, which senses light and sends images along the optic nerve to the brain. The middle layer, where intraocular melanoma forms, is called the uvea or uveal tract, and has three main parts: - Iris The iris is the colored area at the front of the eye (the ""eye color""). It can be seen through the clear cornea. The pupil is in the center of the iris and it changes size to let more or less light into the eye. Intraocular melanoma of the iris is usually a small tumor that grows slowly and rarely spreads to other parts of the body. - Ciliary body The ciliary body is a ring of tissue with muscle fibers that change the size of the pupil and the shape of the lens. It is found behind the iris. Changes in the shape of the lens help the eye focus. The ciliary body also makes the clear fluid that fills the space between the cornea and the iris. Intraocular melanoma of the ciliary body is often larger and more likely to spread to other parts of the body than intraocular melanoma of the iris. - Choroid The choroid is a layer of blood vessels that bring oxygen and nutrients to the eye. Most intraocular melanomas begin in the choroid. Intraocular melanoma of the choroid is often larger and more likely to spread to other parts of the body than intraocular melanoma of the iris. Intraocular melanoma is a rare cancer that forms from cells that make melanin in the iris, ciliary body, and choroid. It is the most common eye cancer in adults.",Intraocular (Uveal) Melanoma,0000017_1,CancerGov,https://www.cancer.gov/types/eye/patient/intraocular-melanoma-treatment-pdq,C0025202,T191,Disorders Who is at risk for Intraocular (Uveal) Melanoma? ?,0000017_1-2,susceptibility,"Being older and having fair skin may increase the risk of intraocular melanoma. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesnt mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for intraocular melanoma include the following: - Having a fair complexion, which includes the following: - Fair skin that freckles and burns easily, does not tan, or tans poorly. - Blue or green or other light-colored eyes. - Older age. - Being white.",Intraocular (Uveal) Melanoma,0000017_1,CancerGov,https://www.cancer.gov/types/eye/patient/intraocular-melanoma-treatment-pdq,C0025202,T191,Disorders What are the symptoms of Intraocular (Uveal) Melanoma ?,0000017_1-3,symptoms,Signs of intraocular melanoma include blurred vision or a dark spot on the iris. Intraocular melanoma may not cause early signs or symptoms. It is sometimes found during a regular eye exam when the doctor dilates the pupil and looks into the eye. Signs and symptoms may be caused by intraocular melanoma or by other conditions. Check with your doctor if you have any of the following: - Blurred vision or other change in vision. - Floaters (spots that drift in your field of vision) or flashes of light. - A dark spot on the iris. - A change in the size or shape of the pupil. - A change in the position of the eyeball in the eye socket.,Intraocular (Uveal) Melanoma,0000017_1,CancerGov,https://www.cancer.gov/types/eye/patient/intraocular-melanoma-treatment-pdq,C0025202,T191,Disorders How to diagnose Intraocular (Uveal) Melanoma ?,0000017_1-4,exams and tests,"Tests that examine the eye are used to help detect (find) and diagnose intraocular melanoma. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Eye exam with dilated pupil: An exam of the eye in which the pupil is dilated (enlarged) with medicated eye drops to allow the doctor to look through the lens and pupil to the retina. The inside of the eye, including the retina and the optic nerve, is checked. Pictures may be taken over time to keep track of changes in the size of the tumor. There are several types of eye exams: - Ophthalmoscopy : An exam of the inside of the back of the eye to check the retina and optic nerve using a small magnifying lens and a light. - Slit-lamp biomicroscopy : An exam of the inside of the eye to check the retina, optic nerve, and other parts of the eye using a strong beam of light and a microscope. - Gonioscopy : An exam of the front part of the eye between the cornea and iris. A special instrument is used to see if the area where fluid drains out of the eye is blocked. - Ultrasound exam of the eye: A procedure in which high-energy sound waves (ultrasound) are bounced off the internal tissues of the eye to make echoes. Eye drops are used to numb the eye and a small probe that sends and receives sound waves is placed gently on the surface of the eye. The echoes make a picture of the inside of the eye and the distance from the cornea to the retina is measured. The picture, called a sonogram, shows on the screen of the ultrasound monitor. - High-resolution ultrasound biomicroscopy : A procedure in which high-energy sound waves (ultrasound) are bounced off the internal tissues of the eye to make echoes. Eye drops are used to numb the eye and a small probe that sends and receives sound waves is placed gently on the surface of the eye. The echoes make a more detailed picture of the inside of the eye than a regular ultrasound. The tumor is checked for its size, shape, and thickness, and for signs that the tumor has spread to nearby tissue. - Transillumination of the globe and iris: An exam of the iris, cornea, lens, and ciliary body with a light placed on either the upper or lower lid. - Fluorescein angiography : A procedure to look at blood vessels and the flow of blood inside the eye. An orange fluorescent dye (fluorescein) is injected into a blood vessel in the arm and goes into the bloodstream. As the dye travels through blood vessels of the eye, a special camera takes pictures of the retina and choroid to find any areas that are blocked or leaking. - Indocyanine green angiography: A procedure to look at blood vessels in the choroid layer of the eye. A green dye (indocyanine green) is injected into a blood vessel in the arm and goes into the bloodstream. As the dye travels through blood vessels of the eye, a special camera takes pictures of the retina and choroid to find any areas that are blocked or leaking. - Ocular coherence tomography : An imaging test that uses light waves to take cross-section pictures of the retina, and sometimes the choroid, to see if there is swelling or fluid beneath the retina. A biopsy of the tumor is rarely needed to diagnose intraocular melanoma. A biopsy is the removal of cells or tissues so they can be viewed under a microscope to check for signs of cancer. Rarely, a biopsy of the tumor is needed to diagnose intraocular melanoma. Tissue that is removed during a biopsy or surgery to remove the tumor may be tested to get more information about prognosis and which treatment options are best. The following tests may be done on the sample of tissue: - Cytogenetic analysis: A laboratory test in which cells in a sample of tissue are viewed under a microscope to look for certain changes in the chromosomes. - Gene expression profiling : A laboratory test in which cells in a sample of tissue are checked for certain types of RNA. A biopsy may result in retinal detachment (the retina separates from other tissues in the eye). This can be repaired by surgery.",Intraocular (Uveal) Melanoma,0000017_1,CancerGov,https://www.cancer.gov/types/eye/patient/intraocular-melanoma-treatment-pdq,C0025202,T191,Disorders What is the outlook for Intraocular (Uveal) Melanoma ?,0000017_1-5,outlook,"Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - How the melanoma cells look under a microscope. - The size and thickness of the tumor. - The part of the eye the tumor is in (the iris, ciliary body, or choroid). - Whether the tumor has spread within the eye or to other places in the body. - Whether there are certain changes in the genes linked to intraocular melanoma. - The patient's age and general health. - Whether the tumor has recurred (come back) after treatment.",Intraocular (Uveal) Melanoma,0000017_1,CancerGov,https://www.cancer.gov/types/eye/patient/intraocular-melanoma-treatment-pdq,C0025202,T191,Disorders What are the stages of Intraocular (Uveal) Melanoma ?,0000017_1-6,stages,"Key Points - After intraocular melanoma has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body. - The following sizes are used to describe intraocular melanoma: - Small - Medium - Large - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - There are two staging systems for intraocular melanoma. - The following stages are used for intraocular melanoma of the iris: - Stage I - Stage II - Stage III - Stage IV - The following stages are used for intraocular melanoma of the ciliary body and choroid: - Stage I - Stage II - Stage III - Stage IV After intraocular melanoma has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body. The process used to find out if cancer has spread to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following tests and procedures may be used in the staging process: - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. - Liver function tests : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by the liver. A higher than normal amount of a substance can be a sign the cancer has spread to the liver. - Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs, such as the liver, and make echoes. The echoes form a picture of body tissues called a sonogram. - Chest x-ray : An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the chest, abdomen, or pelvis, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A very small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. Sometimes a PET scan and a CT scan are done at the same time. If there is any cancer, this increases the chance that it will be found. The following sizes are used to describe intraocular melanoma: Small The tumor is 5 to 16 millimeters in diameter and from 1 to 3 millimeters thick. Medium The tumor is 16 millimeters or smaller in diameter and from 3.1 to 8 millimeters thick. Large The tumor is: - more than 8 millimeters thick and any diameter; or - at least 2 millimeters thick and more than 16 millimeters in diameter. Though most intraocular melanoma tumors are raised, some are flat. These diffuse tumors grow widely across the uvea. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if intraocular melanoma spreads to the liver, the cancer cells in the liver are actually intraocular melanoma cells. The disease is metastatic intraocular melanoma, not liver cancer. There are two staging systems for intraocular melanoma. Intraocular melanoma has two staging systems. The staging system used depends on where in the eye the cancer first formed: - Iris. - Ciliary body and choroid. If intraocular melanoma spreads to the optic nerve or nearby tissue of the eye socket, it is called extraocular extension. The following stages are used for intraocular melanoma of the iris: Stage I In stage I, the tumor is in the iris only and is not more than one fourth the size of the iris. Stage II Stage II is divided into stages IIA and IIB. - In stage IIA, the tumor: - is in the iris only and is more than one fourth the size of the iris; or - is in the iris only and has caused glaucoma; or - has spread next to and/or into the ciliary body, choroid, or both. The tumor has caused glaucoma. - In stage IIB, the tumor has spread next to and/or into the ciliary body, choroid, or both, and has also spread into the sclera. The tumor has caused glaucoma. Stage III Stage III is divided into stages IIIA and IIIB. - In stage IIIA, the tumor has spread through the sclera to the outside of the eyeball. The part of the tumor outside the eyeball is not more than 5 millimeters thick. - In stage IIIB, the tumor has spread through the sclera to the outside of the eyeball. The part of the tumor outside the eyeball is more than 5 millimeters thick. Stage IV In stage IV, the tumor may be any size and has spread: - to nearby lymph nodes; or - to other parts of the body, such as the liver, lung, or bone, or to areas under the skin. The following stages are used for intraocular melanoma of the ciliary body and choroid: Intraocular melanoma of the ciliary body and choroid is grouped into four size categories. The category depends on how wide and thick the tumor is. Category 1 tumors are the smallest and category 4 tumors are the biggest. Category 1: - The tumor is not more than 12 millimeters wide and not more than 3 millimeters thick; or - the tumor is not more than 9 millimeters wide and 3.1 to 6 millimeters thick. Category 2: - The tumor is 12.1 to 18 millimeters wide and not more than 3 millimeters thick; or - the tumor is 9.1 to 15 millimeters wide and 3.1 to 6 millimeters thick; or - the tumor is not more than 12 millimeters wide and 6.1 to 9 millimeters thick. Category 3: - The tumor is 15.1 to 18 millimeters wide and 3.1 to 6 millimeters thick; or - the tumor is 12.1 to 18 millimeters wide and 6.1 to 9 millimeters thick; or - the tumor is 3.1 to 18 millimeters wide and 9.1 to 12 millimeters thick; or - the tumor is 9.1 to 15 millimeters wide and 12.1 to 15 millimeters thick. Category 4: - The tumor is more than 18 millimeters wide and may be any thickness; or - the tumor is 15.1 to 18 millimeters wide and more than 12 millimeters thick; or - the tumor is 12.1 to 15 millimeters wide and more than 15 millimeters thick. Stage I In stage I, the tumor is size category 1 and is in the choroid only. Stage II Stage II is divided into stages IIA and IIB. - In stage IIA, the tumor: - is size category 1 and has spread to the ciliary body; or - is size category 1 and has spread through the sclera to the outside of the eyeball. The part of the tumor outside the eyeball is not more than 5 millimeters thick. The tumor may have spread to the ciliary body; or - is size category 2 and is in the choroid only. - In stage IIB, the tumor: - is size category 2 and has spread to the ciliary body; or - is size category 3 and is in the choroid only. Stage III Stage III is divided into stages IIIA, IIIB, and IIIC. - In stage IIIA, the tumor: - is size category 2 and has spread through the sclera to the outside of the eyeball. The part of the tumor outside the eyeball is not more than 5 millimeters thick. The tumor may have spread to the ciliary body; or - is size category 3 and has spread to the ciliary body; or - is size category 3 and has spread through the sclera to the outside of the eyeball. The part of the tumor outside the eyeball is not more than 5 millimeters thick. The tumor has not spread to the ciliary body; or - is size category 4 and is in the choroid only. - In stage IIIB, the tumor: - is size category 3 and has spread through the sclera to the outside of the eyeball. The part of the tumor outside the eyeball is not more than 5 millimeters thick. The tumor has spread to the ciliary body; or - is size category 4 and has spread to the ciliary body; or - is size category 4 and has spread through the sclera to the outside of the eyeball. The part of the tumor outside the eyeball is not more than 5 millimeters thick. The tumor has not spread to the ciliary body. - In stage IIIC, the tumor: - is size category 4 and has spread through the sclera to the outside of the eyeball. The part of the tumor outside the eyeball is not more than 5 millimeters thick. The tumor has spread to the ciliary body; or - may be any size and has spread through the sclera to the outside of the eyeball. The part of the tumor outside the eyeball is more than 5 millimeters thick. The tumor has not spread to the ciliary body. Stage IV In stage IV, the tumor may be any size and has spread: - to nearby lymph nodes; or - to other parts of the body, such as the liver, lung, or bone, or to areas under the skin.",Intraocular (Uveal) Melanoma,0000017_1,CancerGov,https://www.cancer.gov/types/eye/patient/intraocular-melanoma-treatment-pdq,C0025202,T191,Disorders What are the treatments for Intraocular (Uveal) Melanoma ?,0000017_1-7,treatment,"Key Points - There are different types of treatments for patients with intraocular melanoma. - Five types of standard treatment are used: - Surgery - Watchful Waiting - Radiation therapy - Photocoagulation - Thermotherapy - New types of treatment are being tested in clinical trials. - Treatment for intraocular (uveal) melanoma may cause side effects. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatments for patients with intraocular melanoma. Different types of treatments are available for patients with intraocular melanoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Five types of standard treatment are used: Surgery Surgery is the most common treatment for intraocular melanoma. The following types of surgery may be used: - Resection: Surgery to remove the tumor and a small amount of healthy tissue around it. - Enucleation: Surgery to remove the eye and part of the optic nerve. This is done if vision cannot be saved and the tumor is large, has spread to the optic nerve, or causes high pressure inside the eye. After surgery, the patient is usually fitted for an artificial eye to match the size and color of the other eye. - Exenteration: Surgery to remove the eye and eyelid, and muscles, nerves, and fat in the eye socket. After surgery, the patient may be fitted for an artificial eye to match the size and color of the other eye or a facial prosthesis. Watchful Waiting Watchful waiting is closely monitoring a patients condition without giving any treatment until signs or symptoms appear or change. Pictures are taken over time to keep track of changes in the size of the tumor and how fast it is growing. Watchful waiting is used for patients who do not have signs or symptoms and the tumor is not growing. It is also used when the tumor is in the only eye with useful vision. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. Certain ways of giving radiation therapy can help keep radiation from damaging nearby healthy tissue. These types of external radiation therapy include the following: - Charged-particle external beam radiation therapy is a type of external-beam radiation therapy. A special radiation therapy machine aims tiny, invisible particles, called protons or helium ions, at the cancer cells to kill them with little damage to nearby normal tissues. Charged-particle radiation therapy uses a different type of radiation than the x-ray type of radiation therapy. - Gamma Knife therapy is a type of stereotactic radiosurgery used for some melanomas. This treatment can be given in one treatment. It aims tightly focused gamma rays directly at the tumor so there is little damage to healthy tissue. Gamma Knife therapy does not use a knife to remove the tumor and is not an operation. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. Certain ways of giving radiation therapy can help keep radiation from damaging healthy tissue. This type of internal radiation therapy may include the following: - Localized plaque radiation therapy is a type of internal radiation therapy that may be used for tumors of the eye. Radioactive seeds are attached to one side of a disk, called a plaque, and placed directly on the outside wall of the eye near the tumor. The side of the plaque with the seeds on it faces the eyeball, aiming radiation at the tumor. The plaque helps protect other nearby tissue from the radiation. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External and internal radiation therapy are used to treat intraocular melanoma. Photocoagulation Photocoagulation is a procedure that uses laser light to destroy blood vessels that bring nutrients to the tumor, causing the tumor cells to die. Photocoagulation may be used to treat small tumors. This is also called light coagulation. Thermotherapy Thermotherapy is the use of heat from a laser to destroy cancer cells and shrink the tumor. New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Treatment for intraocular (uveal) melanoma may cause side effects. For information about side effects caused by treatment for cancer, see our Side Effects page. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Intraocular (Uveal) Melanoma Iris Melanoma Treatment of iris melanoma may include the following: - Watchful waiting. - Surgery (resection or enucleation). - Plaque radiation therapy, for tumors that cannot be removed by surgery. Check the list of NCI-supported cancer clinical trials that are now accepting patients with iris melanoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Ciliary Body Melanoma Treatment of tumors in the ciliary body and choroid may include the following: - Plaque radiation therapy. - Charged-particle external-beam radiation therapy. - Surgery (resection or enucleation). Check the list of NCI-supported cancer clinical trials that are now accepting patients with ciliary body and choroid melanoma, small size. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Choroid Melanoma Treatment of small choroid melanoma may include the following: - Watchful waiting. - Plaque radiation therapy. - Charged-particle external-beam radiation therapy. - Gamma Knife therapy. - Thermotherapy. - Surgery (resection or enucleation). Treatment of medium choroid melanoma may include the following: - Plaque radiation therapy with or without photocoagulation or thermotherapy. - Charged-particle external-beam radiation therapy. - Surgery (resection or enucleation). Treatment of large choroid melanoma may include the following: - Enucleation when the tumor is too large for treatments that save the eye. Check the list of NCI-supported cancer clinical trials that are now accepting patients with ciliary body and choroid melanoma, small size. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Extraocular Extension Melanoma and Metastatic Intraocular (Uveal) Melanoma Treatment of extraocular extension melanoma that has spread to the bone around the eye may include the following: - Surgery (exenteration). - A clinical trial. An effective treatment for metastatic intraocular melanoma has not been found. A clinical trial may be a treatment option. Talk with your doctor about your treatment options. Check the list of NCI-supported cancer clinical trials that are now accepting patients with extraocular extension melanoma and metastatic intraocular melanoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Recurrent Intraocular (Uveal) Melanoma An effective treatment for recurrent intraocular melanoma has not been found. A clinical trial may be a treatment option. Talk with your doctor about your treatment options. Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent intraocular melanoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Intraocular (Uveal) Melanoma,0000017_1,CancerGov,https://www.cancer.gov/types/eye/patient/intraocular-melanoma-treatment-pdq,C0025202,T191,Disorders what research (or clinical trials) is being done for Intraocular (Uveal) Melanoma ?,0000017_1-8,research,"New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Intraocular (Uveal) Melanoma,0000017_1,CancerGov,https://www.cancer.gov/types/eye/patient/intraocular-melanoma-treatment-pdq,C0025202,T191,Disorders What is (are) Retinoblastoma ?,0000018_1-1,information,"Key Points - Retinoblastoma is a disease in which malignant (cancer) cells form in the tissues of the retina. - Retinoblastoma occurs in heritable and nonheritable forms. - Treatment for both forms of retinoblastoma should include genetic counseling. - Children with a family history of retinoblastoma should have eye exams to check for retinoblastoma. - A child who has heritable retinoblastoma has an increased risk of trilateral retinoblastoma and other cancers. - Signs and symptoms of retinoblastoma include ""white pupil"" and eye pain or redness. - Tests that examine the retina are used to detect (find) and diagnose retinoblastoma. - Certain factors affect prognosis (chance of recovery) and treatment options. Retinoblastoma is a disease in which malignant (cancer) cells form in the tissues of the retina. The retina is the nerve tissue that lines the inside of the back of the eye. The retina senses light and sends images to the brain by way of the optic nerve. Although retinoblastoma may occur at any age, it occurs most often in children younger than 2 years. The cancer may be in one eye (unilateral) or in both eyes (bilateral). Retinoblastoma rarely spreads from the eye to nearby tissue or other parts of the body. Cavitary retinoblastoma is a rare type of retinoblastoma in which cavities (hollow spaces) form within the tumor. Treatment for both forms of retinoblastoma should include genetic counseling. Parents should receive genetic counseling (a discussion with a trained professional about the risk of genetic diseases) to discuss genetic testing to check for a mutation (change) in the RB1 gene. Genetic counseling also includes a discussion of the risk of retinoblastoma for the child and the child's brothers or sisters.",Retinoblastoma,0000018_1,CancerGov,https://www.cancer.gov/types/retinoblastoma/patient/retinoblastoma-treatment-pdq,C0035335,T191,Disorders Is Retinoblastoma inherited ?,0000018_1-2,inheritance,"Retinoblastoma occurs in heritable and nonheritable forms. A child is thought to have the heritable form of retinoblastoma when one of the following is true: - There is a family history of retinoblastoma. - There is a certain mutation (change) in the RB1 gene. The mutation in the RB1 gene may be passed from the parent to the child or it may occur in the egg or sperm before conception or soon after conception. - There is more than one tumor in the eye or there is a tumor in both eyes. - There is a tumor in one eye and the child is younger than 1 year. After heritable retinoblastoma has been diagnosed and treated, new tumors may continue to form for a few years. Regular eye exams to check for new tumors are usually done every 2 to 4 months for at least 28 months. Nonheritable retinoblastoma is retinoblastoma that is not the heritable form. Most cases of retinoblastoma are the nonheritable form. Treatment for both forms of retinoblastoma should include genetic counseling. Parents should receive genetic counseling (a discussion with a trained professional about the risk of genetic diseases) to discuss genetic testing to check for a mutation (change) in the RB1 gene. Genetic counseling also includes a discussion of the risk of retinoblastoma for the child and the child's brothers or sisters. Children with a family history of retinoblastoma should have eye exams to check for retinoblastoma. A child with a family history of retinoblastoma should have regular eye exams beginning early in life to check for retinoblastoma, unless it is known that the child does not have the RB1 gene change. Early diagnosis of retinoblastoma may mean the child will need less intense treatment. Brothers or sisters of a child with retinoblastoma should have regular eye exams by an ophthalmologist until age 3 to 5 years, unless it is known that the brother or sister does not have the RB1 gene change. A child who has heritable retinoblastoma has an increased risk of trilateral retinoblastoma and other cancers. A child with heritable retinoblastoma has an increased risk of a pineal tumor in the brain. When retinoblastoma and a brain tumor occur at the same time, it is called trilateral retinoblastoma. The brain tumor is usually diagnosed between 20 and 36 months of age. Regular screening using MRI (magnetic resonance imaging) may be done for a child thought to have heritable retinoblastoma or for a child with retinoblastoma in one eye and a family history of the disease. CT (computerized tomography) scans are usually not used for routine screening in order to avoid exposing the child to ionizing radiation. Heritable retinoblastoma also increases the child's risk of other types of cancer such as lung cancer, bladder cancer, or melanoma in later years. Regular follow-up exams are important.",Retinoblastoma,0000018_1,CancerGov,https://www.cancer.gov/types/retinoblastoma/patient/retinoblastoma-treatment-pdq,C0035335,T191,Disorders Who is at risk for Retinoblastoma? ?,0000018_1-3,susceptibility,"A child who has heritable retinoblastoma has an increased risk of trilateral retinoblastoma and other cancers. A child with heritable retinoblastoma has an increased risk of a pineal tumor in the brain. When retinoblastoma and a brain tumor occur at the same time, it is called trilateral retinoblastoma. The brain tumor is usually diagnosed between 20 and 36 months of age. Regular screening using MRI (magnetic resonance imaging) may be done for a child thought to have heritable retinoblastoma or for a child with retinoblastoma in one eye and a family history of the disease. CT (computerized tomography) scans are usually not used for routine screening in order to avoid exposing the child to ionizing radiation. Heritable retinoblastoma also increases the child's risk of other types of cancer such as lung cancer, bladder cancer, or melanoma in later years. Regular follow-up exams are important.",Retinoblastoma,0000018_1,CancerGov,https://www.cancer.gov/types/retinoblastoma/patient/retinoblastoma-treatment-pdq,C0035335,T191,Disorders What are the symptoms of Retinoblastoma ?,0000018_1-4,symptoms,These and other signs and symptoms may be caused by retinoblastoma or by other conditions. Check with a doctor if your child has any of the following: - Pupil of the eye appears white instead of red when light shines into it. This may be seen in flash photographs of the child. - Eyes appear to be looking in different directions (lazy eye). - Pain or redness in the eye. - Infection around the eye. - Eyeball is larger than normal. - Colored part of the eye and pupil look cloudy.,Retinoblastoma,0000018_1,CancerGov,https://www.cancer.gov/types/retinoblastoma/patient/retinoblastoma-treatment-pdq,C0035335,T191,Disorders How to diagnose Retinoblastoma ?,0000018_1-5,exams and tests,"The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. The doctor will ask if there is a family history of retinoblastoma. - Eye exam with dilated pupil: An exam of the eye in which the pupil is dilated (opened wider) with medicated eye drops to allow the doctor to look through the lens and pupil to the retina. The inside of the eye, including the retina and the optic nerve, is examined with a light. Depending on the age of the child, this exam may be done under anesthesia. There are several types of eye exams that are done with the pupil dilated: - Ophthalmoscopy : An exam of the inside of the back of the eye to check the retina and optic nerve using a small magnifying lens and a light. - Slit-lamp biomicroscopy : An exam of the inside of the eye to check the retina, optic nerve, and other parts of the eye using a strong beam of light and a microscope. - Fluorescein angiography : A procedure to look at blood vessels and the flow of blood inside the eye. An orange fluorescent dye called fluorescein is injected into a blood vessel in the arm and goes into the bloodstream. As the dye travels through blood vessels of the eye, a special camera takes pictures of the retina and choroid to find any blood vessels that are blocked or leaking. - RB1 gene test: A laboratory test in which a sample of blood or tissue is tested for a change in the RB1 gene. - Ultrasound exam of the eye: A procedure in which high-energy sound waves (ultrasound) are bounced off the internal tissues of the eye to make echoes. Eye drops are used to numb the eye and a small probe that sends and receives sound waves is placed gently on the surface of the eye. The echoes make a picture of the inside of the eye and the distance from the cornea to the retina is measured. The picture, called a sonogram, shows on the screen of the ultrasound monitor. The picture can be printed to be looked at later. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body, such as the eye. This procedure is also called nuclear magnetic resonance imaging (NMRI). - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the eye, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. Retinoblastoma can usually be diagnosed without a biopsy. When retinoblastoma is in one eye, it sometimes forms in the other eye. Exams of the unaffected eye are done until it is known if the retinoblastoma is the heritable form.",Retinoblastoma,0000018_1,CancerGov,https://www.cancer.gov/types/retinoblastoma/patient/retinoblastoma-treatment-pdq,C0035335,T191,Disorders What is the outlook for Retinoblastoma ?,0000018_1-6,outlook,"The prognosis (chance of recovery) and treatment options depend on the following: - Whether the cancer is in one or both eyes. - The size and number of tumors. - Whether the tumor has spread to the area around the eye, to the brain, or to other parts of the body. - Whether there are symptoms at the time of diagnosis, for trilateral retinoblastoma. - The age of the child. - How likely it is that vision can be saved in one or both eyes. - Whether a second type of cancer has formed.",Retinoblastoma,0000018_1,CancerGov,https://www.cancer.gov/types/retinoblastoma/patient/retinoblastoma-treatment-pdq,C0035335,T191,Disorders What are the stages of Retinoblastoma ?,0000018_1-7,stages,"Key Points - After retinoblastoma has been diagnosed, tests are done to find out if cancer cells have spread within the eye or to other parts of the body. - The International Retinoblastoma Staging System (IRSS) may be used for staging retinoblastoma. - Stage 0 - Stage I - Stage II - Stage III - Stage IV - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - Treatment for retinoblastoma depends on whether it is intraocular (within the eye) or extraocular (outside the eye). - Intraocular retinoblastoma - Extraocular retinoblastoma (metastatic) After retinoblastoma has been diagnosed, tests are done to find out if cancer cells have spread within the eye or to other parts of the body. The process used to find out if cancer has spread within the eye or to other parts of the body is called staging. The information gathered from the staging process determines whether retinoblastoma is only in the eye (intraocular) or has spread outside the eye (extraocular). It is important to know the stage in order to plan treatment. The results of the tests used to diagnose cancer are often also used to stage the disease. (See the General Information section.) The following tests and procedures may be used in the staging process: - Bone scan : A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones and is detected by a scanner that also takes a picture of the body. Areas of bone with cancer show up brighter in the picture because they take up more radioactive material than normal bone cells do. - Bone marrow aspiration and biopsy : The removal of bone marrow and a small piece of bone by inserting a hollow needle into the hipbone or breastbone. A pathologist views the bone marrow under a microscope to look for signs of cancer. A bone marrow aspiration and biopsy is done if the doctor thinks the cancer has spread outside of the eye. - Lumbar puncture : A procedure used to collect cerebrospinal fluid (CSF) from the spinal column. This is done by placing a needle between two bones in the spine and into the CSF around the spinal cord and removing a sample of the fluid. The sample of CSF is checked under a microscope for signs that the cancer has spread to the brain and spinal cord. This procedure is also called an LP or spinal tap. The International Retinoblastoma Staging System (IRSS) may be used for staging retinoblastoma. There are several staging systems for retinoblastoma. The IRSS stages are based on how much cancer remains after surgery to remove the tumor and whether the cancer has spread. Stage 0 The tumor is in the eye only. The eye has not been removed and the tumor was treated without surgery. Stage I The tumor is in the eye only. The eye has been removed and no cancer cells remain. Stage II The tumor is in the eye only. The eye has been removed and there are cancer cells left that can be seen only with a microscope. Stage III Stage III is divided into stages IIIa and IIIb: - In stage IIIa, cancer has spread from the eye to tissues around the eye socket. - In stage IIIb, cancer has spread from the eye to lymph nodes near the ear or in the neck. Stage IV Stage IV is divided into stages IVa and IVb: - In stage IVa, cancer has spread to the blood but not to the brain or spinal cord. One or more tumors may have spread to other parts of the body such as the bone or liver. - In stage IVb, cancer has spread to the brain or spinal cord. It also may have spread to other parts of the body. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if retinoblastoma spreads to the bone, the cancer cells in the bone are actually retinoblastoma cells. The disease is metastatic retinoblastoma, not bone cancer. Treatment for retinoblastoma depends on whether it is intraocular (within the eye) or extraocular (outside the eye). Intraocular retinoblastoma In intraocular retinoblastoma, cancer is found in one or both eyes and may be in the retina only or may also be in other parts of the eye such as the choroid, ciliary body, or part of the optic nerve. Cancer has not spread to tissues around the outside of the eye or to other parts of the body. Extraocular retinoblastoma (metastatic) In extraocular retinoblastoma, cancer has spread beyond the eye. It may be found in tissues around the eye (orbital retinoblastoma) or it may have spread to the central nervous system (brain and spinal cord) or to other parts of the body such as the liver, bones, bone marrow, or lymph nodes.",Retinoblastoma,0000018_1,CancerGov,https://www.cancer.gov/types/retinoblastoma/patient/retinoblastoma-treatment-pdq,C0035335,T191,Disorders What are the treatments for Retinoblastoma ?,0000018_1-8,treatment,"Key Points - There are different types of treatment for patients with retinoblastoma. - Children with retinoblastoma should have their treatment planned by a team of health care providers who are experts in treating cancer in children. - Treatment for retinoblastoma may cause side effects. - Six types of standard treatment are used: - Cryotherapy - Thermotherapy - Chemotherapy - Radiation therapy - High-dose chemotherapy with stem cell rescue - Surgery (enucleation) - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with retinoblastoma. Different types of treatment are available for patients with retinoblastoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment. Children with retinoblastoma should have their treatment planned by a team of health care providers who are experts in treating cancer in children. The goals of treatment are to save the child's life, to save vision and the eye, and to prevent serious side effects. Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other health care providers who are experts in treating children with eye cancer and who specialize in certain areas of medicine. These may include a pediatric ophthalmologist (children's eye doctor) who has a lot of experience in treating retinoblastoma and the following specialists: - Pediatric surgeon. - Radiation oncologist. - Pediatrician. - Pediatric nurse specialist. - Rehabilitation specialist. - Social worker. - Geneticist or genetic counselor. Treatment for retinoblastoma may cause side effects. For information about side effects that begin during treatment for cancer, see our Side Effects page. Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of treatment for retinoblastoma may include the following: - Physical problems such as seeing or hearing problems or, if the eye is removed, a change in the shape and size of the bone around the eye. - Changes in mood, feelings, thinking, learning, or memory. - Second cancers (new types of cancer), such as lung and bladder cancers, osteosarcoma, soft tissue sarcoma, or melanoma. The following risk factors may increase the risk of having another cancer: - Having the heritable form of retinoblastoma. - Past treatment with radiation therapy, especially before age 1 year. - Having already had a previous second cancer. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. Regular follow-up by health professionals who are experts in diagnosing and treating late effects is important. See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information. Six types of standard treatment are used: Cryotherapy Cryotherapy is a treatment that uses an instrument to freeze and destroy abnormal tissue. This type of treatment is also called cryosurgery. Thermotherapy Thermotherapy is the use of heat to destroy cancer cells. Thermotherapy may be given using a laser beam aimed through the dilated pupil or onto the outside of the eyeball. Thermotherapy may be used alone for small tumors or combined with chemotherapy for larger tumors. This treatment is a type of laser therapy. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. The way the chemotherapy is given depends on the stage of the cancer and where the cancer is in the body. There are different types of chemotherapy: - Systemic chemotherapy: When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body. Systemic chemotherapy is given to shrink the tumor (chemoreduction) and avoid surgery to remove the eye. After chemoreduction, other treatments may include radiation therapy, cryotherapy, laser therapy, or regional chemotherapy. Systemic chemotherapy may also be given to kill any cancer cells that are left after the initial treatment or to patients with retinoblastoma that occurs outside the eye. Treatment given after the initial treatment, to lower the risk that the cancer will come back, is called adjuvant therapy. - Regional chemotherapy: When chemotherapy is placed directly into the cerebrospinal fluid (intrathecal chemotherapy), an organ (such as the eye), or a body cavity, the drugs mainly affect cancer cells in those areas. Several types of regional chemotherapy are used to treat retinoblastoma. - Ophthalmic artery infusion chemotherapy: Ophthalmic artery infusion chemotherapy carries anticancer drugs directly to the eye. A catheter is put into an artery that leads to the eye and the anticancer drug is given through the catheter. After the drug is given, a small balloon may be inserted into the artery to block it and keep most of the anticancer drug trapped near the tumor. This type of chemotherapy may be given as the initial treatment when the tumor is in the eye only or when the tumor has not responded to other types of treatment. Ophthalmic artery infusion chemotherapy is given at special retinoblastoma treatment centers. - Intravitreal chemotherapy: Intravitreal chemotherapy is the injection of anticancer drugs directly into the vitreous humor (jelly-like substance) inside in the eye. It is used to treat cancer that has spread to the vitreous humor and has not responded to treatment or has come back after treatment. See Drugs Approved for Retinoblastoma for more information. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External-beam radiation therapy uses a machine outside the body to send radiation toward the cancer. Certain ways of giving radiation therapy can help keep radiation from damaging nearby healthy tissue. These types of radiation therapy include the following: - Intensity-modulated radiation therapy (IMRT): IMRT is a type of 3-dimensional (3-D) external radiation therapy that uses a computer to make pictures of the size and shape of the tumor. Thin beams of radiation of different intensities (strengths) are aimed at the tumor from many angles. - Proton-beam radiation therapy: Proton-beam therapy is a type of high-energy, external radiation therapy. A radiation therapy machine aims streams of protons (tiny, invisible, positively-charged particles) at the cancer cells to kill them. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. Certain ways of giving radiation therapy can help keep radiation from damaging nearby healthy tissue. This type of internal radiation therapy may include the following: - Plaque radiotherapy: Radioactive seeds are attached to one side of a disk, called a plaque, and placed directly on the outside wall of the eye near the tumor. The side of the plaque with the seeds on it faces the eyeball, aiming radiation at the tumor. The plaque helps protect other nearby tissue from the radiation. The way the radiation therapy is given depends on the type and stage of the cancer being treated and how the cancer responded to other treatments. External and internal radiation therapy are used to treat retinoblastoma. High-dose chemotherapy with stem cell rescue High-dose chemotherapy with stem cell rescue is a way of giving high doses of chemotherapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. See Drugs Approved for Retinoblastoma for more information. Surgery (enucleation) Enucleation is surgery to remove the eye and part of the optic nerve. A sample of the eye tissue that is removed will be checked under a microscope to see if there are any signs that the cancer is likely to spread to other parts of the body. This should be done by an experienced pathologist, who is familiar with retinoblastoma and other diseases of the eye. Enucleation is done if there is little or no chance that vision can be saved and when the tumor is large, did not respond to treatment, or comes back after treatment. The patient will be fitted for an artificial eye. Close follow-up is needed for 2 years or more to check for signs of recurrence in the area around the affected eye and to check the other eye. New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Retinoblastoma Treatment of Unilateral, Bilateral, and Cavitary Retinoblastoma If it is likely that the eye can be saved, treatment may include the following: - Systemic chemotherapy or ophthalmic artery infusion chemotherapy, with or without intravitreal chemotherapy, to shrink the tumor. This may be followed by one or more of the following: - Cryotherapy. - Thermotherapy. - Plaque radiotherapy. - External-beam radiation therapy for bilateral intraocular retinoblastoma that does not respond to other treatments. - A clinical trial of ophthalmic artery infusion for unilateral retinoblastoma that has spread to the vitreous humor. If the tumor is large and it is not likely that the eye can be saved, treatment may include the following: - Surgery (enucleation). After surgery, systemic chemotherapy may be given to lower the risk that the cancer will spread to other parts of the body. When retinoblastoma is in both eyes, the treatment for each eye may be different, depending on the size of the tumor and whether it is likely that the eye can be saved. The dose of systemic chemotherapy is usually based on the eye that has more cancer. Treatment for cavitary retinoblastoma, a type of intraocular retinoblastoma, may include the following: - Systemic chemotherapy or ophthalmic artery infusion chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with intraocular retinoblastoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Treatment of Extraocular Retinoblastoma Treatment for extraocular retinoblastoma that has spread to the area around the eye may include the following: - Systemic chemotherapy and external-beam radiation therapy. - Systemic chemotherapy followed by surgery (enucleation). External-beam radiation therapy and more chemotherapy may be given after surgery. Treatment for extraocular retinoblastoma that has spread to the brain may include the following: - Systemic or intrathecal chemotherapy. - External-beam radiation therapy to the brain and spinal cord. - Chemotherapy followed by high-dose chemotherapy with stem cell rescue. It is not clear whether treatment with chemotherapy, radiation therapy, or high-dose chemotherapy with stem cell rescue helps patients with extraocular retinoblastoma live longer. For trilateral retinoblastoma, treatment may include the following: - Systemic chemotherapy followed by high-dose chemotherapy with stem cell rescue. - Systemic chemotherapy followed by surgery and external-beam radiation therapy. For retinoblastoma that has spread to other parts of the body, but not the brain, treatment may include the following: - Chemotherapy followed by high-dose chemotherapy with stem cell rescue and external-beam radiation therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with extraocular retinoblastoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Treatment of Progressive or Recurrent Retinoblastoma Treatment of progressive or recurrent intraocular retinoblastoma may include the following: - External-beam radiation therapy or plaque radiotherapy. - Cryotherapy. - Thermotherapy. - Systemic chemotherapy or ophthalmic artery infusion chemotherapy. - Intravitreal chemotherapy. - Surgery (enucleation). Treatment of progressive or recurrent extraocular retinoblastoma may include the following: - Systemic chemotherapy and external-beam radiation therapy for retinoblastoma that comes back after surgery to remove the eye. - Systemic chemotherapy followed by high-dose chemotherapy with stem cell rescue and external-beam radiation therapy. - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent retinoblastoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website.",Retinoblastoma,0000018_1,CancerGov,https://www.cancer.gov/types/retinoblastoma/patient/retinoblastoma-treatment-pdq,C0035335,T191,Disorders what research (or clinical trials) is being done for Retinoblastoma ?,0000018_1-9,research,"New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Retinoblastoma,0000018_1,CancerGov,https://www.cancer.gov/types/retinoblastoma/patient/retinoblastoma-treatment-pdq,C0035335,T191,Disorders "What is (are) Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer ?",0000019_1-1,information,"Key Points - Ovarian epithelial cancer, fallopian tube cancer, and primary peritoneal cancer are diseases in which malignant (cancer) cells form in the tissue covering the ovary or lining the fallopian tube or peritoneum. - Ovarian epithelial cancer, fallopian tube cancer, and primary peritoneal cancer form in the same type of tissue and are treated the same way. - Women who have a family history of ovarian cancer are at an increased risk of ovarian cancer. - Some ovarian, fallopian tube, and primary peritoneal cancers are caused by inherited gene mutations (changes). - Women with an increased risk of ovarian cancer may consider surgery to lessen the risk. - Signs and symptoms of ovarian, fallopian tube, or peritoneal cancer include pain or swelling in the abdomen. - Tests that examine the ovaries and pelvic area are used to detect (find) and diagnose ovarian, fallopian tube, and peritoneal cancer. - Certain factors affect treatment options and prognosis (chance of recovery). Ovarian epithelial cancer, fallopian tube cancer, and primary peritoneal cancer are diseases in which malignant (cancer) cells form in the tissue covering the ovary or lining the fallopian tube or peritoneum. The ovaries are a pair of organs in the female reproductive system. They are in the pelvis, one on each side of the uterus (the hollow, pear-shaped organ where a fetus grows). Each ovary is about the size and shape of an almond. The ovaries make eggs and female hormones (chemicals that control the way certain cells or organs work). The fallopian tubes are a pair of long, slender tubes, one on each side of the uterus. Eggs pass from the ovaries, through the fallopian tubes, to the uterus. Cancer sometimes begins at the end of the fallopian tube near the ovary and spreads to the ovary. The peritoneum is the tissue that lines the abdominal wall and covers organs in the abdomen. Primary peritoneal cancer is cancer that forms in the peritoneum and has not spread there from another part of the body. Cancer sometimes begins in the peritoneum and spreads to the ovary. Ovarian epithelial cancer is one type of cancer that affects the ovary. See the following PDQ treatment summaries for information about other types of ovarian tumors: - Ovarian Germ Cell Tumors - Ovarian Low Malignant Potential Tumors - Unusual Cancers of Childhood Treatment (ovarian cancer in children) Ovarian epithelial cancer, fallopian tube cancer, and primary peritoneal cancer form in the same type of tissue and are treated the same way.","Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer",0000019_1,CancerGov,https://www.cancer.gov/types/ovarian/patient/ovarian-epithelial-treatment-pdq,C1299307,T191,Disorders "Who is at risk for Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer? ?",0000019_1-2,susceptibility,"Women who have a family history of ovarian cancer are at an increased risk of ovarian cancer. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Women who have one first-degree relative (mother, daughter, or sister) with a history of ovarian cancer have an increased risk of ovarian cancer. This risk is higher in women who have one first-degree relative and one second-degree relative (grandmother or aunt) with a history of ovarian cancer. This risk is even higher in women who have two or more first-degree relatives with a history of ovarian cancer.","Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer",0000019_1,CancerGov,https://www.cancer.gov/types/ovarian/patient/ovarian-epithelial-treatment-pdq,C1299307,T191,Disorders "Is Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer inherited ?",0000019_1-3,inheritance,"Some ovarian, fallopian tube, and primary peritoneal cancers are caused by inherited gene mutations (changes). The genes in cells carry the hereditary information that is received from a persons parents. Hereditary ovarian cancer makes up about 20% of all cases of ovarian cancer. There are three hereditary patterns: ovarian cancer alone, ovarian and breast cancers, and ovarian and colon cancers. Fallopian tube cancer and peritoneal cancer may also be caused by certain inherited gene mutations. There are tests that can detect gene mutations. These genetic tests are sometimes done for members of families with a high risk of cancer. See the following PDQ summaries for more information: - Ovarian, Fallopian Tube, and Primary Peritoneal Cancer Prevention - Genetics of Breast and Gynecologic Cancers (for health professionals)","Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer",0000019_1,CancerGov,https://www.cancer.gov/types/ovarian/patient/ovarian-epithelial-treatment-pdq,C1299307,T191,Disorders "How to prevent Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer ?",0000019_1-4,prevention,"Women with an increased risk of ovarian cancer may consider surgery to lessen the risk. Some women who have an increased risk of ovarian cancer may choose to have a risk-reducing oophorectomy (the removal of healthy ovaries so that cancer cannot grow in them). In high-risk women, this procedure has been shown to greatly decrease the risk of ovarian cancer. (See the PDQ summary on Ovarian, Fallopian Tube, and Primary Peritoneal Cancer Prevention for more information.)","Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer",0000019_1,CancerGov,https://www.cancer.gov/types/ovarian/patient/ovarian-epithelial-treatment-pdq,C1299307,T191,Disorders "What are the symptoms of Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer ?",0000019_1-5,symptoms,"Signs and symptoms of ovarian, fallopian tube, or peritoneal cancer include pain or swelling in the abdomen. Ovarian, fallopian tube, or peritoneal cancer may not cause early signs or symptoms. When signs or symptoms do appear, the cancer is often advanced. Signs and symptoms may include the following: - Pain, swelling, or a feeling of pressure in the abdomen or pelvis. - Vaginal bleeding that is heavy or irregular, especially after menopause. - Vaginal discharge that is clear, white, or colored with blood. - A lump in the pelvic area. - Gastrointestinal problems, such as gas, bloating, or constipation. These signs and symptoms also may be caused by other conditions and not by ovarian, fallopian tube, or peritoneal cancer. If the signs or symptoms get worse or do not go away on their own, check with your doctor so that any problem can be diagnosed and treated as early as possible.","Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer",0000019_1,CancerGov,https://www.cancer.gov/types/ovarian/patient/ovarian-epithelial-treatment-pdq,C1299307,T191,Disorders "How to diagnose Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer ?",0000019_1-6,exams and tests,"Tests that examine the ovaries and pelvic area are used to detect (find) and diagnose ovarian, fallopian tube, and peritoneal cancer. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Pelvic exam : An exam of the vagina, cervix, uterus, fallopian tubes, ovaries, and rectum. A speculum is inserted into the vagina and the doctor or nurse looks at the vagina and cervix for signs of disease. A Pap test of the cervix is usually done. The doctor or nurse also inserts one or two lubricated, gloved fingers of one hand into the vagina and places the other hand over the lower abdomen to feel the size, shape, and position of the uterus and ovaries. The doctor or nurse also inserts a lubricated, gloved finger into the rectum to feel for lumps or abnormal areas. - CA 125 assay : A test that measures the level of CA 125 in the blood. CA 125 is a substance released by cells into the bloodstream. An increased CA 125 level can be a sign of cancer or another condition such as endometriosis. - Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs in the abdomen, and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later. Some patients may have a transvaginal ultrasound. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A very small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Chest x-ray : An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. - Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. The tissue is usually removed during surgery to remove the tumor.","Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer",0000019_1,CancerGov,https://www.cancer.gov/types/ovarian/patient/ovarian-epithelial-treatment-pdq,C1299307,T191,Disorders "What is the outlook for Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer ?",0000019_1-7,outlook,Certain factors affect treatment options and prognosis (chance of recovery). The prognosis (chance of recovery) and treatment options depend on the following: - The type of ovarian cancer and how much cancer there is. - The stage and grade of the cancer. - Whether the patient has extra fluid in the abdomen that causes swelling. - Whether all of the tumor can be removed by surgery. - Whether there are changes in the BRCA1 or BRCA2 genes. - The patients age and general health. - Whether the cancer has just been diagnosed or has recurred (come back).,"Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer",0000019_1,CancerGov,https://www.cancer.gov/types/ovarian/patient/ovarian-epithelial-treatment-pdq,C1299307,T191,Disorders "What are the stages of Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer ?",0000019_1-8,stages,"Key Points - After ovarian, fallopian tube, or peritoneal cancer has been diagnosed, tests are done to find out if cancer cells have spread within the ovaries or to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - The following stages are used for ovarian epithelial, fallopian tube, and primary peritoneal cancer: - Stage I - Stage II - Stage III - Stage IV - Ovarian epithelial, fallopian tube, and primary peritoneal cancers are grouped for treatment as early or advanced cancer. After ovarian, fallopian tube, or peritoneal cancer has been diagnosed, tests are done to find out if cancer cells have spread within the ovaries or to other parts of the body. The process used to find out whether cancer has spread within the organ or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The results of the tests used to diagnose cancer are often also used to stage the disease. (See the General Information section.) There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if ovarian epithelial cancer spreads to the lung, the cancer cells in the lung are actually ovarian epithelial cancer cells. The disease is metastatic ovarian epithelial cancer, not lung cancer. The following stages are used for ovarian epithelial, fallopian tube, and primary peritoneal cancer: Stage I In stage I, cancer is found in one or both ovaries or fallopian tubes. Stage I is divided into stage IA, stage IB, and stage IC. - Stage IA: Cancer is found inside a single ovary or fallopian tube. - Stage IB: Cancer is found inside both ovaries or fallopian tubes. - Stage IC: Cancer is found inside one or both ovaries or fallopian tubes and one of the following is true: - cancer is also found on the outside surface of one or both ovaries or fallopian tubes; or - the capsule (outer covering) of the ovary ruptured (broke open) before or during surgery; or - cancer cells are found in the fluid of the peritoneal cavity (the body cavity that contains most of the organs in the abdomen) or in washings of the peritoneum (tissue lining the peritoneal cavity). Stage II In stage II, cancer is found in one or both ovaries or fallopian tubes and has spread into other areas of the pelvis, or primary peritoneal cancer is found within the pelvis. Stage II ovarian epithelial and fallopian tube cancers are divided into stage IIA and stage IIB. - Stage IIA: Cancer has spread from where it first formed to the uterus and/or the fallopian tubes and/or the ovaries. - Stage IIB: Cancer has spread from the ovary or fallopian tube to organs in the peritoneal cavity (the space that contains the abdominal organs). Stage III In stage III, cancer is found in one or both ovaries or fallopian tubes, or is primary peritoneal cancer, and has spread outside the pelvis to other parts of the abdomen and/or to nearby lymph nodes. Stage III is divided into stage IIIA, stage IIIB, and stage IIIC. - In stage IIIA, one of the following is true: - Cancer has spread to lymph nodes in the area outside or behind the peritoneum only; or - Cancer cells that can be seen only with a microscope have spread to the surface of the peritoneum outside the pelvis. Cancer may have spread to nearby lymph nodes. - Stage IIIB: Cancer has spread to the peritoneum outside the pelvis and the cancer in the peritoneum is 2 centimeters or smaller. Cancer may have spread to lymph nodes behind the peritoneum. - Stage IIIC: Cancer has spread to the peritoneum outside the pelvis and the cancer in the peritoneum is larger than 2 centimeters. Cancer may have spread to lymph nodes behind the peritoneum or to the surface of the liver or spleen. Stage IV In stage IV, cancer has spread beyond the abdomen to other parts of the body. Stage IV is divided into stage IVA and stage IVB. - Stage IVA: Cancer cells are found in extra fluid that builds up around the lungs. - Stage IVB: Cancer has spread to organs and tissues outside the abdomen, including lymph nodes in the groin. Ovarian epithelial, fallopian tube, and primary peritoneal cancers are grouped for treatment as early or advanced cancer. Stage I ovarian epithelial and fallopian tube cancers are treated as early cancers. Stages II, III, and IV ovarian epithelial, fallopian tube, and primary peritoneal cancers are treated as advanced cancers.","Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer",0000019_1,CancerGov,https://www.cancer.gov/types/ovarian/patient/ovarian-epithelial-treatment-pdq,C1299307,T191,Disorders "What are the treatments for Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer ?",0000019_1-9,treatment,"Key Points - There are different types of treatment for patients with ovarian epithelial cancer. - Three kinds of standard treatment are used. - Surgery - Chemotherapy - Targeted therapy - New types of treatment are being tested in clinical trials. - Radiation therapy - Immunotherapy - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with ovarian epithelial cancer. Different types of treatment are available for patients with ovarian epithelial cancer. Some treatments are standard, and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the treatment currently used as standard treatment, the new treatment may become the standard treatment. Patients with any stage of ovarian cancer may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Three kinds of standard treatment are used. Surgery Most patients have surgery to remove as much of the tumor as possible. Different types of surgery may include: - Hysterectomy: Surgery to remove the uterus and, sometimes, the cervix. When only the uterus is removed, it is called a partial hysterectomy. When both the uterus and the cervix are removed, it is called a total hysterectomy. If the uterus and cervix are taken out through the vagina, the operation is called a vaginal hysterectomy. If the uterus and cervix are taken out through a large incision (cut) in the abdomen, the operation is called a total abdominal hysterectomy. If the uterus and cervix are taken out through a small incision (cut) in the abdomen using a laparoscope, the operation is called a total laparoscopic hysterectomy. - Unilateral salpingo-oophorectomy: A surgical procedure to remove one ovary and one fallopian tube. - Bilateral salpingo-oophorectomy: A surgical procedure to remove both ovaries and both fallopian tubes. - Omentectomy: A surgical procedure to remove the omentum (tissue in the peritoneum that contains blood vessels, nerves, lymph vessels, and lymph nodes). - Lymph node biopsy: The removal of all or part of a lymph node. A pathologist views the tissue under a microscope to look for cancer cells. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). A type of regional chemotherapy used to treat ovarian cancer is intraperitoneal (IP) chemotherapy. In IP chemotherapy, the anticancer drugs are carried directly into the peritoneal cavity (the space that contains the abdominal organs) through a thin tube. Treatment with more than one anticancer drug is called combination chemotherapy. The way the chemotherapy is given depends on the type and stage of the cancer being treated. See Drugs Approved for Ovarian, Fallopian Tube, or Primary Peritoneal Cancer for more information. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Monoclonal antibody therapy is a type of targeted therapy that uses antibodies made in the laboratory, from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Bevacizumab is a monoclonal antibody that may be used with chemotherapy to treat ovarian epithelial cancer, fallopian tube cancer, or primary peritoneal cancer that has recurred (come back). Poly (ADP-ribose) polymerase inhibitors (PARP inhibitors) are targeted therapy drugs that block DNA repair and may cause cancer cells to die. Olaparib and niraparib are PARP inhibitors that may be used to treat advanced ovarian cancer. See Drugs Approved for Ovarian, Fallopian Tube, or Primary Peritoneal Cancer for more information. New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. Some women receive a treatment called intraperitoneal radiation therapy, in which radioactive liquid is put directly in the abdomen through a catheter. Intraperitoneal radiation therapy is being studied to treat advanced ovarian cancer. Immunotherapy Immunotherapy is a treatment that uses the patients immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the bodys natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy. Vaccine therapy uses a substance to stimulate the immune system to destroy a tumor. Vaccine therapy is being studied to treat advanced ovarian cancer. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options by Stage Early Ovarian Epithelial and Fallopian Tube Cancer Treatment of early ovarian epithelial cancer or fallopian tube cancer may include the following: - Hysterectomy, bilateral salpingo-oophorectomy, and omentectomy. Lymph nodes and other tissues in the pelvis and abdomen are removed and checked under a microscope for cancer cells. Chemotherapy may be given after surgery. - Unilateral salpingo-oophorectomy may be done in certain women who wish to have children. Chemotherapy may be given after surgery. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I ovarian epithelial cancer and fallopian tube cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Advanced Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer Treatment of advanced ovarian epithelial cancer, fallopian tube cancer, or primary peritoneal cancer may include the following: - Hysterectomy, bilateral salpingo-oophorectomy, and omentectomy. Lymph nodes and other tissues in the pelvis and abdomen are removed and checked under a microscope to look for cancer cells. Surgery is followed by one of the following: - Intravenous chemotherapy. - Intraperitoneal chemotherapy. - Chemotherapy and targeted therapy (bevacizumab, olaparib, or niraparib). - Chemotherapy followed by hysterectomy, bilateral salpingo-oophorectomy, and omentectomy. - Chemotherapy alone for patients who cannot have surgery. - A clinical trial of intraperitoneal radiation therapy, immunotherapy (vaccine therapy), or targeted therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage II ovarian epithelial cancer, stage III ovarian epithelial cancer, stage IV ovarian epithelial cancer, fallopian tube cancer and primary peritoneal cavity cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.","Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer",0000019_1,CancerGov,https://www.cancer.gov/types/ovarian/patient/ovarian-epithelial-treatment-pdq,C1299307,T191,Disorders "what research (or clinical trials) is being done for Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer ?",0000019_1-10,research,"New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. Some women receive a treatment called intraperitoneal radiation therapy, in which radioactive liquid is put directly in the abdomen through a catheter. Intraperitoneal radiation therapy is being studied to treat advanced ovarian cancer. Immunotherapy Immunotherapy is a treatment that uses the patients immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the bodys natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy. Vaccine therapy uses a substance to stimulate the immune system to destroy a tumor. Vaccine therapy is being studied to treat advanced ovarian cancer. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.","Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer",0000019_1,CancerGov,https://www.cancer.gov/types/ovarian/patient/ovarian-epithelial-treatment-pdq,C1299307,T191,Disorders What is (are) Ovarian Germ Cell Tumors ?,0000019_2-1,information,"Key Points - Ovarian germ cell tumor is a disease in which malignant (cancer) cells form in the germ (egg) cells of the ovary. - Signs of ovarian germ cell tumor are swelling of the abdomen or vaginal bleeding after menopause. - Tests that examine the ovaries, pelvic area, blood, and ovarian tissue are used to detect (find) and diagnose ovarian germ cell tumor. - Certain factors affect prognosis (chance of recovery and treatment options). Ovarian germ cell tumor is a disease in which malignant (cancer) cells form in the germ (egg) cells of the ovary. Germ cell tumors begin in the reproductive cells (egg or sperm) of the body. Ovarian germ cell tumors usually occur in teenage girls or young women and most often affect just one ovary. The ovaries are a pair of organs in the female reproductive system. They are in the pelvis, one on each side of the uterus (the hollow, pear-shaped organ where a fetus grows). Each ovary is about the size and shape of an almond. The ovaries make eggs and female hormones. Ovarian germ cell tumor is a general name that is used to describe several different types of cancer. The most common ovarian germ cell tumor is called dysgerminoma. See the following PDQ summaries for information about other types of ovarian tumors: - Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer Treatment - Ovarian Low Malignant Potential Tumors Treatment",Ovarian Germ Cell Tumors,0000019_2,CancerGov,https://www.cancer.gov/types/ovarian/patient/ovarian-germ-cell-treatment-pdq,C0238324,T191,Disorders What are the symptoms of Ovarian Germ Cell Tumors ?,0000019_2-2,symptoms,"Signs of ovarian germ cell tumor are swelling of the abdomen or vaginal bleeding after menopause. Ovarian germ cell tumors can be hard to diagnose (find) early. Often there are no symptoms in the early stages, but tumors may be found during regular gynecologic exams (checkups). Check with your doctor if you have either of the following: - Swollen abdomen without weight gain in other parts of the body. - Bleeding from the vagina after menopause (when you are no longer having menstrual periods).",Ovarian Germ Cell Tumors,0000019_2,CancerGov,https://www.cancer.gov/types/ovarian/patient/ovarian-germ-cell-treatment-pdq,C0238324,T191,Disorders How to diagnose Ovarian Germ Cell Tumors ?,0000019_2-3,exams and tests,"Tests that examine the ovaries, pelvic area, blood, and ovarian tissue are used to detect (find) and diagnose ovarian germ cell tumor. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Pelvic exam : An exam of the vagina, cervix, uterus, fallopian tubes, ovaries, and rectum. A speculum is inserted into the vagina and the doctor or nurse looks at the vagina and cervix for signs of disease. A Pap test of the cervix is usually done. The doctor or nurse also inserts one or two lubricated, gloved fingers of one hand into the vagina and places the other hand over the lower abdomen to feel the size, shape, and position of the uterus and ovaries. The doctor or nurse also inserts a lubricated, gloved finger into the rectum to feel for lumps or abnormal areas. - Laparotomy : A surgical procedure in which an incision (cut) is made in the wall of the abdomen to check the inside of the abdomen for signs of disease. The size of the incision depends on the reason the laparotomy is being done. Sometimes organs are removed or tissue samples are taken and checked under a microscope for signs of disease. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - Serum tumor marker test : A procedure in which a sample of blood is checked to measure the amounts of certain substances released into the blood by organs, tissues, or tumor cells in the body. Certain substances are linked to specific types of cancer when found in increased levels in the blood. These are called tumor markers. An increased level of alpha fetoprotein (AFP) or human chorionic gonadotropin (HCG) in the blood may be a sign of ovarian germ cell tumor.",Ovarian Germ Cell Tumors,0000019_2,CancerGov,https://www.cancer.gov/types/ovarian/patient/ovarian-germ-cell-treatment-pdq,C0238324,T191,Disorders What is the outlook for Ovarian Germ Cell Tumors ?,0000019_2-4,outlook,"Certain factors affect prognosis (chance of recovery and treatment options). The prognosis (chance of recovery) and treatment options depend on the following: - The type of cancer. - The size of the tumor. - The stage of cancer (whether it affects part of the ovary, involves the whole ovary, or has spread to other places in the body). - The way the cancer cells look under a microscope. - The patients general health. Ovarian germ cell tumors are usually cured if found and treated early.",Ovarian Germ Cell Tumors,0000019_2,CancerGov,https://www.cancer.gov/types/ovarian/patient/ovarian-germ-cell-treatment-pdq,C0238324,T191,Disorders What are the stages of Ovarian Germ Cell Tumors ?,0000019_2-5,stages,"Key Points - After ovarian germ cell tumor has been diagnosed, tests are done to find out if cancer cells have spread within the ovary or to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - The following stages are used for ovarian germ cell tumors: - Stage I - Stage II - Stage III - Stage IV After ovarian germ cell tumor has been diagnosed, tests are done to find out if cancer cells have spread within the ovary or to other parts of the body. The process used to find out whether cancer has spread within the ovary or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. Unless a doctor is sure the cancer has spread from the ovaries to other parts of the body, an operation called a laparotomy is done to see if the cancer has spread. The doctor must cut into the abdomen and carefully look at all the organs to see if they have cancer in them. The doctor will cut out small pieces of tissue so they can be checked under a microscope for signs of cancer. The doctor may also wash the abdominal cavity with fluid, which is also checked under a microscope to see if it has cancer cells in it. Usually the doctor will remove the cancer and other organs that have cancer in them during the laparotomy. It is important to know the stage in order to plan treatment. Many of the tests used to diagnose ovarian germ cell tumor are also used for staging. The following tests and procedures may also be used for staging: - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Transvaginal ultrasound exam: A procedure used to examine the vagina, uterus, fallopian tubes, and bladder. An ultrasound transducer (probe) is inserted into the vagina and used to bounce high-energy sound waves (ultrasound) off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The doctor can identify tumors by looking at the sonogram. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of tumor as the primary tumor. For example, if an ovarian germ cell tumor spreads to the liver, the tumor cells in the liver are actually cancerous ovarian germ cells. The disease is metastatic ovarian germ cell tumor, not liver cancer. The following stages are used for ovarian germ cell tumors: Stage I In stage I, cancer is found in one or both ovaries. Stage I is divided into stage IA, stage IB, and stage IC. - Stage IA: Cancer is found inside a single ovary. - Stage IB: Cancer is found inside both ovaries. - Stage IC: Cancer is found inside one or both ovaries and one of the following is true: - cancer is also found on the outside surface of one or both ovaries; or - the capsule (outer covering) of the ovary has ruptured (broken open); or - cancer cells are found in the fluid of the peritoneal cavity (the body cavity that contains most of the organs in the abdomen) or in washings of the peritoneum (tissue lining the peritoneal cavity). Stage II In stage II, cancer is found in one or both ovaries and has spread into other areas of the pelvis. Stage II is divided into stage IIA, stage IIB, and stage IIC. - Stage IIA: Cancer has spread to the uterus and/or fallopian tubes (the long slender tubes through which eggs pass from the ovaries to the uterus). - Stage IIB: Cancer has spread to other tissue within the pelvis. - Stage IIC: Cancer is found inside one or both ovaries and has spread to the uterus and/or fallopian tubes, or to other tissue within the pelvis. Also, one of the following is true: - cancer is found on the outside surface of one or both ovaries; or - the capsule (outer covering) of the ovary has ruptured (broken open); or - cancer cells are found in the fluid of the peritoneal cavity (the body cavity that contains most of the organs in the abdomen) or in washings of the peritoneum (tissue lining the peritoneal cavity). Stage III In stage III, cancer is found in one or both ovaries and has spread outside the pelvis to other parts of the abdomen and/or nearby lymph nodes. Stage III is divided into stage IIIA, stage IIIB, and stage IIIC. - Stage IIIA: The tumor is found in the pelvis only, but cancer cells that can be seen only with a microscope have spread to the surface of the peritoneum (tissue that lines the abdominal wall and covers most of the organs in the abdomen), the small intestines, or the tissue that connects the small intestines to the wall of the abdomen. - Stage IIIB: Cancer has spread to the peritoneum and the cancer in the peritoneum is 2 centimeters or smaller. - Stage IIIC: Cancer has spread to the peritoneum and the cancer in the peritoneum is larger than 2 centimeters and/or cancer has spread to lymph nodes in the abdomen. Cancer that has spread to the surface of the liver is also considered stage III ovarian cancer. Stage IV In stage IV, cancer has spread beyond the abdomen to other parts of the body, such as the lungs or tissue inside the liver. Cancer cells in the fluid around the lungs is also considered stage IV ovarian cancer.",Ovarian Germ Cell Tumors,0000019_2,CancerGov,https://www.cancer.gov/types/ovarian/patient/ovarian-germ-cell-treatment-pdq,C0238324,T191,Disorders What are the treatments for Ovarian Germ Cell Tumors ?,0000019_2-6,treatment,"Key Points - There are different types of treatment for patients with ovarian germ cell tumors. - Four types of standard treatment are used: - Surgery - Observation - Chemotherapy - Radiation therapy - New types of treatment are being tested in clinical trials. - High-dose chemotherapy with bone marrow transplant - New treatment options - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with ovarian germ cell tumors. Different types of treatment are available for patients with ovarian germ cell tumor. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Four types of standard treatment are used: Surgery Surgery is the most common treatment of ovarian germ cell tumor. A doctor may take out the cancer using one of the following types of surgery. - Unilateral salpingo-oophorectomy: A surgical procedure to remove one ovary and one fallopian tube. - Total hysterectomy: A surgical procedure to remove the uterus, including the cervix. If the uterus and cervix are taken out through the vagina, the operation is called a vaginal hysterectomy. If the uterus and cervix are taken out through a large incision (cut) in the abdomen, the operation is called a total abdominal hysterectomy. If the uterus and cervix are taken out through a small incision (cut) in the abdomen using a laparoscope, the operation is called a total laparoscopic hysterectomy. - Bilateral salpingo-oophorectomy: A surgical procedure to remove both ovaries and both fallopian tubes. - Tumor debulking: A surgical procedure in which as much of the tumor as possible is removed. Some tumors cannot be completely removed. Even if the doctor removes all the cancer that can be seen at the time of the operation, some patients may be offered chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. After chemotherapy for an ovarian germ cell tumor, a second-look laparotomy may be done. This is similar to the laparotomy that is done to find out the stage of the cancer. Second-look laparotomy is a surgical procedure to find out if tumor cells are left after primary treatment. During this procedure, the doctor will take samples of lymph nodes and other tissues in the abdomen to see if any cancer is left. This procedure is not done for dysgerminomas. Observation Observation is closely watching a patients condition without giving any treatment unless signs or symptoms appear or change. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is treatment using more than one anticancer drug. The way the chemotherapy is given depends on the type and stage of the cancer being treated. See Drugs Approved for Ovarian, Fallopian Tube, or Primary Peritoneal Cancer for more information. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External radiation therapy is used to treat ovarian germ cell tumors. New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. High-dose chemotherapy with bone marrow transplant High-dose chemotherapy with bone marrow transplant is a method of giving very high doses of chemotherapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the bodys blood cells. New treatment options Combination chemotherapy (the use of more than one anticancer drug) is being tested in clinical trials. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options By Stage Stage I Ovarian Germ Cell Tumors Treatment depends on whether the tumor is a dysgerminoma or another type of ovarian germ cell tumor. Treatment of dysgerminoma may include the following: - Unilateral salpingo-oophorectomy with or without lymphangiography or CT scan. - Unilateral salpingo-oophorectomy followed by observation. - Unilateral salpingo-oophorectomy followed by radiation therapy. - Unilateral salpingo-oophorectomy followed by chemotherapy. Treatment of other ovarian germ cell tumors may be either: - unilateral salpingo-oophorectomy followed by careful observation; or - unilateral salpingo-oophorectomy, sometimes followed by combination chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I ovarian germ cell tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage II Ovarian Germ Cell Tumors Treatment depends on whether the tumor is a dysgerminoma or another type of ovarian germ cell tumor. Treatment of dysgerminoma may be either: - total abdominal hysterectomy and bilateral salpingo-oophorectomy followed by radiation therapy or combination chemotherapy; or - unilateral salpingo-oophorectomy followed by chemotherapy. Treatment of other ovarian germ cell tumors may include the following: - Unilateral salpingo-oophorectomy followed by combination chemotherapy. - Second-look laparotomy (surgery done after primary treatment to see if tumor cells remain). - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage II ovarian germ cell tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage III Ovarian Germ Cell Tumors Treatment depends on whether the tumor is a dysgerminoma or another type of ovarian germ cell tumor. Treatment of dysgerminoma may include the following: - Total abdominal hysterectomy and bilateral salpingo-oophorectomy, with removal of as much of the cancer in the pelvis and abdomen as possible. - Unilateral salpingo-oophorectomy followed by chemotherapy. Treatment of other ovarian germ cell tumors may include the following: - Total abdominal hysterectomy and bilateral salpingo-oophorectomy, with removal of as much of the cancer in the pelvis and abdomen as possible. Chemotherapy will be given before and/or after surgery. - Unilateral salpingo-oophorectomy followed by chemotherapy. - Second-look laparotomy (surgery done after primary treatment to see if tumor cells remain). - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III ovarian germ cell tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage IV Ovarian Germ Cell Tumors Treatment depends on whether the tumor is a dysgerminoma or another type of ovarian germ cell tumor. Treatment of dysgerminoma may include the following: - Total abdominal hysterectomy and bilateral salpingo-oophorectomy followed by chemotherapy, with removal of as much of the cancer in the pelvis and abdomen as possible. - Unilateral salpingo-oophorectomy followed by chemotherapy. Treatment of other ovarian germ cell tumors may include the following: - Total abdominal hysterectomy and bilateral salpingo-oophorectomy, with removal of as much of the cancer in the pelvis and abdomen as possible. Chemotherapy will be given before and/or after surgery. - Unilateral salpingo-oophorectomy followed by chemotherapy. - Second-look laparotomy (surgery done after primary treatment to see if tumor cells remain). - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage IV ovarian germ cell tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Ovarian Germ Cell Tumors,0000019_2,CancerGov,https://www.cancer.gov/types/ovarian/patient/ovarian-germ-cell-treatment-pdq,C0238324,T191,Disorders what research (or clinical trials) is being done for Ovarian Germ Cell Tumors ?,0000019_2-7,research,"New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. High-dose chemotherapy with bone marrow transplant High-dose chemotherapy with bone marrow transplant is a method of giving very high doses of chemotherapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the bodys blood cells. New treatment options Combination chemotherapy (the use of more than one anticancer drug) is being tested in clinical trials. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Ovarian Germ Cell Tumors,0000019_2,CancerGov,https://www.cancer.gov/types/ovarian/patient/ovarian-germ-cell-treatment-pdq,C0238324,T191,Disorders What is (are) Ovarian Low Malignant Potential Tumors ?,0000019_3-1,information,"Key Points - Ovarian low malignant potential tumor is a disease in which abnormal cells form in the tissue covering the ovary. - Signs and symptoms of ovarian low malignant potential tumor include pain or swelling in the abdomen. - Tests that examine the ovaries are used to detect (find), diagnose, and stage ovarian low malignant potential tumor. - Certain factors affect prognosis (chance of recovery) and treatment options. Ovarian low malignant potential tumor is a disease in which abnormal cells form in the tissue covering the ovary. Ovarian low malignant potential tumors have abnormal cells that may become cancer, but usually do not. This disease usually remains in the ovary. When disease is found in one ovary, the other ovary should also be checked carefully for signs of disease. The ovaries are a pair of organs in the female reproductive system. They are in the pelvis, one on each side of the uterus (the hollow, pear-shaped organ where a fetus grows). Each ovary is about the size and shape of an almond. The ovaries make eggs and female hormones.",Ovarian Low Malignant Potential Tumors,0000019_3,CancerGov,https://www.cancer.gov/types/ovarian/patient/ovarian-low-malignant-treatment-pdq,C1533592,T191,Disorders What are the symptoms of Ovarian Low Malignant Potential Tumors ?,0000019_3-2,symptoms,"Signs and symptoms of ovarian low malignant potential tumor include pain or swelling in the abdomen.Ovarian low malignant potential tumor may not cause early signs or symptoms. If you do have signs or symptoms, they may include the following: - Pain or swelling in the abdomen. - Pain in the pelvis. - Gastrointestinal problems, such as gas, bloating, or constipation. These signs and symptoms may be caused by other conditions. If they get worse or do not go away on their own, check with your doctor.",Ovarian Low Malignant Potential Tumors,0000019_3,CancerGov,https://www.cancer.gov/types/ovarian/patient/ovarian-low-malignant-treatment-pdq,C1533592,T191,Disorders How to diagnose Ovarian Low Malignant Potential Tumors ?,0000019_3-3,exams and tests,"Tests that examine the ovaries are used to detect (find), diagnose, and stage ovarian low malignant potential tumor. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Pelvic exam : An exam of the vagina, cervix, uterus, fallopian tubes, ovaries, and rectum. A speculum is inserted into the vagina and the doctor or nurse looks at the vagina and cervix for signs of disease. A Pap test of the cervix is usually done. The doctor or nurse also inserts one or two lubricated, gloved fingers of one hand into the vagina and places the other hand over the lower abdomen to feel the size, shape, and position of the uterus and ovaries. The doctor or nurse also inserts a lubricated, gloved finger into the rectum to feel for lumps or abnormal areas. - Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later.Other patients may have a transvaginal ultrasound. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - CA 125 assay : A test that measures the level of CA 125 in the blood. CA 125 is a substance released by cells into the bloodstream. An increased CA 125 level is sometimes a sign of cancer or other condition. - Chest x-ray : An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. - Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. The tissue is usually removed during surgery to remove the tumor.",Ovarian Low Malignant Potential Tumors,0000019_3,CancerGov,https://www.cancer.gov/types/ovarian/patient/ovarian-low-malignant-treatment-pdq,C1533592,T191,Disorders What is the outlook for Ovarian Low Malignant Potential Tumors ?,0000019_3-4,outlook,"Certain factors affect prognosis (chance of recovery) and treatment options.The prognosis (chance of recovery) and treatment options depend on the following: - The stage of the disease (whether it affects part of the ovary, involves the whole ovary, or has spread to other places in the body). - What type of cells make up the tumor. - The size of the tumor. - The patients general health. Patients with ovarian low malignant potential tumors have a good prognosis, especially when the tumor is found early.",Ovarian Low Malignant Potential Tumors,0000019_3,CancerGov,https://www.cancer.gov/types/ovarian/patient/ovarian-low-malignant-treatment-pdq,C1533592,T191,Disorders What are the stages of Ovarian Low Malignant Potential Tumors ?,0000019_3-5,stages,"Key Points - After ovarian low malignant potential tumor has been diagnosed, tests are done to find out if abnormal cells have spread within the ovary or to other parts of the body. - The following stages are used for ovarian low malignant potential tumor: - Stage I - Stage II - Stage III - Stage IV After ovarian low malignant potential tumor has been diagnosed, tests are done to find out if abnormal cells have spread within the ovary or to other parts of the body. The process used to find out whether abnormal cells have spread within the ovary or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. Certain tests or procedures are used for staging. Staging laparotomy (a surgical incision made in the wall of the abdomen to remove ovarian tissue) may be used. Most patients are diagnosed with stage I disease. The following stages are used for ovarian low malignant potential tumor: Stage I In stage I, the tumor is found in one or both ovaries. Stage I is divided into stage IA, stage IB, and stage IC. - Stage IA: The tumor is found inside a single ovary. - Stage IB: The tumor is found inside both ovaries. - Stage IC: The tumor is found inside one or both ovaries and one of the following is true: - tumor cells are found on the outside surface of one or both ovaries; or - the capsule (outer covering) of the ovary has ruptured (broken open); or - tumor cells are found in the fluid of the peritoneal cavity (the body cavity that contains most of the organs in the abdomen) or in washings of the peritoneum (tissue lining the peritoneal cavity). Stage II In stage II, the tumor is found in one or both ovaries and has spread into other areas of the pelvis. Stage II is divided into stage IIA, stage IIB, and stage IIC. - Stage IIA: The tumor has spread to the uterus and/or fallopian tubes (the long slender tubes through which eggs pass from the ovaries to the uterus). - Stage IIB: The tumor has spread to other tissue within the pelvis. - Stage IIC: The tumor is found inside one or both ovaries and has spread to the uterus and/or fallopian tubes, or to other tissue within the pelvis. Also, one of the following is true: - tumor cells are found on the outside surface of one or both ovaries; or - the capsule (outer covering) of the ovary has ruptured (broken open); or - tumor cells are found in the fluid of the peritoneal cavity (the body cavity that contains most of the organs in the abdomen) or in washings of the peritoneum (tissue lining the peritoneal cavity). Stage III In stage III, the tumor is found in one or both ovaries and has spread outside the pelvis to other parts of the abdomen and/or nearby lymph nodes. Stage III is divided into stage IIIA, stage IIIB, and stage IIIC. - Stage IIIA: The tumor is found in the pelvis only, but tumor cells that can be seen only with a microscope have spread to the surface of the peritoneum (tissue that lines the abdominal wall and covers most of the organs in the abdomen), the small intestines, or the tissue that connects the small intestines to the wall of the abdomen. - Stage IIIB: The tumor has spread to the peritoneum and the tumor in the peritoneum is 2 centimeters or smaller. - Stage IIIC: The tumor has spread to the peritoneum and the tumor in the peritoneum is larger than 2 centimeters and/or has spread to lymph nodes in the abdomen. The spread of tumor cells to the surface of the liver is also considered stage III disease. Stage IV In stage IV, tumor cells have spread beyond the abdomen to other parts of the body, such as the lungs or tissue inside the liver. Tumor cells in the fluid around the lungs is also considered stage IV disease. Ovarian low malignant potential tumors almost never reach stage IV.",Ovarian Low Malignant Potential Tumors,0000019_3,CancerGov,https://www.cancer.gov/types/ovarian/patient/ovarian-low-malignant-treatment-pdq,C1533592,T191,Disorders What are the treatments for Ovarian Low Malignant Potential Tumors ?,0000019_3-6,treatment,"Key Points - There are different types of treatment for patients with ovarian low malignant potential tumor. - Two types of standard treatment are used: - Surgery - Chemotherapy - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their treatment. - Follow-up tests may be needed. There are different types of treatment for patients with ovarian low malignant potential tumor. Different types of treatment are available for patients with ovarian low malignant potential tumor. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer, tumors, and related conditions. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Two types of standard treatment are used: Surgery The type of surgery (removing the tumor in an operation) depends on the size and spread of the tumor and the womans plans for having children. Surgery may include the following: - Unilateral salpingo-oophorectomy: Surgery to remove one ovary and one fallopian tube. - Bilateral salpingo-oophorectomy: Surgery to remove both ovaries and both fallopian tubes. - Total hysterectomy and bilateral salpingo-oophorectomy: Surgery to remove the uterus, cervix, and both ovaries and fallopian tubes. If the uterus and cervix are taken out through the vagina, the operation is called a vaginal hysterectomy. If the uterus and cervix are taken out through a large incision (cut) in the abdomen, the operation is called a total abdominal hysterectomy. If the uterus and cervix are taken out through a small incision (cut) in the abdomen using a laparoscope, the operation is called a total laparoscopic hysterectomy. - Partial oophorectomy: Surgery to remove part of one ovary or part of both ovaries. - Omentectomy: Surgery to remove the omentum (a piece of the tissue lining the abdominal wall). Even if the doctor removes all disease that can be seen at the time of the operation, the patient may be given chemotherapy after surgery to kill any tumor cells that are left. Treatment given after the surgery, to lower the risk that the tumor will come back, is called adjuvant therapy. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI Web site. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the medical research process. Clinical trials are done to find out if new treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for disease are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way diseases will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose disease has not gotten better. There are also clinical trials that test new ways to stop a disease from recurring (coming back) or reduce the side effects of treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's clinical trials database. Follow-up tests may be needed. Some of the tests that were done to diagnose the disease may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. This is sometimes called re-staging. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the disease has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Ovarian Low Malignant Potential Tumors Early Stage Ovarian Low Malignant Potential Tumors (Stage I and II) Surgery is the standard treatment for early stage ovarian low malignant potential tumor. The type of surgery usually depends on whether a woman plans to have children. For women who plan to have children, surgery is either: - unilateral salpingo-oophorectomy; or - partial oophorectomy. To prevent recurrence of disease, most doctors recommend surgery to remove the remaining ovarian tissue when a woman no longer plans to have children. For women who do not plan to have children, treatment may be hysterectomy and bilateral salpingo-oophorectomy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I borderline ovarian surface epithelial-stromal tumor and stage II borderline ovarian surface epithelial-stromal tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Late Stage Ovarian Low Malignant Potential Tumors (Stage III and IV) Treatment for late stage ovarian low malignant potential tumor may be hysterectomy, bilateral salpingo-oophorectomy, and omentectomy. A lymph node dissection may also be done. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III borderline ovarian surface epithelial-stromal tumor and stage IV borderline ovarian surface epithelial-stromal tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Recurrent Ovarian Low Malignant Potential Tumors Treatment for recurrent ovarian low malignant potential tumor may include the following: - Surgery. - Surgery followed by chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent borderline ovarian surface epithelial-stromal tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Ovarian Low Malignant Potential Tumors,0000019_3,CancerGov,https://www.cancer.gov/types/ovarian/patient/ovarian-low-malignant-treatment-pdq,C1533592,T191,Disorders what research (or clinical trials) is being done for Ovarian Low Malignant Potential Tumors ?,0000019_3-7,research,"New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI Web site. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the medical research process. Clinical trials are done to find out if new treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for disease are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way diseases will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose disease has not gotten better. There are also clinical trials that test new ways to stop a disease from recurring (coming back) or reduce the side effects of treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's clinical trials database.",Ovarian Low Malignant Potential Tumors,0000019_3,CancerGov,https://www.cancer.gov/types/ovarian/patient/ovarian-low-malignant-treatment-pdq,C1533592,T191,Disorders "What is (are) Ovarian, Fallopian Tube, and Primary Peritoneal Cancer ?",0000019_4-1,information,"Key Points - Ovarian, fallopian tube, and primary peritoneal cancers are diseases in which malignant (cancer) cells form in the ovaries, fallopian tubes, or peritoneum. - Ovarian cancer is the leading cause of death from cancer of the female reproductive system. Ovarian, fallopian tube, and primary peritoneal cancers are diseases in which malignant (cancer) cells form in the ovaries, fallopian tubes, or peritoneum. The ovaries are a pair of organs in the female reproductive system. They are in the pelvis, one on each side of the uterus (the hollow, pear-shaped organ where a fetus grows). Each ovary is about the size and shape of an almond. The ovaries make eggs and female hormones (chemicals that control the way certain cells or organs work in the body). The fallopian tubes are a pair of long, slender tubes, one on each side of the uterus. Eggs pass from the ovaries, through the fallopian tubes, to the uterus. Cancer sometimes begins at the end of the fallopian tube near the ovary and spreads to the ovary. The peritoneum is the tissue that lines the abdominal wall and covers organs in the abdomen. Primary peritoneal cancer is cancer that forms in the peritoneum and has not spread there from another part of the body. Cancer sometimes begins in the peritoneum and spreads to the ovary. Ovarian cancer is the leading cause of death from cancer of the female reproductive system. In recent years, there has been a small decrease in the number of new cases of ovarian cancer and the number of deaths from ovarian cancer. New cases of ovarian cancer and deaths from ovarian cancer are higher among white women than black women, but have decreased in both groups. Women who have a family history of ovarian cancer and/or certain inherited gene changes, such as BRCA1 or BRCA2 gene changes, have a higher risk than women who do not have a family history or who have not inherited these gene changes. For women with inherited risk, genetic counseling and genetic testing can be used to find out more about how likely they are to develop ovarian cancer. It is hard to find ovarian cancer early. Early ovarian cancer may not cause any symptoms. When symptoms do appear, ovarian cancer is often advanced. See the following PDQ summaries for more information about ovarian, fallopian tube, and primary peritoneal cancers: - Genetics of Breast and Gynecologic Cancers (written for health professionals) - Ovarian, Fallopian Tube, and Primary Peritoneal Cancer Screening - Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer Treatment","Ovarian, Fallopian Tube, and Primary Peritoneal Cancer",0000019_4,CancerGov,https://www.cancer.gov/types/ovarian/patient/ovarian-prevention-pdq,C1299307,T191,Disorders "How to prevent Ovarian, Fallopian Tube, and Primary Peritoneal Cancer ?",0000019_4-2,prevention,"Key Points - Avoiding risk factors and increasing protective factors may help prevent cancer. - The following are risk factors for ovarian, fallopian tube, and primary peritoneal cancer: - Family history of ovarian, fallopian tube, and primary peritoneal cancer - Inherited risk - Hormone replacement therapy - Weight and height - The following are protective factors for ovarian, fallopian tube, and primary peritoneal cancer: - Oral contraceptives - Tubal ligation - Breastfeeding - Risk-reducing salpingo-oophorectomy - It is not clear whether the following affect the risk of ovarian, fallopian tube, and primary peritoneal cancer: - Diet - Alcohol - Aspirin and non-steroidal anti-inflammatory drugs - Smoking - Talc - Infertility treatment - Cancer prevention clinical trials are used to study ways to prevent cancer. - New ways to prevent ovarian, fallopian tube, and primary peritoneal cancer are being studied in clinical trials. Avoiding risk factors and increasing protective factors may help prevent cancer. Avoiding cancer risk factors may help prevent certain cancers. Risk factors include smoking, being overweight, and not getting enough exercise. Increasing protective factors such as quitting smoking and exercising may also help prevent some cancers. Talk to your doctor or other health care professional about how you might lower your risk of cancer. The following are risk factors for ovarian, fallopian tube, and primary peritoneal cancer: Family history of ovarian, fallopian tube, and primary peritoneal cancer A woman whose mother or sister had ovarian cancer has an increased risk of ovarian cancer. A woman with two or more relatives with ovarian cancer also has an increased risk of ovarian cancer. Inherited risk The risk of ovarian cancer is increased in women who have inherited certain changes in the BRCA1, BRCA2, or other genes. The risk of ovarian cancer is also increased in women who have certain inherited syndromes that include: - Familial site-specific ovarian cancer syndrome. - Familial breast/ovarian cancer syndrome. - Hereditary nonpolyposis colorectal cancer (HNPCC; Lynch syndrome). Hormone replacement therapy The use of estrogen -only hormone replacement therapy (HRT) after menopause is linked to a slightly increased risk of ovarian cancer in women who are taking HRT or have taken HRT within the past 3 years. The risk of ovarian cancer increases the longer a woman uses estrogen-only HRT. When hormone therapy is stopped, the risk of ovarian cancer decreases over time. It is not clear whether there is an increased risk of ovarian cancer with the use of HRT that has both estrogen and progestin. Weight and height Being overweight or obese during the teenage years is linked to an increased risk of ovarian cancer. Being obese is linked to an increased risk of death from ovarian cancer. Being tall (5'8"" or taller) may also be linked to a slight increase in the risk of ovarian cancer. The following are protective factors for ovarian, fallopian tube, and primary peritoneal cancer: Oral contraceptives Taking oral contraceptives (the pill) lowers the risk of ovarian cancer. The longer oral contraceptives are used, the lower the risk may be. The decrease in risk may last up to 30 years after a woman has stopped taking oral contraceptives. Taking oral contraceptives increases the risk of blood clots. This risk is higher in women who also smoke. Tubal ligation The risk of ovarian cancer is decreased in women who have a tubal ligation (surgery to close both fallopian tubes). Breastfeeding Breastfeeding is linked to a decreased risk of ovarian cancer. The longer a woman breastfeeds, the lower her risk of ovarian cancer. Risk-reducing salpingo-oophorectomy Some women who have a high risk of ovarian cancer may choose to have a risk-reducing salpingo-oophorectomy (surgery to remove the fallopian tubes and ovaries when there are no signs of cancer). This includes women who have inherited certain changes in the BRCA1 and BRCA2 genes or have an inherited syndrome. (See the Risk-reducing salpingo-oophorectomy section in the PDQ health professional summary on Genetics of Breast and Gynecologic Cancers for more information.) It is very important to have a cancer risk assessment and counseling before making this decision. These and other factors may be discussed: - Infertility. - Early menopause: The drop in estrogen levels caused by removing the ovaries can cause early menopause. Symptoms of menopause include the following: - Hot flashes. - Night sweats. - Trouble sleeping. - Mood changes. - Decreased sex drive. - Heart disease. - Vaginal dryness. - Frequent urination. - Osteoporosis (decreased bone density). These symptoms may not be the same in all women. Hormone replacement therapy (HRT) may be used to lessen these symptoms. - Risk of ovarian cancer in the peritoneum: Women who have had a risk-reducing salpingo-oophorectomy continue to have a small risk of ovarian cancer in the peritoneum (thin layer of tissue that lines the inside of the abdomen). This may occur if ovarian cancer cells had already spread to the peritoneum before the surgery or if some ovarian tissue remains after surgery. It is not clear whether the following affect the risk of ovarian, fallopian tube, and primary peritoneal cancer: Diet Studies of dietary factors including various foods, teas, and nutrients have not found a strong link to ovarian cancer. Alcohol Studies have not shown a link between drinking alcohol and the risk of ovarian cancer. Aspirin and non-steroidal anti-inflammatory drugs Some studies of aspirin and non-steroidal anti-inflammatory drugs (NSAIDs) have found a decreased risk of ovarian cancer and others have not. Smoking Some studies found a very small increased risk of one rare type of ovarian cancer in women who were current smokers compared with women who never smoked. Talc Studies of women who used talcum powder (talc) dusted on the perineum (the area between the vagina and the anus) have not found clear evidence of an increased risk of ovarian cancer. Infertility treatment Overall, studies in women using fertility drugs have not found clear evidence of an increased risk of ovarian cancer. Risk of ovarian borderline malignant tumors may be higher in women who take fertility drugs. The risk of invasive ovarian cancer may be higher in women who do not get pregnant after taking fertility drugs. Cancer prevention clinical trials are used to study ways to prevent cancer. Cancer prevention clinical trials are used to study ways to lower the risk of developing certain types of cancer. Some cancer prevention trials are conducted with healthy people who have not had cancer but who have an increased risk for cancer. Other prevention trials are conducted with people who have had cancer and are trying to prevent another cancer of the same type or to lower their chance of developing a new type of cancer. Other trials are done with healthy volunteers who are not known to have any risk factors for cancer. The purpose of some cancer prevention clinical trials is to find out whether actions people take can prevent cancer. These may include eating fruits and vegetables, exercising, quitting smoking, or taking certain medicines, vitamins, minerals, or food supplements. New ways to prevent ovarian, fallopian tube, and primary peritoneal cancer are being studied in clinical trials. Clinical trials are taking place in many parts of the country. Information about clinical trials can be found in the Clinical Trials section of the NCI website. Check NCI's list of cancer clinical trials for ovarian cancer prevention trials that are now accepting patients.","Ovarian, Fallopian Tube, and Primary Peritoneal Cancer",0000019_4,CancerGov,https://www.cancer.gov/types/ovarian/patient/ovarian-prevention-pdq,C1299307,T191,Disorders "Who is at risk for Ovarian, Fallopian Tube, and Primary Peritoneal Cancer? ?",0000019_4-3,susceptibility,"Key Points - Avoiding risk factors and increasing protective factors may help prevent cancer. - The following are risk factors for ovarian, fallopian tube, and primary peritoneal cancer: - Family history of ovarian, fallopian tube, and primary peritoneal cancer - Inherited risk - Hormone replacement therapy - Weight and height - The following are protective factors for ovarian, fallopian tube, and primary peritoneal cancer: - Oral contraceptives - Tubal ligation - Breastfeeding - Risk-reducing salpingo-oophorectomy - It is not clear whether the following affect the risk of ovarian, fallopian tube, and primary peritoneal cancer: - Diet - Alcohol - Aspirin and non-steroidal anti-inflammatory drugs - Smoking - Talc - Infertility treatment - Cancer prevention clinical trials are used to study ways to prevent cancer. - New ways to prevent ovarian, fallopian tube, and primary peritoneal cancer are being studied in clinical trials. Avoiding risk factors and increasing protective factors may help prevent cancer. Avoiding cancer risk factors may help prevent certain cancers. Risk factors include smoking, being overweight, and not getting enough exercise. Increasing protective factors such as quitting smoking and exercising may also help prevent some cancers. Talk to your doctor or other health care professional about how you might lower your risk of cancer. The following are risk factors for ovarian, fallopian tube, and primary peritoneal cancer: Family history of ovarian, fallopian tube, and primary peritoneal cancer A woman whose mother or sister had ovarian cancer has an increased risk of ovarian cancer. A woman with two or more relatives with ovarian cancer also has an increased risk of ovarian cancer. Inherited risk The risk of ovarian cancer is increased in women who have inherited certain changes in the BRCA1, BRCA2, or other genes. The risk of ovarian cancer is also increased in women who have certain inherited syndromes that include: - Familial site-specific ovarian cancer syndrome. - Familial breast/ovarian cancer syndrome. - Hereditary nonpolyposis colorectal cancer (HNPCC; Lynch syndrome). Hormone replacement therapy The use of estrogen -only hormone replacement therapy (HRT) after menopause is linked to a slightly increased risk of ovarian cancer in women who are taking HRT or have taken HRT within the past 3 years. The risk of ovarian cancer increases the longer a woman uses estrogen-only HRT. When hormone therapy is stopped, the risk of ovarian cancer decreases over time. It is not clear whether there is an increased risk of ovarian cancer with the use of HRT that has both estrogen and progestin. Weight and height Being overweight or obese during the teenage years is linked to an increased risk of ovarian cancer. Being obese is linked to an increased risk of death from ovarian cancer. Being tall (5'8"" or taller) may also be linked to a slight increase in the risk of ovarian cancer. It is not clear whether the following affect the risk of ovarian, fallopian tube, and primary peritoneal cancer: Diet Studies of dietary factors including various foods, teas, and nutrients have not found a strong link to ovarian cancer. Alcohol Studies have not shown a link between drinking alcohol and the risk of ovarian cancer. Aspirin and non-steroidal anti-inflammatory drugs Some studies of aspirin and non-steroidal anti-inflammatory drugs (NSAIDs) have found a decreased risk of ovarian cancer and others have not. Smoking Some studies found a very small increased risk of one rare type of ovarian cancer in women who were current smokers compared with women who never smoked. Talc Studies of women who used talcum powder (talc) dusted on the perineum (the area between the vagina and the anus) have not found clear evidence of an increased risk of ovarian cancer. Infertility treatment Overall, studies in women using fertility drugs have not found clear evidence of an increased risk of ovarian cancer. Risk of ovarian borderline malignant tumors may be higher in women who take fertility drugs. The risk of invasive ovarian cancer may be higher in women who do not get pregnant after taking fertility drugs.","Ovarian, Fallopian Tube, and Primary Peritoneal Cancer",0000019_4,CancerGov,https://www.cancer.gov/types/ovarian/patient/ovarian-prevention-pdq,C1299307,T191,Disorders "what research (or clinical trials) is being done for Ovarian, Fallopian Tube, and Primary Peritoneal Cancer ?",0000019_4-4,research,"Cancer prevention clinical trials are used to study ways to prevent cancer. Cancer prevention clinical trials are used to study ways to lower the risk of developing certain types of cancer. Some cancer prevention trials are conducted with healthy people who have not had cancer but who have an increased risk for cancer. Other prevention trials are conducted with people who have had cancer and are trying to prevent another cancer of the same type or to lower their chance of developing a new type of cancer. Other trials are done with healthy volunteers who are not known to have any risk factors for cancer. The purpose of some cancer prevention clinical trials is to find out whether actions people take can prevent cancer. These may include eating fruits and vegetables, exercising, quitting smoking, or taking certain medicines, vitamins, minerals, or food supplements. New ways to prevent ovarian, fallopian tube, and primary peritoneal cancer are being studied in clinical trials. Clinical trials are taking place in many parts of the country. Information about clinical trials can be found in the Clinical Trials section of the NCI website. Check NCI's list of cancer clinical trials for ovarian cancer prevention trials that are now accepting patients.","Ovarian, Fallopian Tube, and Primary Peritoneal Cancer",0000019_4,CancerGov,https://www.cancer.gov/types/ovarian/patient/ovarian-prevention-pdq,C1299307,T191,Disorders "What is (are) Ovarian, Fallopian Tube, and Primary Peritoneal Cancer ?",0000019_5-1,information,"Key Points - Ovarian, fallopian tube, and primary peritoneal cancers are diseases in which malignant (cancer) cells form in the ovaries, fallopian tubes, or peritoneum. - In the United States, ovarian cancer is the fifth leading cause of cancer death in women. - Different factors increase or decrease the risk of getting ovarian, fallopian tube, and primary peritoneal cancer. Ovarian, fallopian tube, and primary peritoneal cancers are diseases in which malignant (cancer) cells form in the ovaries, fallopian tubes, or peritoneum. The ovaries are a pair of organs in the female reproductive system. They are located in the pelvis, one on each side of the uterus (the hollow, pear-shaped organ where a fetus grows). Each ovary is about the size and shape of an almond. The ovaries produce eggs and female hormones (chemicals that control the way certain cells or organs function). The fallopian tubes are a pair of long, slender tubes, one on each side of the uterus. Eggs pass from the ovaries, through the fallopian tubes, to the uterus. Cancer sometimes begins at the end of the fallopian tube near the ovary and spreads to the ovary. The peritoneum is the tissue that lines the abdominal wall and covers organs in the abdomen. Primary peritoneal cancer is cancer that forms in the peritoneum and has not spread there from another part of the body. Cancer sometimes begins in the peritoneum and spreads to the ovary. Ovarian epithelial cancer, fallopian tube cancer, and primary peritoneal cancer form in the same type of tissue. Studies of screening tests look at these cancers together. See the following PDQ summaries for more information about ovarian, fallopian tube, and primary peritoneal cancers: - Ovarian, Fallopian Tube, and Primary Peritoneal Cancer Prevention - Genetics of Breast and Gynecologic Cancers - Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer Treatment - Ovarian Germ Cell Tumors Treatment - Ovarian Low Malignant Potential Tumors Treatment In the United States, ovarian cancer is the fifth leading cause of cancer death in women. Ovarian cancer is also the leading cause of death from cancer of the female reproductive system. Over the last 20 years, the number of new cases of ovarian cancer has gone down slightly in white women and in black women. Since 2005, the number of deaths from ovarian cancer also decreased slightly in white and black women.","Ovarian, Fallopian Tube, and Primary Peritoneal Cancer",0000019_5,CancerGov,https://www.cancer.gov/types/ovarian/patient/ovarian-screening-pdq,C1299307,T191,Disorders "Who is at risk for Ovarian, Fallopian Tube, and Primary Peritoneal Cancer? ?",0000019_5-2,susceptibility,"Different factors increase or decrease the risk of getting ovarian, fallopian tube, and primary peritoneal cancer. Anything that increases your chance of getting a disease is called a risk factor. Anything that decreases your chance of getting a disease is called a protective factor. For information about risk factors and protective factors for ovarian cancer, see the Ovarian, Fallopian Tube, and Primary Peritoneal Cancer Prevention summary. Talk to your doctor about your risk of ovarian cancer. Screening tests have risks. Decisions about screening tests can be difficult. Not all screening tests are helpful and most have risks. Before having any screening test, you may want to talk about the test with your doctor. It is important to know the risks of the test and whether it has been proven to reduce the risk of dying from cancer. The risks of ovarian, fallopian tube, and primary peritoneal cancer screening tests include the following: Finding ovarian, fallopian tube, and primary peritoneal cancer may not improve health or help a woman live longer. Screening may not improve your health or help you live longer if you have advanced ovarian cancer or if it has already spread to other places in your body. False-negative test results can occur. Screening test results may appear to be normal even though ovarian cancer is present. A woman who receives a false-negative test result (one that shows there is no cancer when there really is) may delay seeking medical care even if she has symptoms. False-positive test results can occur. Screening test results may appear to be abnormal even though no cancer is present. A false-positive test result (one that shows there is cancer when there really isn't) can cause anxiety and is usually followed by more tests (such as a laparoscopy or a laparotomy to see if cancer is present), which also have risks. Problems caused by tests used to diagnose ovarian cancer include infection, blood loss, bowel injury, and heart and blood vessel problems. A false-positive test result can also lead to an unneeded oophorectomy (removal of one or both ovaries).","Ovarian, Fallopian Tube, and Primary Peritoneal Cancer",0000019_5,CancerGov,https://www.cancer.gov/types/ovarian/patient/ovarian-screening-pdq,C1299307,T191,Disorders What is (are) Gallbladder Cancer ?,0000020_1-1,information,"Key Points - Gallbladder cancer is a disease in which malignant (cancer) cells form in the tissues of the gallbladder. - Being female can increase the risk of developing gallbladder cancer. - Signs and symptoms of gallbladder cancer include jaundice, fever, and pain. - Gallbladder cancer is difficult to detect (find) and diagnose early. - Tests that examine the gallbladder and nearby organs are used to detect (find), diagnose, and stage gallbladder cancer. - Certain factors affect the prognosis (chance of recovery) and treatment options. Gallbladder cancer is a disease in which malignant (cancer) cells form in the tissues of the gallbladder. Gallbladder cancer is a rare disease in which malignant (cancer) cells are found in the tissues of the gallbladder. The gallbladder is a pear-shaped organ that lies just under the liver in the upper abdomen. The gallbladder stores bile, a fluid made by the liver to digest fat. When food is being broken down in the stomach and intestines, bile is released from the gallbladder through a tube called the common bile duct, which connects the gallbladder and liver to the first part of the small intestine. The wall of the gallbladder has 3 main layers of tissue. - Mucosal (inner) layer. - Muscularis (middle, muscle) layer. - Serosal (outer) layer. Between these layers is supporting connective tissue. Primary gallbladder cancer starts in the inner layer and spreads through the outer layers as it grows. Gallbladder cancer is difficult to detect (find) and diagnose early. Gallbladder cancer is difficult to detect and diagnose for the following reasons: - There are no signs or symptoms in the early stages of gallbladder cancer. - The symptoms of gallbladder cancer, when present, are like the symptoms of many other illnesses. - The gallbladder is hidden behind the liver. Gallbladder cancer is sometimes found when the gallbladder is removed for other reasons. Patients with gallstones rarely develop gallbladder cancer.",Gallbladder Cancer,0000020_1,CancerGov,https://www.cancer.gov/types/gallbladder/patient/gallbladder-treatment-pdq,C0153452,T191,Disorders Who is at risk for Gallbladder Cancer? ?,0000020_1-2,susceptibility,Being female can increase the risk of developing gallbladder cancer. Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for gallbladder cancer include the following: - Being female. - Being Native American.,Gallbladder Cancer,0000020_1,CancerGov,https://www.cancer.gov/types/gallbladder/patient/gallbladder-treatment-pdq,C0153452,T191,Disorders What are the symptoms of Gallbladder Cancer ?,0000020_1-3,symptoms,"Signs and symptoms of gallbladder cancer include jaundice, fever, and pain. These and other signs and symptoms may be caused by gallbladder cancer or by other conditions. Check with your doctor if you have any of the following: - Jaundice (yellowing of the skin and whites of the eyes). - Pain above the stomach. - Fever. - Nausea and vomiting. - Bloating. - Lumps in the abdomen.",Gallbladder Cancer,0000020_1,CancerGov,https://www.cancer.gov/types/gallbladder/patient/gallbladder-treatment-pdq,C0153452,T191,Disorders How to diagnose Gallbladder Cancer ?,0000020_1-4,exams and tests,"Tests that examine the gallbladder and nearby organs are used to detect (find), diagnose, and stage gallbladder cancer. Procedures that make pictures of the gallbladder and the area around it help diagnose gallbladder cancer and show how far the cancer has spread. The process used to find out if cancer cells have spread within and around the gallbladder is called staging. In order to plan treatment, it is important to know if the gallbladder cancer can be removed by surgery. Tests and procedures to detect, diagnose, and stage gallbladder cancer are usually done at the same time. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Liver function tests : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by the liver. A higher than normal amount of a substance can be a sign of liver disease that may be caused by gallbladder cancer. - Carcinoembryonic antigen (CEA) assay : A test that measures the level of CEA in the blood. CEA is released into the bloodstream from both cancer cells and normal cells. When found in higher than normal amounts, it can be a sign of gallbladder cancer or other conditions. - CA 19-9 assay : A test that measures the level of CA 19-9 in the blood. CA 19-9 is released into the bloodstream from both cancer cells and normal cells. When found in higher than normal amounts, it can be a sign of gallbladder cancer or other conditions. - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the chest, abdomen, and pelvis, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. An abdominal ultrasound is done to diagnose gallbladder cancer. - PTC (percutaneous transhepatic cholangiography): A procedure used to x-ray the liver and bile ducts. A thin needle is inserted through the skin below the ribs and into the liver. Dye is injected into the liver or bile ducts and an x-ray is taken. If a blockage is found, a thin, flexible tube called a stent is sometimes left in the liver to drain bile into the small intestine or a collection bag outside the body. - Chest x-ray : An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. - ERCP (endoscopic retrograde cholangiopancreatography): A procedure used to x-ray the ducts (tubes) that carry bile from the liver to the gallbladder and from the gallbladder to the small intestine. Sometimes gallbladder cancer causes these ducts to narrow and block or slow the flow of bile, causing jaundice. An endoscope (a thin, lighted tube) is passed through the mouth, esophagus, and stomach into the first part of the small intestine. A catheter (a smaller tube) is then inserted through the endoscope into the bile ducts. A dye is injected through the catheter into the ducts and an x-ray is taken. If the ducts are blocked by a tumor, a fine tube may be inserted into the duct to unblock it. This tube (or stent) may be left in place to keep the duct open. Tissue samples may also be taken. - Laparoscopy : A surgical procedure to look at the organs inside the abdomen to check for signs of disease. Small incisions (cuts) are made in the wall of the abdomen and a laparoscope (a thin, lighted tube) is inserted into one of the incisions. Other instruments may be inserted through the same or other incisions to perform procedures such as removing organs or taking tissue samples for biopsy. The laparoscopy helps to find out if the cancer is within the gallbladder only or has spread to nearby tissues and if it can be removed by surgery. - Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. The biopsy may be done after surgery to remove the tumor. If the tumor clearly cannot be removed by surgery, the biopsy may be done using a fine needle to remove cells from the tumor.",Gallbladder Cancer,0000020_1,CancerGov,https://www.cancer.gov/types/gallbladder/patient/gallbladder-treatment-pdq,C0153452,T191,Disorders What is the outlook for Gallbladder Cancer ?,0000020_1-5,outlook,"Certain factors affect the prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - The stage of the cancer (whether the cancer has spread from the gallbladder to other places in the body). - Whether the cancer can be completely removed by surgery. - The type of gallbladder cancer (how the cancer cell looks under a microscope). - Whether the cancer has just been diagnosed or has recurred (come back). Treatment may also depend on the age and general health of the patient and whether the cancer is causing signs or symptoms. Gallbladder cancer can be cured only if it is found before it has spread, when it can be removed by surgery. If the cancer has spread, palliative treatment can improve the patient's quality of life by controlling the symptoms and complications of this disease. Taking part in one of the clinical trials being done to improve treatment should be considered. Information about ongoing clinical trials is available from the NCI website.",Gallbladder Cancer,0000020_1,CancerGov,https://www.cancer.gov/types/gallbladder/patient/gallbladder-treatment-pdq,C0153452,T191,Disorders What are the stages of Gallbladder Cancer ?,0000020_1-6,stages,"Key Points - Tests and procedures to stage gallbladder cancer are usually done at the same time as diagnosis. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - The following stages are used for gallbladder cancer: - Stage 0 (Carcinoma in Situ) - Stage I - Stage II - Stage IIIA - Stage IIIB - Stage IVA - Stage IVB - For gallbladder cancer, stages are also grouped according to how the cancer may be treated. There are two treatment groups: - Localized (Stage I) - Unresectable, recurrent, or metastatic (Stage II, Stage III, and Stage IV) Tests and procedures to stage gallbladder cancer are usually done at the same time as diagnosis. See the General Information section for a description of tests and procedures used to detect, diagnose, and stage gallbladder cancer. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if gallbladder cancer spreads to the liver, the cancer cells in the liver are actually gallbladder cancer cells. The disease is metastatic gallbladder cancer, not liver cancer. The following stages are used for gallbladder cancer: Stage 0 (Carcinoma in Situ) In stage 0, abnormal cells are found in the inner (mucosal) layer of the gallbladder. These abnormal cells may become cancer and spread into nearby normal tissue. Stage 0 is also called carcinoma in situ. Stage I In stage I, cancer has formed and has spread beyond the inner (mucosal) layer to a layer of tissue with blood vessels or to the muscle layer. Stage II In stage II, cancer has spread beyond the muscle layer to the connective tissue around the muscle. Stage IIIA In stage IIIA, cancer has spread through the thin layers of tissue that cover the gallbladder and/or to the liver and/or to one nearby organ (such as the stomach, small intestine, colon, pancreas, or bile ducts outside the liver). Stage IIIB In stage IIIB, cancer has spread to nearby lymph nodes and: - beyond the inner layer of the gallbladder to a layer of tissue with blood vessels or to the muscle layer; or - beyond the muscle layer to the connective tissue around the muscle; or - through the thin layers of tissue that cover the gallbladder and/or to the liver and/or to one nearby organ (such as the stomach, small intestine, colon, pancreas, or bile ducts outside the liver). Stage IVA In stage IVA, cancer has spread to a main blood vessel of the liver or to 2 or more nearby organs or areas other than the liver. Cancer may have spread to nearby lymph nodes. Stage IVB In stage IVB, cancer has spread to either: - lymph nodes along large arteries in the abdomen and/or near the lower part of the backbone; or - to organs or areas far away from the gallbladder. For gallbladder cancer, stages are also grouped according to how the cancer may be treated. There are two treatment groups: Localized (Stage I) Cancer is found in the wall of the gallbladder and can be completely removed by surgery. Unresectable, recurrent, or metastatic (Stage II, Stage III, and Stage IV) Unresectable cancer cannot be removed completely by surgery. Most patients with gallbladder cancer have unresectable cancer. Recurrent cancer is cancer that has recurred (come back) after it has been treated. Gallbladder cancer may come back in the gallbladder or in other parts of the body. Metastasis is the spread of cancer from the primary site (place where it started) to other places in the body. Metastatic gallbladder cancer may spread to surrounding tissues, organs, throughout the abdominal cavity, or to distant parts of the body.",Gallbladder Cancer,0000020_1,CancerGov,https://www.cancer.gov/types/gallbladder/patient/gallbladder-treatment-pdq,C0153452,T191,Disorders What are the treatments for Gallbladder Cancer ?,0000020_1-7,treatment,"Key Points - There are different types of treatment for patients with gallbladder cancer. - Three types of standard treatment are used: - Surgery - Radiation therapy - Chemotherapy - New types of treatment are being tested in clinical trials. - Radiation sensitizers - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with gallbladder cancer. Different types of treatments are available for patients with gallbladder cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Three types of standard treatment are used: Surgery Gallbladder cancer may be treated with a cholecystectomy, surgery to remove the gallbladder and some of the tissues around it. Nearby lymph nodes may be removed. A laparoscope is sometimes used to guide gallbladder surgery. The laparoscope is attached to a video camera and inserted through an incision (port) in the abdomen. Surgical instruments are inserted through other ports to perform the surgery. Because there is a risk that gallbladder cancer cells may spread to these ports, tissue surrounding the port sites may also be removed. If the cancer has spread and cannot be removed, the following types of palliative surgery may relieve symptoms: - Surgical biliary bypass: If the tumor is blocking the small intestine and bile is building up in the gallbladder, a biliary bypass may be done. During this operation, the gallbladder or bile duct will be cut and sewn to the small intestine to create a new pathway around the blocked area. - Endoscopic stent placement: If the tumor is blocking the bile duct, surgery may be done to put in a stent (a thin, flexible tube) to drain bile that has built up in the area. The stent may be placed through a catheter that drains to the outside of the body or the stent may go around the blocked area and drain the bile into the small intestine. - Percutaneous transhepatic biliary drainage: A procedure done to drain bile when there is a blockage and endoscopic stent placement is not possible. An x-ray of the liver and bile ducts is done to locate the blockage. Images made by ultrasound are used to guide placement of a stent, which is left in the liver to drain bile into the small intestine or a collection bag outside the body. This procedure may be done to relieve jaundice before surgery. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External radiation therapy is used to treat gallbladder cancer. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Radiation sensitizers Clinical trials are studying ways to improve the effect of radiation therapy on tumor cells, including the following: - Hyperthermia therapy: A treatment in which body tissue is exposed to high temperatures to damage and kill cancer cells or to make cancer cells more sensitive to the effects of radiation therapy and certain anticancer drugs. - Radiosensitizers: Drugs that make tumor cells more sensitive to radiation therapy. Giving radiation therapy together with radiosensitizers may kill more tumor cells. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Gallbladder Cancer Localized Gallbladder Cancer Treatment of localized gallbladder cancer may include the following: - Surgery to remove the gallbladder and some of the tissue around it. Part of the liver and nearby lymph nodes may also be removed. Radiation therapy with or without chemotherapy may follow surgery. - Radiation therapy with or without chemotherapy. - A clinical trial of radiation therapy with radiosensitizers. Check the list of NCI-supported cancer clinical trials that are now accepting patients with localized gallbladder cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Unresectable, Recurrent, or Metastatic Gallbladder Cancer Treatment of unresectable, recurrent, or metastatic gallbladder cancer is usually within a clinical trial. Treatment may include the following: - Percutaneous transhepatic biliary drainage or the placement of stents to relieve symptoms caused by blocked bile ducts. This may be followed by radiation therapy as palliative treatment. - Surgery as palliative treatment to relieve symptoms caused by blocked bile ducts. - Chemotherapy. - A clinical trial of new ways to give palliative radiation therapy, such as giving it together with hyperthermia therapy, radiosensitizers, or chemotherapy. - A clinical trial of new drugs and drug combinations. Check the list of NCI-supported cancer clinical trials that are now accepting patients with unresectable gallbladder cancer, recurrent gallbladder cancer and metastatic gallbladder cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Gallbladder Cancer,0000020_1,CancerGov,https://www.cancer.gov/types/gallbladder/patient/gallbladder-treatment-pdq,C0153452,T191,Disorders what research (or clinical trials) is being done for Gallbladder Cancer ?,0000020_1-8,research,"New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Radiation sensitizers Clinical trials are studying ways to improve the effect of radiation therapy on tumor cells, including the following: - Hyperthermia therapy: A treatment in which body tissue is exposed to high temperatures to damage and kill cancer cells or to make cancer cells more sensitive to the effects of radiation therapy and certain anticancer drugs. - Radiosensitizers: Drugs that make tumor cells more sensitive to radiation therapy. Giving radiation therapy together with radiosensitizers may kill more tumor cells. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Gallbladder Cancer,0000020_1,CancerGov,https://www.cancer.gov/types/gallbladder/patient/gallbladder-treatment-pdq,C0153452,T191,Disorders Who is at risk for Testicular Cancer? ?,0000021_1-2,susceptibility,Health history can affect the risk of testicular cancer. Anything that increases the chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesnt mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for testicular cancer include: - Having had an undescended testicle. - Having had abnormal development of the testicles. - Having a personal history of testicular cancer. - Having a family history of testicular cancer (especially in a father or brother). - Being white. Treatment for testicular cancer can cause infertility. Certain treatments for testicular cancer can cause infertility that may be permanent. Patients who may wish to have children should consider sperm banking before having treatment. Sperm banking is the process of freezing sperm and storing it for later use.,Testicular Cancer,0000021_1,CancerGov,https://www.cancer.gov/types/testicular/patient/testicular-treatment-pdq,C0153594,T191,Disorders What are the stages of Testicular Cancer ?,0000021_1-6,stages,"Key Points - After testicular cancer has been diagnosed, tests are done to find out if cancer cells have spread within the testicles or to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - The following stages are used for testicular cancer: - Stage 0 (Testicular Intraepithelial Neoplasia) - Stage I - Stage II - Stage III After testicular cancer has been diagnosed, tests are done to find out if cancer cells have spread within the testicles or to other parts of the body. The process used to find out if cancer has spread within the testicles or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following tests and procedures may be used in the staging process: - Chest x-ray : An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Abdominal lymph node dissection : A surgical procedure in which lymph nodes in the abdomen are removed and a sample of tissue is checked under a microscope for signs of cancer. This procedure is also called lymphadenectomy. For patients with nonseminoma, removing the lymph nodes may help stop the spread of disease. Cancer cells in the lymph nodes of seminoma patients can be treated with radiation therapy. - Serum tumor marker test : A procedure in which a sample of blood is examined to measure the amounts of certain substances released into the blood by organs, tissues, or tumor cells in the body. Certain substances are linked to specific types of cancer when found in increased levels in the blood. These are called tumor markers. The following 3 tumor markers are used in staging testicular cancer: - Alpha-fetoprotein (AFP) - Beta-human chorionic gonadotropin (-hCG). - Lactate dehydrogenase (LDH). Tumor marker levels are measured again, after inguinal orchiectomy and biopsy, in order to determine the stage of the cancer. This helps to show if all of the cancer has been removed or if more treatment is needed. Tumor marker levels are also measured during follow-up as a way of checking if the cancer has come back. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if testicular cancer spreads to the lung, the cancer cells in the lung are actually testicular cancer cells. The disease is metastatic testicular cancer, not lung cancer. The following stages are used for testicular cancer: Stage 0 (Testicular Intraepithelial Neoplasia) In stage 0, abnormal cells are found in the tiny tubules where the sperm cells begin to develop. These abnormal cells may become cancer and spread into nearby normal tissue. All tumor marker levels are normal. Stage 0 is also called testicular intraepithelial neoplasia and testicular intratubular germ cell neoplasia. Stage I In stage I, cancer has formed. Stage I is divided into stage IA, stage IB, and stage IS and is determined after an inguinal orchiectomy is done. - In stage IA, cancer is in the testicle and epididymis and may have spread to the inner layer of the membrane surrounding the testicle. All tumor marker levels are normal. - In stage IB, cancer: - is in the testicle and the epididymis and has spread to the blood vessels or lymph vessels in the testicle; or - has spread to the outer layer of the membrane surrounding the testicle; or - is in the spermatic cord or the scrotum and may be in the blood vessels or lymph vessels of the testicle. All tumor marker levels are normal. - In stage IS, cancer is found anywhere within the testicle, spermatic cord, or the scrotum and either: - all tumor marker levels are slightly above normal; or - one or more tumor marker levels are moderately above normal or high. Stage II Stage II is divided into stage IIA, stage IIB, and stage IIC and is determined after an inguinal orchiectomy is done. - In stage IIA, cancer: - is anywhere within the testicle, spermatic cord, or scrotum; and - has spread to up to 5 lymph nodes in the abdomen, none larger than 2 centimeters. All tumor marker levels are normal or slightly above normal. - In stage IIB, cancer is anywhere within the testicle, spermatic cord, or scrotum; and either: - has spread to up to 5 lymph nodes in the abdomen; at least one of the lymph nodes is larger than 2 centimeters, but none are larger than 5 centimeters; or - has spread to more than 5 lymph nodes; the lymph nodes are not larger than 5 centimeters. All tumor marker levels are normal or slightly above normal. - In stage IIC, cancer: - is anywhere within the testicle, spermatic cord, or scrotum; and - has spread to a lymph node in the abdomen that is larger than 5 centimeters. All tumor marker levels are normal or slightly above normal. Stage III Stage III is divided into stage IIIA, stage IIIB, and stage IIIC and is determined after an inguinal orchiectomy is done. - In stage IIIA, cancer: - is anywhere within the testicle, spermatic cord, or scrotum; and - may have spread to one or more lymph nodes in the abdomen; and - has spread to distant lymph nodes or to the lungs. Tumor marker levels may range from normal to slightly above normal. - In stage IIIB, cancer: - is anywhere within the testicle, spermatic cord, or scrotum; and - may have spread to one or more lymph nodes in the abdomen, to distant lymph nodes, or to the lungs. The level of one or more tumor markers is moderately above normal. - In stage IIIC, cancer: - is anywhere within the testicle, spermatic cord, or scrotum; and - may have spread to one or more lymph nodes in the abdomen, to distant lymph nodes, or to the lungs. The level of one or more tumor markers is high. or Cancer: - is anywhere within the testicle, spermatic cord, or scrotum; and - may have spread to one or more lymph nodes in the abdomen; and - has not spread to distant lymph nodes or the lung but has spread to other parts of the body. Tumor marker levels may range from normal to high.",Testicular Cancer,0000021_1,CancerGov,https://www.cancer.gov/types/testicular/patient/testicular-treatment-pdq,C0153594,T191,Disorders What are the treatments for Testicular Cancer ?,0000021_1-7,treatment,"Key Points - There are different types of treatment for patients with testicular cancer. - Testicular tumors are divided into 3 groups, based on how well the tumors are expected to respond to treatment. - Good Prognosis - Intermediate Prognosis - Poor Prognosis - Five types of standard treatment are used: - Surgery - Radiation therapy - Chemotherapy - Surveillance - High-dose chemotherapy with stem cell transplant - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with testicular cancer. Different types of treatments are available for patients with testicular cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Testicular tumors are divided into 3 groups, based on how well the tumors are expected to respond to treatment. Good Prognosis For nonseminoma, all of the following must be true: - The tumor is found only in the testicle or in the retroperitoneum (area outside or behind the abdominal wall); and - The tumor has not spread to organs other than the lungs; and - The levels of all the tumor markers are slightly above normal. For seminoma, all of the following must be true: - The tumor has not spread to organs other than the lungs; and - The level of alpha-fetoprotein (AFP) is normal. Beta-human chorionic gonadotropin (-hCG) and lactate dehydrogenase (LDH) may be at any level. Intermediate Prognosis For nonseminoma, all of the following must be true: - The tumor is found in one testicle only or in the retroperitoneum (area outside or behind the abdominal wall); and - The tumor has not spread to organs other than the lungs; and - The level of any one of the tumor markers is more than slightly above normal. For seminoma, all of the following must be true: - The tumor has spread to organs other than the lungs; and - The level of AFP is normal. -hCG and LDH may be at any level. Poor Prognosis For nonseminoma, at least one of the following must be true: - The tumor is in the center of the chest between the lungs; or - The tumor has spread to organs other than the lungs; or - The level of any one of the tumor markers is high. There is no poor prognosis grouping for seminoma testicular tumors. Five types of standard treatment are used: Surgery Surgery to remove the testicle (inguinal orchiectomy) and some of the lymph nodes may be done at diagnosis and staging. (See the General Information and Stages sections of this summary.) Tumors that have spread to other places in the body may be partly or entirely removed by surgery. Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External radiation therapy is used to treat testicular cancer. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. See Drugs Approved for Testicular Cancer for more information. Surveillance Surveillance is closely following a patient's condition without giving any treatment unless there are changes in test results. It is used to find early signs that the cancer has recurred (come back). In surveillance, patients are given certain exams and tests on a regular schedule. High-dose chemotherapy with stem cell transplant High-dose chemotherapy with stem cell transplant is a method of giving high doses of chemotherapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the bodys blood cells. See Drugs Approved for Testicular Cancer for more information. New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Men who have had testicular cancer have an increased risk of developing cancer in the other testicle. A patient is advised to regularly check the other testicle and report any unusual symptoms to a doctor right away. Long-term clinical exams are very important. The patient will probably have check-ups frequently during the first year after surgery and less often after that. Treatment Options by Stage Stage 0 (Testicular Intraepithelial Neoplasia) Treatment of stage 0 may include the following: - Radiation therapy. - Surveillance. - Surgery to remove the testicle. Check the list of NCI-supported cancer clinical trials that are now accepting patients with testicular cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage I Testicular Cancer Treatment of stage I testicular cancer depends on whether the cancer is a seminoma or a nonseminoma. Treatment of seminoma may include the following: - Surgery to remove the testicle, followed by surveillance. - For patients who want active treatment rather than surveillance, treatment may include: - - Surgery to remove the testicle, followed by chemotherapy. Treatment of nonseminoma may include the following: - Surgery to remove the testicle, with long-term follow-up. - Surgery to remove the testicle and lymph nodes in the abdomen, with long-term follow-up. - Surgery followed by chemotherapy for patients at high risk of recurrence, with long-term follow-up. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I testicular cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage II Testicular Cancer Treatment of stage II testicular cancer depends on whether the cancer is a seminoma or a nonseminoma. Treatment of seminoma may include the following: - When the tumor is 5 centimeters or smaller: - Surgery to remove the testicle, followed by radiation therapy to lymph nodes in the abdomen and pelvis. - Combination chemotherapy. - Surgery to remove the testicle and lymph nodes in the abdomen. - When the tumor is larger than 5 centimeters: - Surgery to remove the testicle, followed by combination chemotherapy or radiation therapy to lymph nodes in the abdomen and pelvis, with long-term follow-up. Treatment of nonseminoma may include the following: - Surgery to remove the testicle and lymph nodes, with long-term follow-up. - Surgery to remove the testicle and lymph nodes, followed by combination chemotherapy and long-term follow-up. - Surgery to remove the testicle, followed by combination chemotherapy and a second surgery if cancer remains, with long-term follow-up. - Combination chemotherapy before surgery to remove the testicle, for cancer that has spread and is thought to be life-threatening. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage II testicular cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage III Testicular Cancer Treatment of stage III testicular cancer depends on whether the cancer is a seminoma or a nonseminoma. Treatment of seminoma may include the following: - Surgery to remove the testicle, followed by combination chemotherapy. If there are tumors remaining after chemotherapy, treatment may be one of the following: - Surveillance with no treatment unless tumors grow. - Surveillance for tumors smaller than 3 centimeters and surgery to remove tumors larger than 3 centimeters. - A PET scan two months after chemotherapy and surgery to remove tumors that show up with cancer on the scan. - A clinical trial of chemotherapy. Treatment of nonseminoma may include the following: - Surgery to remove the testicle, followed by combination chemotherapy. - Combination chemotherapy followed by surgery to remove the testicle and all remaining tumors. Additional chemotherapy may be given if the tumor tissue removed contains cancer cells that are growing or if follow-up tests show that cancer is progressing. - Combination chemotherapy before surgery to remove the testicle, for cancer that has spread and is thought to be life-threatening. - A clinical trial of chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III testicular cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Testicular Cancer,0000021_1,CancerGov,https://www.cancer.gov/types/testicular/patient/testicular-treatment-pdq,C0153594,T191,Disorders what research (or clinical trials) is being done for Testicular Cancer ?,0000021_1-8,research,"New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Testicular Cancer,0000021_1,CancerGov,https://www.cancer.gov/types/testicular/patient/testicular-treatment-pdq,C0153594,T191,Disorders What is (are) Testicular Cancer ?,0000021_2-1,information,Key Points - Testicular cancer is a disease in which malignant (cancer) cells form in the tissues of one or both testicles. - Testicular cancer is the most common cancer in men aged 15 to 34 years. - Testicular cancer can usually be cured. - A condition called cryptorchidism (an undescended testicle) is a risk factor for testicular cancer. Testicular cancer is a disease in which malignant (cancer) cells form in the tissues of one or both testicles. The testicles are 2 egg-shaped glands inside the scrotum (a sac of loose skin that lies directly below the penis). The testicles are held within the scrotum by the spermatic cord. The spermatic cord also contains the vas deferens and vessels and nerves of the testicles. The testicles are the male sex glands and make testosterone and sperm. Germ cells in the testicles make immature sperm. These sperm travel through a network of tubules (tiny tubes) and larger tubes into the epididymis (a long coiled tube next to the testicles). This is where the sperm mature and are stored. Almost all testicular cancers start in the germ cells. The two main types of testicular germ cell tumors are seminomas and nonseminomas. See the PDQ summary on Testicular Cancer Treatment for more information about testicular cancer.,Testicular Cancer,0000021_2,CancerGov,https://www.cancer.gov/types/testicular/patient/testicular-screening-pdq,C0153594,T191,Disorders Who is at risk for Testicular Cancer? ?,0000021_2-2,susceptibility,"Testicular cancer is the most common cancer in men aged 15 to 34 years. Testicular cancer is very rare, but it is the most common cancer found in men between the ages of 15 and 34. White men are four times more likely than black men to have testicular cancer",Testicular Cancer,0000021_2,CancerGov,https://www.cancer.gov/types/testicular/patient/testicular-screening-pdq,C0153594,T191,Disorders What is the outlook for Testicular Cancer ?,0000021_2-3,outlook,"Testicular cancer can usually be cured. Although the number of new cases of testicular cancer has doubled in the last 40 years, the number of deaths caused by testicular cancer has decreased greatly because of better treatments. Testicular cancer can usually be cured, even in late stages of the disease. (See the PDQ summary on Testicular Cancer Treatment for more information.)",Testicular Cancer,0000021_2,CancerGov,https://www.cancer.gov/types/testicular/patient/testicular-screening-pdq,C0153594,T191,Disorders Who is at risk for Testicular Cancer? ?,0000021_2-4,susceptibility,"A condition called cryptorchidism (an undescended testicle) is a risk factor for testicular cancer. Anything that increases the chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk to your doctor if you think you may be at risk. Risk factors for testicular cancer include the following: - Having cryptorchidism (an undescended testicle). - Having a testicle that is not normal, such as a small testicle that does not work the way it should. - Having testicular carcinoma in situ. - Being white. - Having a personal or family history of testicular cancer. - Having Klinefelter syndrome. Men who have cryptorchidism, a testicle that is not normal, or testicular carcinoma in situ have an increased risk of testicular cancer in one or both testicles, and need to be followed closely.",Testicular Cancer,0000021_2,CancerGov,https://www.cancer.gov/types/testicular/patient/testicular-screening-pdq,C0153594,T191,Disorders What is (are) Gestational Trophoblastic Disease ?,0000022_1-1,information,"Key Points - Gestational trophoblastic disease (GTD) is a group of rare diseases in which abnormal trophoblast cells grow inside the uterus after conception. - Hydatidiform mole (HM) is the most common type of GTD. - Gestational trophoblastic neoplasia (GTN) is a type of gestational trophoblastic disease (GTD) that is almost always malignant. - Invasive moles - Choriocarcinomas - Placental-site trophoblastic tumors - Epithelioid trophoblastic tumors - Age and a previous molar pregnancy affect the risk of GTD. - Signs of GTD include abnormal vaginal bleeding and a uterus that is larger than normal. - Tests that examine the uterus are used to detect (find) and diagnose gestational trophoblastic disease. - Certain factors affect prognosis (chance of recovery) and treatment options. Gestational trophoblastic disease (GTD) is a group of rare diseases in which abnormal trophoblast cells grow inside the uterus after conception. In gestational trophoblastic disease (GTD), a tumor develops inside the uterus from tissue that forms after conception (the joining of sperm and egg). This tissue is made of trophoblast cells and normally surrounds the fertilized egg in the uterus. Trophoblast cells help connect the fertilized egg to the wall of the uterus and form part of the placenta (the organ that passes nutrients from the mother to the fetus). Sometimes there is a problem with the fertilized egg and trophoblast cells. Instead of a healthy fetus developing, a tumor forms. Until there are signs or symptoms of the tumor, the pregnancy will seem like a normal pregnancy. Most GTD is benign (not cancer) and does not spread, but some types become malignant (cancer) and spread to nearby tissues or distant parts of the body. Gestational trophoblastic disease (GTD) is a general term that includes different types of disease: - Hydatidiform Moles (HM) - Complete HM. - Partial HM. - Gestational Trophoblastic Neoplasia (GTN) - Invasive moles. - Choriocarcinomas. - Placental-site trophoblastic tumors (PSTT; very rare). - Epithelioid trophoblastic tumors (ETT; even more rare). Hydatidiform mole (HM) is the most common type of GTD. HMs are slow-growing tumors that look like sacs of fluid. An HM is also called a molar pregnancy. The cause of hydatidiform moles is not known. HMs may be complete or partial: - A complete HM forms when sperm fertilizes an egg that does not contain the mothers DNA. The egg has DNA from the father and the cells that were meant to become the placenta are abnormal. - A partial HM forms when sperm fertilizes a normal egg and there are two sets of DNA from the father in the fertilized egg. Only part of the fetus forms and the cells that were meant to become the placenta are abnormal. Most hydatidiform moles are benign, but they sometimes become cancer. Having one or more of the following risk factors increases the risk that a hydatidiform mole will become cancer: - A pregnancy before 20 or after 35 years of age. - A very high level of beta human chorionic gonadotropin (-hCG), a hormone made by the body during pregnancy. - A large tumor in the uterus. - An ovarian cyst larger than 6 centimeters. - High blood pressure during pregnancy. - An overactive thyroid gland (extra thyroid hormone is made). - Severe nausea and vomiting during pregnancy. - Trophoblastic cells in the blood, which may block small blood vessels. - Serious blood clotting problems caused by the HM. Gestational trophoblastic neoplasia (GTN) is a type of gestational trophoblastic disease (GTD) that is almost always malignant. Gestational trophoblastic neoplasia (GTN) includes the following: Invasive moles Invasive moles are made up of trophoblast cells that grow into the muscle layer of the uterus. Invasive moles are more likely to grow and spread than a hydatidiform mole. Rarely, a complete or partial HM may become an invasive mole. Sometimes an invasive mole will disappear without treatment. Choriocarcinomas A choriocarcinoma is a malignant tumor that forms from trophoblast cells and spreads to the muscle layer of the uterus and nearby blood vessels. It may also spread to other parts of the body, such as the brain, lungs, liver, kidney, spleen, intestines, pelvis, or vagina. A choriocarcinoma is more likely to form in women who have had any of the following: - Molar pregnancy, especially with a complete hydatidiform mole. - Normal pregnancy. - Tubal pregnancy (the fertilized egg implants in the fallopian tube rather than the uterus). - Miscarriage. Placental-site trophoblastic tumors A placental-site trophoblastic tumor (PSTT) is a rare type of gestational trophoblastic neoplasia that forms where the placenta attaches to the uterus. The tumor forms from trophoblast cells and spreads into the muscle of the uterus and into blood vessels. It may also spread to the lungs, pelvis, or lymph nodes. A PSTT grows very slowly and signs or symptoms may appear months or years after a normal pregnancy. Epithelioid trophoblastic tumors An epithelioid trophoblastic tumor (ETT) is a very rare type of gestational trophoblastic neoplasia that may be benign or malignant. When the tumor is malignant, it may spread to the lungs.",Gestational Trophoblastic Disease,0000022_1,CancerGov,https://www.cancer.gov/types/gestational-trophoblastic/patient/gtd-treatment-pdq,C1135868,T191,Disorders Who is at risk for Gestational Trophoblastic Disease? ?,0000022_1-2,susceptibility,Age and a previous molar pregnancy affect the risk of GTD. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk to your doctor if you think you may be at risk. Risk factors for GTD include the following: - Being pregnant when you are younger than 20 or older than 35 years of age. - Having a personal history of hydatidiform mole.,Gestational Trophoblastic Disease,0000022_1,CancerGov,https://www.cancer.gov/types/gestational-trophoblastic/patient/gtd-treatment-pdq,C1135868,T191,Disorders What are the symptoms of Gestational Trophoblastic Disease ?,0000022_1-3,symptoms,"Signs of GTD include abnormal vaginal bleeding and a uterus that is larger than normal. These and other signs and symptoms may be caused by gestational trophoblastic disease or by other conditions. Check with your doctor if you have any of the following: - Vaginal bleeding not related to menstruation. - A uterus that is larger than expected during pregnancy. - Pain or pressure in the pelvis. - Severe nausea and vomiting during pregnancy. - High blood pressure with headache and swelling of feet and hands early in the pregnancy. - Vaginal bleeding that continues for longer than normal after delivery. - Fatigue, shortness of breath, dizziness, and a fast or irregular heartbeat caused by anemia. GTD sometimes causes an overactive thyroid. Signs and symptoms of an overactive thyroid include the following: - Fast or irregular heartbeat. - Shakiness. - Sweating. - Frequent bowel movements. - Trouble sleeping. - Feeling anxious or irritable. - Weight loss.",Gestational Trophoblastic Disease,0000022_1,CancerGov,https://www.cancer.gov/types/gestational-trophoblastic/patient/gtd-treatment-pdq,C1135868,T191,Disorders How to diagnose Gestational Trophoblastic Disease ?,0000022_1-4,exams and tests,"Tests that examine the uterus are used to detect (find) and diagnose gestational trophoblastic disease. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Pelvic exam : An exam of the vagina, cervix, uterus, fallopian tubes, ovaries, and rectum. A speculum is inserted into the vagina and the doctor or nurse looks at the vagina and cervix for signs of disease. A Pap test of the cervix is usually done. The doctor or nurse also inserts one or two lubricated, gloved fingers of one hand into the vagina and places the other hand over the lower abdomen to feel the size, shape, and position of the uterus and ovaries. The doctor or nurse also inserts a lubricated, gloved finger into the rectum to feel for lumps or abnormal areas. - Ultrasound exam of the pelvis: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs in the pelvis and make echoes. The echoes form a picture of body tissues called a sonogram. Sometimes a transvaginal ultrasound (TVUS) will be done. For TVUS, an ultrasound transducer (probe) is inserted into the vagina to make the sonogram. - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. Blood is also tested to check the liver, kidney, and bone marrow. - Serum tumor marker test : A procedure in which a sample of blood is checked to measure the amounts of certain substances made by organs, tissues, or tumor cells in the body. Certain substances are linked to specific types of cancer when found in increased levels in the body. These are called tumor markers. For GTD, the blood is checked for the level of beta human chorionic gonadotropin (-hCG), a hormone that is made by the body during pregnancy. -hCG in the blood of a woman who is not pregnant may be a sign of GTD. - Urinalysis : A test to check the color of urine and its contents, such as sugar, protein, blood, bacteria, and the level of -hCG.",Gestational Trophoblastic Disease,0000022_1,CancerGov,https://www.cancer.gov/types/gestational-trophoblastic/patient/gtd-treatment-pdq,C1135868,T191,Disorders What is the outlook for Gestational Trophoblastic Disease ?,0000022_1-5,outlook,"Certain factors affect prognosis (chance of recovery) and treatment options. Gestational trophoblastic disease usually can be cured. Treatment and prognosis depend on the following: - The type of GTD. - Whether the tumor has spread to the uterus, lymph nodes, or distant parts of the body. - The number of tumors and where they are in the body. - The size of the largest tumor. - The level of -hCG in the blood. - How soon the tumor was diagnosed after the pregnancy began. - Whether GTD occurred after a molar pregnancy, miscarriage, or normal pregnancy. - Previous treatment for gestational trophoblastic neoplasia. Treatment options also depend on whether the woman wishes to become pregnant in the future.",Gestational Trophoblastic Disease,0000022_1,CancerGov,https://www.cancer.gov/types/gestational-trophoblastic/patient/gtd-treatment-pdq,C1135868,T191,Disorders What are the stages of Gestational Trophoblastic Disease ?,0000022_1-6,stages,"Key Points - After gestational trophoblastic neoplasia has been diagnosed, tests are done to find out if cancer has spread from where it started to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - There is no staging system for hydatidiform moles. - The following stages are used for GTN: - Stage I - Stage II - Stage III - Stage IV - The treatment of gestational trophoblastic neoplasia is based on the type of disease, stage, or risk group. After gestational trophoblastic neoplasia has been diagnosed, tests are done to find out if cancer has spread from where it started to other parts of the body. The process used to find out the extent or spread of cancer is called staging, The information gathered from the staging process helps determine the stage of disease. For GTN, stage is one of the factors used to plan treatment. The following tests and procedures may be done to help find out the stage of the disease: - Chest x-ray : An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body onto film, making pictures of areas inside the body. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - MRI (magnetic resonance imaging) with gadolinium : A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body, such as brain and spinal cord. A substance called gadolinium is injected into a vein. The gadolinium collects around the cancer cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Lumbar puncture : A procedure used to collect cerebrospinal fluid (CSF) from the spinal column. This is done by placing a needle between two bones in the spine and into the CSF around the spinal cord and removing a sample of the fluid. The sample of CSF is checked under a microscope for signs that the cancer has spread to the brain and spinal cord. This procedure is also called an LP or spinal tap. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if choriocarcinoma spreads to the lung, the cancer cells in the lung are actually choriocarcinoma cells. The disease is metastatic choriocarcinoma, not lung cancer. There is no staging system for hydatidiform moles. Hydatidiform moles (HM) are found in the uterus only and do not spread to other parts of the body. The following stages are used for GTN: Stage I In stage I, the tumor is in the uterus only. Stage II In stage II, cancer has spread outside of the uterus to the ovary, fallopian tube, vagina, and/or the ligaments that support the uterus. Stage III In stage III, cancer has spread to the lung. Stage IV In stage IV, cancer has spread to distant parts of the body other than the lungs. The treatment of gestational trophoblastic neoplasia is based on the type of disease, stage, or risk group. Invasive moles and choriocarcinomas are treated based on risk groups. The stage of the invasive mole or choriocarcinoma is one factor used to determine risk group. Other factors include the following: - The age of the patient when the diagnosis is made. - Whether the GTN occurred after a molar pregnancy, miscarriage, or normal pregnancy. - How soon the tumor was diagnosed after the pregnancy began. - The level of beta human chorionic gonadotropin (-hCG) in the blood. - The size of the largest tumor. - Where the tumor has spread to and the number of tumors in the body. - How many chemotherapy drugs the tumor has been treated with (for recurrent or resistant tumors). There are two risk groups for invasive moles and choriocarcinomas: low risk and high risk. Patients with low-risk disease usually receive less aggressive treatment than patients with high-risk disease. Placental-site trophoblastic tumor (PSTT) and epithelioid trophoblastic tumor (ETT) treatments depend on the stage of disease.",Gestational Trophoblastic Disease,0000022_1,CancerGov,https://www.cancer.gov/types/gestational-trophoblastic/patient/gtd-treatment-pdq,C1135868,T191,Disorders What are the treatments for Gestational Trophoblastic Disease ?,0000022_1-7,treatment,"Key Points - There are different types of treatment for patients with gestational trophoblastic disease. - Three types of standard treatment are used: - Surgery - Chemotherapy - Radiation therapy - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with gestational trophoblastic disease. Different types of treatment are available for patients with gestational trophoblastic disease. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. Before starting treatment, patients may want to think about taking part in a clinical trial. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Clinical trials are taking place in many parts of the country. Information about ongoing clinical trials is available from the NCI website. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team. Three types of standard treatment are used: Surgery The doctor may remove the cancer using one of the following operations: - Dilatation and curettage (D&C) with suction evacuation: A surgical procedure to remove abnormal tissue and parts of the inner lining of the uterus. The cervix is dilated and the material inside the uterus is removed with a small vacuum-like device. The walls of the uterus are then gently scraped with a curette (spoon-shaped instrument) to remove any material that may remain in the uterus. This procedure may be used for molar pregnancies. - Hysterectomy: Surgery to remove the uterus, and sometimes the cervix. If the uterus and cervix are taken out through the vagina, the operation is called a vaginal hysterectomy. If the uterus and cervix are taken out through a large incision (cut) in the abdomen, the operation is called a total abdominal hysterectomy. If the uterus and cervix are taken out through a small incision (cut) in the abdomen using a laparoscope, the operation is called a total laparoscopic hysterectomy. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated, or whether the tumor is low-risk or high-risk. Combination chemotherapy is treatment using more than one anticancer drug. Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy after surgery to kill any tumor cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. See Drugs Approved for Gestational Trophoblastic Disease for more information. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type of gestational trophoblastic disease being treated. External radiation therapy is used to treat gestational trophoblastic disease. New types of treatment are being tested in clinical trials. Information about ongoing clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Blood levels of beta human chorionic gonadotropin (-hCG) will be checked for up to 6 months after treatment has ended. This is because a -hCG level that is higher than normal may mean that the tumor has not responded to treatment or it has become cancer. Treatment Options for Gestational Trophoblastic Disease Hydatidiform Moles Treatment of a hydatidiform mole may include the following: - Surgery (Dilatation and curettage with suction evacuation) to remove the tumor. After surgery, beta human chorionic gonadotropin (-hCG) blood tests are done every week until the -hCG level returns to normal. Patients also have follow-up doctor visits monthly for up to 6 months. If the level of -hCG does not return to normal or increases, it may mean the hydatidiform mole was not completely removed and it has become cancer. Pregnancy causes -hCG levels to increase, so your doctor will ask you not to become pregnant until follow-up is finished. For disease that remains after surgery, treatment is usually chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with hydatidiform mole. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Gestational Trophoblastic Neoplasia Low-risk Gestational Trophoblastic Neoplasia Treatment of low-risk gestational trophoblastic neoplasia (GTN) (invasive mole or choriocarcinoma) may include the following: - Chemotherapy with one or more anticancer drugs. Treatment is given until the beta human chorionic gonadotropin (-hCG) level is normal for at least 3 weeks after treatment ends. If the level of -hCG in the blood does not return to normal or the tumor spreads to distant parts of the body, chemotherapy regimens used for high-risk metastatic GTN are given. Check the list of NCI-supported cancer clinical trials that are now accepting patients with low risk metastatic gestational trophoblastic tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. High-risk Metastatic Gestational Trophoblastic Neoplasia Treatment of high-risk metastatic gestational trophoblastic neoplasia (invasive mole or choriocarcinoma) may include the following: - Combination chemotherapy. - Intrathecal chemotherapy and radiation therapy to the brain (for cancer that has spread to the lung, to keep it from spreading to the brain). - High-dose chemotherapy or intrathecal chemotherapy and/or radiation therapy to the brain (for cancer that has spread to the brain). Check the list of NCI-supported cancer clinical trials that are now accepting patients with high risk metastatic gestational trophoblastic tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Placental-Site Gestational Trophoblastic Tumors and Epithelioid Trophoblastic Tumors Treatment of stage I placental-site gestational trophoblastic tumors and epithelioid trophoblastic tumors may include the following: - Surgery to remove the uterus. Treatment of stage II placental-site gestational trophoblastic tumors and epithelioid trophoblastic tumors may include the following: - Surgery to remove the tumor, which may be followed by combination chemotherapy. Treatment of stage III and IV placental-site gestational trophoblastic tumors and epithelioid trophoblastic tumors may include following: - Combination chemotherapy. - Surgery to remove cancer that has spread to other places, such as the lung or abdomen. Check the list of NCI-supported cancer clinical trials that are now accepting patients with placental-site gestational trophoblastic tumor and epithelioid trophoblastic tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Recurrent or Resistant Gestational Trophoblastic Neoplasia Treatment of recurrent or resistant gestational trophoblastic tumor may include the following: - Chemotherapy with one or more anticancer drugs for tumors previously treated with surgery. - Combination chemotherapy for tumors previously treated with chemotherapy. - Surgery for tumors that do not respond to chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent gestational trophoblastic tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Gestational Trophoblastic Disease,0000022_1,CancerGov,https://www.cancer.gov/types/gestational-trophoblastic/patient/gtd-treatment-pdq,C1135868,T191,Disorders what research (or clinical trials) is being done for Gestational Trophoblastic Disease ?,0000022_1-8,research,"New types of treatment are being tested in clinical trials. Information about ongoing clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Gestational Trophoblastic Disease,0000022_1,CancerGov,https://www.cancer.gov/types/gestational-trophoblastic/patient/gtd-treatment-pdq,C1135868,T191,Disorders What is (are) Langerhans Cell Histiocytosis ?,0000023_1-1,information,"Key Points - Langerhans cell histiocytosis is a type of cancer that can damage tissue or cause lesions to form in one or more places in the body. - Family history or having a parent who was exposed to certain chemicals may increase the risk of LCH. - The signs and symptoms of LCH depend on where it is in the body. - Skin and nails - Mouth - Bone - Lymph nodes and thymus - Endocrine system - Central nervous system (CNS) - Liver and spleen - Lung - Bone marrow - Tests that examine the organs and body systems where LCH may occur are used to detect (find) and diagnose LCH. - Certain factors affect prognosis (chance of recovery) and treatment options. Langerhans cell histiocytosis is a type of cancer that can damage tissue or cause lesions to form in one or more places in the body. Langerhans cell histiocytosis (LCH) is a rare cancer that begins in LCH cells (a type of dendritic cell which fights infection). Sometimes there are mutations (changes) in LCH cells as they form. These include mutations of the BRAF gene. These changes may make the LCH cells grow and multiply quickly. This causes LCH cells to build up in certain parts of the body, where they can damage tissue or form lesions. LCH is not a disease of the Langerhans cells that normally occur in the skin. LCH may occur at any age, but is most common in young children. Treatment of LCH in children is different from treatment of LCH in adults. The treatments for LCH in children and adults are described in separate sections of this summary. Check the list of NCI-supported cancer clinical trials that are now accepting patients with childhood Langerhans cell histiocytosis. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website.",Langerhans Cell Histiocytosis,0000023_1,CancerGov,https://www.cancer.gov/types/langerhans/patient/langerhans-treatment-pdq,C0019621,T191,Disorders Who is at risk for Langerhans Cell Histiocytosis? ?,0000023_1-2,susceptibility,"Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for LCH include the following: - Having a parent who was exposed to certain chemicals such as benzene. - Having a parent who was exposed to metal, granite, or wood dust in the workplace. - A family history of cancer, including LCH. - Having infections as a newborn. - Having a personal history or family history of thyroid disease. - Smoking, especially in young adults. - Being Hispanic.",Langerhans Cell Histiocytosis,0000023_1,CancerGov,https://www.cancer.gov/types/langerhans/patient/langerhans-treatment-pdq,C0019621,T191,Disorders What are the symptoms of Langerhans Cell Histiocytosis ?,0000023_1-3,symptoms,"These and other signs and symptoms may be caused by LCH or by other conditions. Check with your doctor if you or your child have any of the following: Skin and nails LCH in infants may affect the skin only. In some cases, skin-only LCH may get worse over weeks or months and become a form called high-risk multisystem LCH. In infants, signs or symptoms of LCH that affects the skin may include: - Flaking of the scalp that may look like cradle cap. - Raised, brown or purple skin rash anywhere on the body. In children and adults, signs or symptoms of LCH that affects the skin and nails may include: - Flaking of the scalp that may look like dandruff. - Raised, red or brown, crusted rash in the groin area, abdomen, back, or chest, that may be itchy. - Bumps or ulcers on the scalp. - Ulcers behind the ears, under the breasts, or in the groin area. - Fingernails that fall off or have discolored grooves that run the length of the nail. Mouth Signs or symptoms of LCH that affects the mouth may include: - Swollen gums. - Sores on the roof of the mouth, inside the cheeks, or on the tongue or lips. - Teeth that become uneven. - Tooth loss. Bone Signs or symptoms of LCH that affects the bone may include: - Swelling or a lump over a bone, such as the skull, ribs, spine, thigh bone, upper arm bone, elbow, eye socket, or bones around the ear. - Pain where there is swelling or a lump over a bone. Children with LCH lesions in bones around the ears or eyes have a high risk for diabetes insipidus and other central nervous system disease. Lymph nodes and thymus Signs or symptoms of LCH that affects the lymph nodes or thymus may include: - Swollen lymph nodes. - Trouble breathing. - Superior vena cava syndrome. This can cause coughing, trouble breathing, and swelling of the face, neck, and upper arms. Endocrine system Signs or symptoms of LCH that affects the pituitary gland may include: - Diabetes insipidus. This can cause a strong thirst and frequent urination. - Slow growth. - Early or late puberty. - Being very overweight. Signs or symptoms of LCH that affects the thyroid may include: - Swollen thyroid gland. - Hypothyroidism. This can cause tiredness, lack of energy, being sensitive to cold, constipation, dry skin, thinning hair, memory problems, trouble concentrating, and depression. In infants, this can also cause a loss of appetite and choking on food. In children and adolescents, this can also cause behavior problems, weight gain, slow growth, and late puberty. - Trouble breathing. Central nervous system (CNS) Signs or symptoms of LCH that affects the CNS (brain and spinal cord) may include: - Loss of balance, uncoordinated body movements, and trouble walking. - Trouble speaking. - Trouble seeing. - Headaches. - Changes in behavior or personality. - Memory problems. These signs and symptoms may be caused by lesions in the CNS or by CNS neurodegenerative syndrome. Liver and spleen Signs or symptoms of LCH that affects the liver or spleen may include: - Swelling in the abdomen caused by a buildup of extra fluid. - Trouble breathing. - Yellowing of the skin and whites of the eyes. - Itching. - Easy bruising or bleeding. - Feeling very tired. Lung Signs or symptoms of LCH that affects the lung may include: - Collapsed lung. This condition can cause chest pain or tightness, trouble breathing, feeling tired, and a bluish color to the skin. - Trouble breathing, especially in adults who smoke. - Dry cough. - Chest pain. Bone marrow Signs or symptoms of LCH that affects the bone marrow may include: - Easy bruising or bleeding. - Fever. - Frequent infections.",Langerhans Cell Histiocytosis,0000023_1,CancerGov,https://www.cancer.gov/types/langerhans/patient/langerhans-treatment-pdq,C0019621,T191,Disorders How to diagnose Langerhans Cell Histiocytosis ?,0000023_1-4,exams and tests,"The following tests and procedures may be used to detect (find) and diagnose LCH or conditions caused by LCH: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient's health habits and past illnesses and treatments will also be taken. - Neurological exam : A series of questions and tests to check the brain, spinal cord, and nerve function. The exam checks a person's mental status, coordination, and ability to walk normally, and how well the muscles, senses, and reflexes work. This may also be called a neuro exam or a neurologic exam. - Complete blood count (CBC) with differential : A procedure in which a sample of blood is drawn and checked for the following: - The amount of hemoglobin (the protein that carries oxygen) in the red blood cells. - The portion of the blood sample made up of red blood cells. - The number and type of white blood cells. - The number of red blood cells and platelets. - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the body by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. - Liver function test : A blood test to measure the blood levels of certain substances released by the liver. A high or low level of these substances can be a sign of disease in the liver. - BRAF gene testing : A laboratory test in which a sample of blood or tissue is tested for mutations of the BRAF gene. - Urinalysis : A test to check the color of urine and its contents, such as sugar, protein, red blood cells, and white blood cells. - Water deprivation test : A test to check how much urine is made and whether it becomes concentrated when little or no water is given. This test is used to diagnose diabetes insipidus, which may be caused by LCH. - Bone marrow aspiration and biopsy : The removal of bone marrow and a small piece of bone by inserting a hollow needle into the hipbone. A pathologist views the bone marrow and bone under a microscope to look for signs of LCH. The following tests may be done on the tissue that was removed: - Immunohistochemistry : A test that uses antibodies to check for certain antigens in a sample of tissue. The antibody is usually linked to a radioactive substance or a dye that causes the tissue to light up under a microscope. This type of test may be used to tell the difference between different types of cancer. - Flow cytometry : A laboratory test that measures the number of cells in a sample, how many cells are live, and the size of the cells. It also shows the shapes of the cells and whether there are tumor markers on the surface of the cells. The cells are stained with a light-sensitive dye, placed in a fluid, and passed in a stream before a laser or other type of light. The measurements are based on how the light-sensitive dye reacts to the light. - Bone scan : A procedure to check if there are rapidly dividing cells in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones with cancer and is detected by a scanner. - X-ray : An x-ray of the organs and bones inside the body. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. Sometimes a skeletal survey is done. This is a procedure to x-ray all of the bones in the body. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. A substance called gadolinium may be injected into a vein. The gadolinium collects around the LCH cells so that they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI). - PET scan (positron emission tomography scan): A procedure to find tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. - Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later. - Bronchoscopy : A procedure to look inside the trachea and large airways in the lung for abnormal areas. A bronchoscope is inserted through the nose or mouth into the trachea and lungs. A bronchoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer. - Endoscopy : A procedure to look at organs and tissues inside the body to check for abnormal areas in the gastrointestinal tract or lungs. An endoscope is inserted through an incision (cut) in the skin or opening in the body, such as the mouth. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of disease. - Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for LCH cells. To diagnose LCH, a biopsy of bone lesions, skin, lymph nodes, or the liver may be done.",Langerhans Cell Histiocytosis,0000023_1,CancerGov,https://www.cancer.gov/types/langerhans/patient/langerhans-treatment-pdq,C0019621,T191,Disorders What is the outlook for Langerhans Cell Histiocytosis ?,0000023_1-5,outlook,"LCH in organs such as the skin, bones, lymph nodes, or pituitary gland usually gets better with treatment and is called ""low- risk"". LCH in the spleen, liver, or bone marrow is harder to treat and is called ""high-risk"". The prognosis (chance of recovery) and treatment options depend on the following: - Whether there are mutations of the BRAF gene. - How old the patient is when diagnosed with LCH. - How many organs or body systems the cancer affects. - Whether the cancer is found in the liver, spleen, bone marrow, or certain bones in the skull. - How quickly the cancer responds to initial treatment. - Whether the cancer has just been diagnosed or has come back (recurred). In infants up to one year of age, LCH may go away without treatment.",Langerhans Cell Histiocytosis,0000023_1,CancerGov,https://www.cancer.gov/types/langerhans/patient/langerhans-treatment-pdq,C0019621,T191,Disorders What are the stages of Langerhans Cell Histiocytosis ?,0000023_1-6,stages,"Key Points - There is no staging system for Langerhans cell histiocytosis (LCH). - Treatment of LCH is based on where LCH cells are found in the body and how many body systems are affected. There is no staging system for Langerhans cell histiocytosis (LCH). The extent or spread of cancer is usually described as stages. There is no staging system for LCH. Treatment of LCH is based on where LCH cells are found in the body and how many body systems are affected. LCH is described as single-system disease or multisystem disease, depending on how many body systems are affected: - Single-system LCH: LCH is found in one part of an organ or body system (unifocal) or in more than one part of that organ or body system (multifocal). Bone is the most common single place for LCH to be found. - Multisystem LCH: LCH occurs in two or more organs or body systems or may be spread throughout the body. Multisystem LCH is less common than single-system LCH. LCH may affect low-risk organs or high-risk organs: - Low-risk organs include the skin, bone, lungs, lymph nodes, gastrointestinal tract, pituitary gland, and central nervous system (CNS). - High-risk organs include the liver, spleen, and bone marrow.",Langerhans Cell Histiocytosis,0000023_1,CancerGov,https://www.cancer.gov/types/langerhans/patient/langerhans-treatment-pdq,C0019621,T191,Disorders What are the treatments for Langerhans Cell Histiocytosis ?,0000023_1-7,treatment,"Key Points - There are different types of treatment for patients with Langerhans cell histiocytosis (LCH). - Children with LCH should have their treatment planned by a team of health care providers who are experts in treating childhood cancer. - Some cancer treatments cause side effects months or years after treatment for childhood cancer has ended. - Nine types of standard treatment are used: - Chemotherapy - Surgery - Radiation therapy - Photodynamic therapy - Biologic therapy - Targeted therapy - Other drug therapy - Stem cell transplant - Observation - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their treatment. - When treatment of LCH stops, new lesions may appear or old lesions may come back. - Follow-up tests may be needed. There are different types of treatment for patients with Langerhans cell histiocytosis (LCH). Different types of treatments are available for patients with LCH. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Whenever possible, patients should take part in a clinical trial in order to receive new types of treatment for LCH. Some clinical trials are open only to patients who have not started treatment. Clinical trials are taking place in many parts of the country. Information about ongoing clinical trials is available from the NCI website. Choosing the most appropriate treatment is a decision that ideally involves the patient, family, and health care team. Children with LCH should have their treatment planned by a team of health care providers who are experts in treating childhood cancer. Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other pediatric healthcare providers who are experts in treating children with LCH and who specialize in certain areas of medicine. These may include the following specialists: - Pediatrician. - Primary care physician. - Pediatric surgeon. - Pediatric hematologist. - Radiation oncologist. - Neurologist. - Endocrinologist. - Pediatric nurse specialist. - Rehabilitation specialist. - Psychologist. - Social worker. Some cancer treatments cause side effects months or years after treatment for childhood cancer has ended. Side effects from cancer treatment that begin during or after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include the following: - Slow growth and development. - Hearing loss. - Bone, tooth, liver, and lung problems. - Changes in mood, feeling, learning, thinking, or memory. - Second cancers, such as leukemia, retinoblastoma, Ewing sarcoma, brain or liver cancer. Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information.) Many patients with multisystem LCH have late effects caused by treatment or by the disease itself. These patients often have long-term health problems that affect their quality of life. Nine types of standard treatment are used: Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly onto the skin or into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Chemotherapy agents given by injection or by mouth are used to treat LCH. Chemotherapy agents include vinblastine, cytarabine, cladribine, and methotrexate. Nitrogen mustard is a drug that is put directly on the skin to treat small LCH lesions. Surgery Surgery may be used to remove LCH lesions and a small amount of nearby healthy tissue. Curettage is a type of surgery that uses a curette (a sharp, spoon-shaped tool) to scrape LCH cells from bone. When there is severe liver or lung damage, the entire organ may be removed and replaced with a healthy liver or lung from a donor. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. External radiation therapy uses a machine outside the body to send radiation toward the cancer. In LCH, a special lamp may be used to send ultraviolet B (UVB) radiation toward LCH skin lesions. Photodynamic therapy Photodynamic therapy is a cancer treatment that uses a drug and a certain type of laser light to kill cancer cells. A drug that is not active until it is exposed to light is injected into a vein. The drug collects more in cancer cells than in normal cells. For LCH, laser light is aimed at the skin and the drug becomes active and kills the cancer cells. Photodynamic therapy causes little damage to healthy tissue. Patients who have photodynamic therapy should not spend too much time in the sun. In one type of photodynamic therapy, called psoralen and ultraviolet A (PUVA) therapy, the patient receives a drug called psoralen and then ultraviolet A radiation is directed to the skin. Biologic therapy Biologic therapy is a treatment that uses the patients immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the bodys natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy. Interferon is a type of biologic therapy used to treat LCH of the skin. Immunomodulators are also a type of biologic therapy. Thalidomide is an immunomodulator used to treat LCH. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to find and attack LCH cells without harming normal cells. Imatinib mesylate is a type of targeted therapy called a tyrosine kinase inhibitor. It stops blood stem cells from turning into dendritic cells that may become cancer cells. Other types of kinase inhibitors that affect cells with mutations (changes) in the BRAF gene, such as dabrafenib and vemurafenib, are being studied in clinical trials for LCH. A family of genes, called ras genes, may cause cancer when they are mutated. Ras genes make proteins that are involved in cell signaling pathways, cell growth, and cell death. Ras pathway inhibitors are a type of targeted therapy being studied in clinical trials. They block the actions of a mutated ras gene or its protein and may stop the growth of cancer. Other drug therapy Other drugs used to treat LCH include the following: - Steroid therapy, such as prednisone, is used to treat LCH lesions. - Bisphosphonate therapy (such as pamidronate, zoledronate, or alendronate) is used to treat LCH lesions of the bone and to lessen bone pain. - Nonsteroidal anti-inflammatory drugs (NSAIDs) are drugs (such as aspirin and ibuprofen) that are commonly used to decrease fever, swelling, pain, and redness. Sometimes an NSAID called indomethacin is used to treat LCH. - Retinoids, such as isotretinoin, are drugs related to vitamin A that can slow the growth of LCH cells in the skin. The retinoids are taken by mouth. Stem cell transplant Stem cell transplant is a method of giving chemotherapy and replacing blood-forming cells destroyed by the LCH treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. Observation Observation is closely monitoring a patient's condition without giving any treatment until signs or symptoms appear or change. New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options for Childhood LCH and the Treatment Options for Adult LCH sections for links to current treatment clinical trials. These have been retrieved from NCI's clinical trials database. When treatment of LCH stops, new lesions may appear or old lesions may come back. Many patients with LCH get better with treatment. However, when treatment stops, new lesions may appear or old lesions may come back. This is called reactivation (recurrence) and may occur within one year after stopping treatment. Patients with multisystem disease are more likely to have a reactivation. More common sites of reactivation are bone, ears, or skin. Diabetes insipidus also may develop. Less common sites of reactivation include lymph nodes, bone marrow, spleen, liver, or lung. Some patients may have more than one reactivation over a number of years. Follow-up tests may be needed. Some of the tests that were done to diagnose LCH may be repeated. This is to see how well the treatment is working and if there are any new lesions. These tests may include: - Physical exam. - Neurological exam. - Ultrasound exam. - MRI. - CT scan. - PET scan. Other tests that may be needed include: - Brain stem auditory evoked response (BAER) test: A test that measures the brain's response to clicking sounds or certain tones. - Pulmonary function test (PFT): A test to see how well the lungs are working. It measures how much air the lungs can hold and how quickly air moves into and out of the lungs. It also measures how much oxygen is used and how much carbon dioxide is given off during breathing. This is also called a lung function test. - Chest x-ray: An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for LCH in Children Treatment of Low-Risk Disease in Children Skin Lesions Treatment of childhood Langerhans cell histiocytosis (LCH) skin lesions may include the following: - Observation. When severe rashes, pain, ulceration, or bleeding occur, treatment may include the following: - Steroid therapy. - Chemotherapy, given by mouth. - Nitrogen mustard applied to the skin. - Photodynamic therapy with psoralen and ultraviolet A (PUVA) therapy. - UVB radiation therapy. Lesions in Bones or Other Low-Risk Organs Treatment of childhood LCH bone lesions in the front, sides, or back of the skull, or in any other single bone may include the following: - Surgery (curettage) with or without steroid therapy. - Low-dose radiation therapy for lesions that affect nearby organs. Treatment of childhood LCH lesions in bones around the ears or eyes is done to lower the risk of diabetes insipidus and other long-term problems. Treatment may include: - Chemotherapy and steroid therapy. - Surgery (curettage). Treatment of childhood LCH lesions of the spine or thigh bone lesions may include: - Observation. - Low-dose radiation therapy. - Chemotherapy, for lesions that spread from the spine into nearby tissue. - Surgery to strengthen the weakened bone by bracing or fusing the bones together. Treatment of two or more bone lesions may include: - Chemotherapy and steroid therapy. Treatment of two or more bone lesions combined with childhood LCH skin lesions, lymph node lesions, or diabetes insipidus may include: - Chemotherapy with or without steroid therapy. - Bisphosphonate therapy. Treatment of High-Risk Disease in Children Treatment of childhood LCH multisystem disease lesions in the spleen, liver, or bone marrow (with or without skin, bone, lymph node, lung, or pituitary gland lesions) may include: - Chemotherapy and steroid therapy. Higher doses of combination chemotherapy and steroid therapy may be given to patients whose tumors do not respond to initial chemotherapy. - A liver transplant for patients with severe liver damage. Treatment of childhood LCH central nervous system (CNS) lesions may include: - Chemotherapy with or without steroid therapy. - Steroid therapy. Treatment of LCH CNS neurodegenerative syndrome may include: - Retinoid therapy. - Chemotherapy. Treatment Options for Recurrent, Refractory, and Progressive Childhood LCH in Children Recurrent LCH is cancer that cannot be detected for some time after treatment and then comes back. Treatment of recurrent childhood LCH in the skin, bone, lymph nodes, gastrointestinal tract, pituitary gland, or central nervous system (low-risk organs) may include: - Chemotherapy with or without steroid therapy. - Bisphosphonate therapy. - Nonsteroidal anti-inflammatory drug (NSAID) therapy with indomethacin. - A clinical trial of a targeted therapy. Refractory LCH is cancer that does not get better with treatment. Treatment of refractory childhood LCH in high-risk organs and in multisystem low-risk organs may include high-dose chemotherapy. Treatment of childhood LCH in multisystem high-risk organs that did not respond to chemotherapy may include stem cell transplant. Progressive LCH is cancer that continues to grow during treatment. Treatment of progressive childhood LCH in patients with multisystem disease may include anticancer drugs that have not been given to the patient before. Treatment Options for LCH in Adults Treatment Options for LCH of the Lung in Adults Treatment for LCH of the lung in adults may include: - Quitting smoking for all patients who smoke. Lung damage will get worse over time in patients who do not quit smoking. In patients who quit smoking, lung damage may get better or it may get worse over time. - Chemotherapy. - Lung transplant for patients with severe lung damage. Sometimes LCH of the lung will go away or not get worse even if it's not treated. Treatment Options for LCH of the Bone in Adults Treatment for LCH that affects only the bone in adults may include: - Surgery with or without steroid therapy. - Chemotherapy with or without low-dose radiation therapy. - Radiation therapy. - Bisphosphonate therapy, for severe bone pain. Treatment Options for LCH of the Skin in Adults Treatment for LCH that affects only the skin in adults may include: - Surgery. - Steroid or other drug therapy applied or injected into the skin. - Photodynamic therapy with psoralen and ultraviolet A (PUVA) radiation. - UVB radiation therapy. - Chemotherapy or biologic therapy given by mouth, such as methotrexate, thalidomide, or interferon. - Retinoid therapy may be used if the skin lesions do not get better with other treatment. Treatment for LCH that affects the skin and other body systems in adults may include: - Chemotherapy. Treatment Options for Single-System and Multisystem LCH in Adults Treatment of single-system and multisystem disease in adults may include: - Chemotherapy with or without a drug given to weaken the immune system. - Bisphosphonate therapy, for severe bone pain. - A clinical trial of a targeted therapy.",Langerhans Cell Histiocytosis,0000023_1,CancerGov,https://www.cancer.gov/types/langerhans/patient/langerhans-treatment-pdq,C0019621,T191,Disorders what research (or clinical trials) is being done for Langerhans Cell Histiocytosis ?,0000023_1-8,research,"New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options for Childhood LCH and the Treatment Options for Adult LCH sections for links to current treatment clinical trials. These have been retrieved from NCI's clinical trials database.",Langerhans Cell Histiocytosis,0000023_1,CancerGov,https://www.cancer.gov/types/langerhans/patient/langerhans-treatment-pdq,C0019621,T191,Disorders What is (are) Hypopharyngeal Cancer ?,0000024_1-1,information,"Key Points - Hypopharyngeal cancer is a disease in which malignant (cancer) cells form in the tissues of the hypopharynx. - Use of tobacco products and heavy drinking can affect the risk of developing hypopharyngeal cancer. - Signs and symptoms of hypopharyngeal cancer include a sore throat and ear pain. - Tests that examine the throat and neck are used to help detect (find) and diagnose hypopharyngeal cancer. - Certain factors affect prognosis (chance of recovery) and treatment options. Hypopharyngeal cancer is a disease in which malignant (cancer) cells form in the tissues of the hypopharynx. The hypopharynx is the bottom part of the pharynx (throat). The pharynx is a hollow tube about 5 inches long that starts behind the nose, goes down the neck, and ends at the top of the trachea (windpipe) and esophagus (the tube that goes from the throat to the stomach). Air and food pass through the pharynx on the way to the trachea or the esophagus. Most hypopharyngeal cancers form in squamous cells, the thin, flat cells lining the inside of the hypopharynx. The hypopharynx has 3 different areas. Cancer may be found in 1 or more of these areas. Hypopharyngeal cancer is a type of head and neck cancer.",Hypopharyngeal Cancer,0000024_1,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/hypopharyngeal-treatment-pdq,C0153398,T191,Disorders Who is at risk for Hypopharyngeal Cancer? ?,0000024_1-2,susceptibility,Use of tobacco products and heavy drinking can affect the risk of developing hypopharyngeal cancer. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors include the following: - Smoking tobacco. - Chewing tobacco. - Heavy alcohol use. - Eating a diet without enough nutrients. - Having Plummer-Vinson syndrome.,Hypopharyngeal Cancer,0000024_1,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/hypopharyngeal-treatment-pdq,C0153398,T191,Disorders What are the symptoms of Hypopharyngeal Cancer ?,0000024_1-3,symptoms,Signs and symptoms of hypopharyngeal cancer include a sore throat and ear pain. These and other signs and symptoms may be caused by hypopharyngeal cancer or by other conditions. Check with your doctor if you have any of the following: - A sore throat that does not go away. - Ear pain. - A lump in the neck. - Painful or difficult swallowing. - A change in voice.,Hypopharyngeal Cancer,0000024_1,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/hypopharyngeal-treatment-pdq,C0153398,T191,Disorders How to diagnose Hypopharyngeal Cancer ?,0000024_1-4,exams and tests,"Tests that examine the throat and neck are used to help detect (find) and diagnose hypopharyngeal cancer. The following tests and procedures may be used: - Physical exam of the throat: An exam in which the doctor feels for swollen lymph nodes in the neck and looks down the throat with a small, long-handled mirror to check for abnormal areas. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. A PET scan and CT scan may be done at the same time. This is called a PET-CT. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Bone scan : A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones and is detected by a scanner. - Barium esophagogram: An x-ray of the esophagus. The patient drinks a liquid that contains barium (a silver-white metallic compound). The liquid coats the esophagus and x-rays are taken. - Endoscopy : A procedure used to look at areas in the throat that cannot be seen with a mirror during the physical exam of the throat. An endoscope (a thin, lighted tube) is inserted through the nose or mouth to check the throat for anything that seems unusual. Tissue samples may be taken for biopsy. - Esophagoscopy : A procedure to look inside the esophagus to check for abnormal areas. An esophagoscope (a thin, lighted tube) is inserted through the mouth or nose and down the throat into the esophagus. Tissue samples may be taken for biopsy. - Bronchoscopy : A procedure to look inside the trachea and large airways in the lung for abnormal areas. A bronchoscope (a thin, lighted tube) is inserted through the nose or mouth into the trachea and lungs. Tissue samples may be taken for biopsy. - Biopsy: The removal of cells or tissues so they can be viewed under a microscope to check for signs of cancer.",Hypopharyngeal Cancer,0000024_1,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/hypopharyngeal-treatment-pdq,C0153398,T191,Disorders What is the outlook for Hypopharyngeal Cancer ?,0000024_1-5,outlook,"Certain factors affect prognosis (chance of recovery) and treatment options. Prognosis (chance of recovery) depends on the following: - The stage of the cancer (whether it affects part of the hypopharynx, involves the whole hypopharynx, or has spread to other places in the body). Hypopharyngeal cancer is usually detected in later stages because early signs and symptoms rarely occur. - The patient's age, gender, and general health. - The location of the cancer. - Whether the patient smokes during radiation therapy. Treatment options depend on the following: - The stage of the cancer. - Keeping the patient's ability to talk, eat, and breathe as normal as possible. - The patient's general health. Patients who have had hypopharyngeal cancer are at an increased risk of developing a second cancer in the head or neck. Frequent and careful follow-up is important.",Hypopharyngeal Cancer,0000024_1,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/hypopharyngeal-treatment-pdq,C0153398,T191,Disorders What are the stages of Hypopharyngeal Cancer ?,0000024_1-6,stages,"Key Points - After hypopharyngeal cancer has been diagnosed, tests are done to find out if cancer cells have spread within the hypopharynx or to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - The following stages are used for hypopharyngeal cancer: - Stage 0 (Carcinoma in Situ) - Stage I - Stage II - Stage III - Stage IV After hypopharyngeal cancer has been diagnosed, tests are done to find out if cancer cells have spread within the hypopharynx or to other parts of the body. The process used to find out if cancer has spread within the hypopharynx or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage of the disease in order to plan treatment. The results of some of the tests used to diagnose hypopharyngeal cancer are often also used to stage the disease. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if hypopharyngeal cancer spreads to the lung, the cancer cells in the lung are actually hypopharyngeal cancer cells. The disease is metastatic hypopharyngeal cancer, not lung cancer. The following stages are used for hypopharyngeal cancer: Stage 0 (Carcinoma in Situ) In stage 0, abnormal cells are found in the lining of the hypopharynx. These abnormal cells may become cancer and spread into nearby normal tissue. Stage 0 is also called carcinoma in situ. Stage I In stage I, cancer has formed in one area of the hypopharynx only and/or the tumor is 2 centimeters or smaller. Stage II In stage II, the tumor is either: - larger than 2 centimeters but not larger than 4 centimeters and has not spread to the larynx (voice box); or - found in more than one area of the hypopharynx or in nearby tissues. Stage III In stage III, the tumor: - is larger than 4 centimeters or has spread to the larynx (voice box) or esophagus. Cancer may have spread to one lymph node on the same side of the neck as the tumor and the lymph node is 3 centimeters or smaller; or - has spread to one lymph node on the same side of the neck as the tumor and the lymph node is 3 centimeters or smaller and cancer is found: - in one area of the hypopharynx and/or is 2 centimeters or smaller; or - in more than one area of the hypopharynx or in nearby tissues, or is larger than 2 centimeters but not larger than 4 centimeters and has not spread to the larynx. Stage IV Stage IV is divided into stage IVA, IVB, and IVC as follows: - In stage IVA, cancer: - has spread to cartilage around the thyroid or trachea, the bone under the tongue, the thyroid, or nearby soft tissue. Cancer may have spread to one lymph node on the same side of the neck as the tumor and the lymph node is 3 centimeters or smaller; or - has spread to one lymph node on the same side of the neck as the tumor (the lymph node is larger than 3 centimeters but not larger than 6 centimeters) or to lymph nodes anywhere in the neck (affected lymph nodes are 6 centimeters or smaller), and one of the following is true: - cancer is found in one area of the hypopharynx and/or is 2 centimeters or smaller; or - cancer is found in more than one area of the hypopharynx or in nearby tissues, or is larger than 2 centimeters but not larger than 4 centimeters and has not spread to the larynx (voice box); or - cancer has spread to the larynx or esophagus and is more than 4 centimeters; or - cancer has spread to cartilage around the thyroid or trachea, the bone under the tongue, the thyroid, or nearby soft tissue. - In stage IVB, the tumor: - has spread to muscles around the upper part of the spinal column, the carotid artery, or the lining of the chest cavity and may have spread to lymph nodes which can be any size; or - may be any size and has spread to one or more lymph nodes that are larger than 6 centimeters. - In stage IVC, the tumor may be any size and has spread beyond the hypopharynx to other parts of the body.",Hypopharyngeal Cancer,0000024_1,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/hypopharyngeal-treatment-pdq,C0153398,T191,Disorders What are the treatments for Hypopharyngeal Cancer ?,0000024_1-7,treatment,"Key Points - There are different types of treatment for patients with hypopharyngeal cancer. - Three types of standard treatment are used: - Surgery - Radiation therapy - Chemotherapy - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with hypopharyngeal cancer. Different types of treatment are available for patients with hypopharyngeal cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Three types of standard treatment are used: Surgery Surgery (removing the cancer in an operation) is a common treatment for all stages of hypopharyngeal cancer. The following surgical procedures may be used: - Laryngopharyngectomy: Surgery to remove the larynx (voice box) and part of the pharynx (throat). - Partial laryngopharyngectomy: Surgery to remove part of the larynx and part of the pharynx. A partial laryngopharyngectomy prevents loss of the voice. - Neck dissection: Surgery to remove lymph nodes and other tissues in the neck. Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External radiation therapy is used to treat hypopharyngeal cancer. Radiation therapy may work better in patients who have stopped smoking before beginning treatment. External radiation therapy to the thyroid or the pituitary gland may change the way the thyroid gland works. A blood test to check the thyroid hormone level in the body may be done before and after therapy to make sure the thyroid gland is working properly. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. Chemotherapy may be used to shrink the tumor before surgery or radiation therapy. This is called neoadjuvant chemotherapy. See Drugs Approved for Head and Neck Cancer for more information. (Hypopharyngeal cancer is a type of head and neck cancer.) New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. For hypopharyngeal cancer, follow-up to check for recurrence should include careful head and neck exams once a month in the first year after treatment ends, every 2 months in the second year, every 3 months in the third year, and every 6 months thereafter. Treatment Options by Stage Stage I Hypopharyngeal Cancer Treatment of stage I hypopharyngeal cancer may include the following: - Laryngopharyngectomy and neck dissection with or without high-dose radiation therapy to the lymph nodes of the neck. - Partial laryngopharyngectomy with or without high-dose radiation therapy to the lymph nodes on both sides of the neck. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I hypopharyngeal cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage II Hypopharyngeal Cancer Treatment of stage II hypopharyngeal cancer may include the following: - Laryngopharyngectomy and neck dissection. High-dose radiation therapy to the lymph nodes of the neck may be given before or after surgery. - Partial laryngopharyngectomy. High-dose radiation therapy to the lymph nodes of the neck may be given before or after surgery. - Chemotherapy given during or after radiation therapy or after surgery. - A clinical trial of chemotherapy followed by radiation therapy or surgery. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage II hypopharyngeal cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage III Hypopharyngeal Cancer Treatment of stage III hypopharyngeal cancer may include the following: - Radiation therapy before or after surgery. - Chemotherapy given during or after radiation therapy or after surgery. - A clinical trial of chemotherapy followed by surgery and/or radiation therapy. - A clinical trial of chemotherapy given at the same time as radiation therapy. - A clinical trial of surgery followed by chemotherapy given at the same time as radiation therapy. Treatment and follow-up of stage III hypopharyngeal cancer is complex and is ideally overseen by a team of specialists with experience and expertise in treating this type of cancer. If all or part of the hypopharynx is removed, the patient may need plastic surgery and other special help with breathing, eating, and talking. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III hypopharyngeal cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage IV Hypopharyngeal Cancer Treatment of stage IV hypopharyngeal cancer that can be treated with surgery may include the following: - Radiation therapy before or after surgery. - A clinical trial of chemotherapy followed by surgery and/or radiation therapy. - A clinical trial of surgery followed by chemotherapy given at the same time as radiation therapy. Surgical treatment and follow-up of stage IV hypopharyngeal cancer is complex and is ideally overseen by a team of specialists with experience and expertise in treating this type of cancer. If all or part of the hypopharynx is removed, the patient may need plastic surgery and other special help with breathing, eating, and talking. Treatment of stage IV hypopharyngeal cancer that cannot be treated with surgery may include the following: - Radiation therapy. - Chemotherapy given at the same time as radiation therapy. - A clinical trial of radiation therapy with chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage IV hypopharyngeal cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Hypopharyngeal Cancer,0000024_1,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/hypopharyngeal-treatment-pdq,C0153398,T191,Disorders what research (or clinical trials) is being done for Hypopharyngeal Cancer ?,0000024_1-8,research,"New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Hypopharyngeal Cancer,0000024_1,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/hypopharyngeal-treatment-pdq,C0153398,T191,Disorders What is (are) Oral Cavity and Oropharyngeal Cancer ?,0000024_10-1,information,"Key Points - Oral cavity and oropharyngeal cancer are diseases in which malignant (cancer) cells form in the mouth and throat. - The number of new cases of oral cavity and oropharyngeal cancer and the number of deaths from oral cavity and oropharyngeal cancer varies by race and gender. - Different factors increase or decrease the risk of oral cavity and oropharyngeal cancer. Oral cavity and oropharyngeal cancer are diseases in which malignant (cancer) cells form in the mouth and throat. Oral cavity cancer forms in any of these tissues of the oral cavity: - The front two thirds of the tongue. - The gingiva (gums). - The buccal mucosa (the lining of the inside of the cheeks). - The floor (bottom) of the mouth under the tongue. - The hard palate (the front of the roof of the mouth). - The retromolar trigone (the small area behind the wisdom teeth). Oropharyngeal cancer forms in any of these tissues of the oropharynx: - The middle part of the pharynx (throat) behind the mouth. - The back one third of the tongue. - The soft palate (the back of the roof of the mouth). - The side and back walls of the throat. - The tonsils. Most oral cavity and oropharyngeal cancers start in squamous cells, the thin, flat cells that line the lips, oral cavity, and oropharynx. Cancer that forms in squamous cells is called squamous cell carcinoma. See the following PDQ summaries for more information about the screening, diagnosis, and treatment of oral cavity and oropharyngeal cancer: - Oral Cavity and Oropharyngeal Cancer Prevention - Lip and Oral Cavity Cancer Treatment - Oropharyngeal Cancer Treatment The number of new cases of oral cavity and oropharyngeal cancer and the number of deaths from oral cavity and oropharyngeal cancer varies by race and gender. Over the past ten years, the number of new cases and deaths from oral cavity and oropharyngeal cancer slightly increased in white men and women. The number slightly decreased among black men and women. Oral cavity and oropharyngeal cancer is more common in men than in women. Although oral cavity and oropharyngeal cancer may occur in adults of any age, it occurs most often in those aged 55 to 64 years. France, Brazil, and parts of Asia have much higher rates of oral cavity and oropharyngeal cancer than most other countries. The number of new cases of oropharyngeal cancer caused by certain types of human papillomavirus (HPV) infection has increased. One kind of HPV, called HPV 16, is often passed from one person to another during sexual activity.",Oral Cavity and Oropharyngeal Cancer,0000024_10,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/oral-screening-pdq,C0153382,T191,Disorders Who is at risk for Oral Cavity and Oropharyngeal Cancer? ?,0000024_10-2,susceptibility,"The number of new cases of oral cavity and oropharyngeal cancer and the number of deaths from oral cavity and oropharyngeal cancer varies by race and gender. Over the past ten years, the number of new cases and deaths from oral cavity and oropharyngeal cancer slightly increased in white men and women. The number slightly decreased among black men and women. Oral cavity and oropharyngeal cancer is more common in men than in women. Although oral cavity and oropharyngeal cancer may occur in adults of any age, it occurs most often in those aged 55 to 64 years. France, Brazil, and parts of Asia have much higher rates of oral cavity and oropharyngeal cancer than most other countries. The number of new cases of oropharyngeal cancer caused by certain types of human papillomavirus (HPV) infection has increased. One kind of HPV, called HPV 16, is often passed from one person to another during sexual activity.",Oral Cavity and Oropharyngeal Cancer,0000024_10,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/oral-screening-pdq,C0153382,T191,Disorders Who is at risk for Oral Cavity and Oropharyngeal Cancer? ?,0000024_10-3,susceptibility,"Different factors increase or decrease the risk of oral cavity and oropharyngeal cancer. Anything that increases your chance of getting a disease is called a risk factor. Anything that decreases your chance of getting a disease is called a protective factor. For information about risk factors and protective factors for oral cavity and oropharyngeal cancer, see the PDQ summary on Oral Cavity and Oropharyngeal Cancer Prevention.",Oral Cavity and Oropharyngeal Cancer,0000024_10,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/oral-screening-pdq,C0153382,T191,Disorders What is (are) Laryngeal Cancer ?,0000024_2-1,information,"Key Points - Laryngeal cancer is a disease in which malignant (cancer) cells form in the tissues of the larynx. - Use of tobacco products and drinking too much alcohol can affect the risk of laryngeal cancer. - Signs and symptoms of laryngeal cancer include a sore throat and ear pain. - Tests that examine the throat and neck are used to help detect (find), diagnose, and stage laryngeal cancer. - Certain factors affect prognosis (chance of recovery) and treatment options. Laryngeal cancer is a disease in which malignant (cancer) cells form in the tissues of the larynx. The larynx is a part of the throat, between the base of the tongue and the trachea. The larynx contains the vocal cords, which vibrate and make sound when air is directed against them. The sound echoes through the pharynx, mouth, and nose to make a person's voice. There are three main parts of the larynx: - Supraglottis: The upper part of the larynx above the vocal cords, including the epiglottis. - Glottis: The middle part of the larynx where the vocal cords are located. - Subglottis: The lower part of the larynx between the vocal cords and the trachea (windpipe). Most laryngeal cancers form in squamous cells, the thin, flat cells lining the inside of the larynx. Laryngeal cancer is a type of head and neck cancer.",Laryngeal Cancer,0000024_2,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/laryngeal-treatment-pdq,C0595989,T191,Disorders Who is at risk for Laryngeal Cancer? ?,0000024_2-2,susceptibility,Use of tobacco products and drinking too much alcohol can affect the risk of laryngeal cancer.Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk.,Laryngeal Cancer,0000024_2,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/laryngeal-treatment-pdq,C0595989,T191,Disorders What are the symptoms of Laryngeal Cancer ?,0000024_2-3,symptoms,Signs and symptoms of laryngeal cancer include a sore throat and ear pain. These and other signs and symptoms may be caused by laryngeal cancer or by other conditions. Check with your doctor if you have any of the following: - A sore throat or cough that does not go away. - Trouble or pain when swallowing. - Ear pain. - A lump in the neck or throat. - A change or hoarseness in the voice.,Laryngeal Cancer,0000024_2,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/laryngeal-treatment-pdq,C0595989,T191,Disorders How to diagnose Laryngeal Cancer ?,0000024_2-4,exams and tests,"Tests that examine the throat and neck are used to help detect (find), diagnose, and stage laryngeal cancer.The following tests and procedures may be used: - Physical exam of the throat and neck: An exam to check the throat and neck for abnormal areas. The doctor will feel the inside of the mouth with a gloved finger and examine the mouth and throat with a small long-handled mirror and light. This will include checking the insides of the cheeks and lips; the gums; the back, roof, and floor of the mouth; the top, bottom, and sides of the tongue; and the throat. The neck will be felt for swollen lymph nodes. A history of the patients health habits and past illnesses and medical treatments will also be taken. - Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. The sample of tissue may be removed during one of the following procedures: - Laryngoscopy : A procedure to look at the larynx (voice box) for abnormal areas. A mirror or a laryngoscope (a thin, tube-like instrument with a light and a lens for viewing) is inserted through the mouth to see the larynx. A special tool on the laryngoscope may be used to remove samples of tissue. - Endoscopy : A procedure to look at organs and tissues inside the body, such as the throat, esophagus, and trachea to check for abnormal areas. An endoscope (a thin, lighted tube with a light and a lens for viewing) is inserted through an opening in the body, such as the mouth. A special tool on the endoscope may be used to remove samples of tissue. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. - Bone scan : A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones and is detected by a scanner. - Barium swallow : A series of x-rays of the esophagus and stomach. The patient drinks a liquid that contains barium (a silver-white metallic compound). The liquid coats the esophagus and stomach, and x-rays are taken. This procedure is also called an upper GI series.",Laryngeal Cancer,0000024_2,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/laryngeal-treatment-pdq,C0595989,T191,Disorders What is the outlook for Laryngeal Cancer ?,0000024_2-5,outlook,"Certain factors affect prognosis (chance of recovery) and treatment options. Prognosis (chance of recovery) depends on the following: - The stage of the disease. - The location and size of the tumor. - The grade of the tumor. - The patient's age, gender, and general health, including whether the patient is anemic. Treatment options depend on the following: - The stage of the disease. - The location and size of the tumor. - Keeping the patient's ability to talk, eat, and breathe as normal as possible. - Whether the cancer has come back (recurred). Smoking tobacco and drinking alcohol decrease the effectiveness of treatment for laryngeal cancer. Patients with laryngeal cancer who continue to smoke and drink are less likely to be cured and more likely to develop a second tumor. After treatment for laryngeal cancer, frequent and careful follow-up is important.",Laryngeal Cancer,0000024_2,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/laryngeal-treatment-pdq,C0595989,T191,Disorders What are the stages of Laryngeal Cancer ?,0000024_2-6,stages,"Key Points - After laryngeal cancer has been diagnosed, tests are done to find out if cancer cells have spread within the larynx or to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - The following stages are used for laryngeal cancer: - Stage 0 (Carcinoma in Situ) - Stage I - Stage II - Stage III - Stage IV After laryngeal cancer has been diagnosed, tests are done to find out if cancer cells have spread within the larynx or to other parts of the body. The process used to find out if cancer has spread within the larynx or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage of the disease in order to plan treatment. The results of some of the tests used to diagnose laryngeal cancer are often also used to stage the disease. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if laryngeal cancer spreads to the lung, the cancer cells in the lung are actually laryngeal cancer cells. The disease is metastatic laryngeal cancer, not lung cancer. The following stages are used for laryngeal cancer: Stage 0 (Carcinoma in Situ) In stage 0, abnormal cells are found in the lining of the larynx. These abnormal cells may become cancer and spread into nearby normal tissue. Stage 0 is also called carcinoma in situ. Stage I In stage I, cancer has formed. Stage I laryngeal cancer depends on where cancer began in the larynx: - Supraglottis: Cancer is in one area of the supraglottis only and the vocal cords can move normally. - Glottis: Cancer is in one or both vocal cords and the vocal cords can move normally. - Subglottis: Cancer is in the subglottis only. Stage II In stage II, cancer is in the larynx only. Stage II laryngeal cancer depends on where cancer began in the larynx: - Supraglottis: Cancer is in more than one area of the supraglottis or surrounding tissues. - Glottis: Cancer has spread to the supraglottis and/or the subglottis and/or the vocal cords cannot move normally. - Subglottis: Cancer has spread to one or both vocal cords, which may not move normally. Stage III Stage III laryngeal cancer depends on whether cancer has spread from the supraglottis, glottis, or subglottis. In stage III cancer of the supraglottis: - cancer is in the larynx only and the vocal cords cannot move, and/or cancer is in tissues next to the larynx. Cancer may have spread to one lymph node on the same side of the neck as the original tumor and the lymph node is 3 centimeters or smaller; or - cancer is in one area of the supraglottis and in one lymph node on the same side of the neck as the original tumor; the lymph node is 3 centimeters or smaller and the vocal cords can move normally; or - cancer is in more than one area of the supraglottis or surrounding tissues and in one lymph node on the same side of the neck as the original tumor; the lymph node is 3 centimeters or smaller. In stage III cancer of the glottis: - cancer is in the larynx only and the vocal cords cannot move, and/or cancer is in tissues next to the larynx; cancer may have spread to one lymph node on the same side of the neck as the original tumor and the lymph node is 3 centimeters or smaller; or - cancer is in one or both vocal cords and in one lymph node on the same side of the neck as the original tumor; the lymph node is 3 centimeters or smaller and the vocal cords can move normally; or - cancer has spread to the supraglottis and/or the subglottis and/or the vocal cords cannot move normally. Cancer has also spread to one lymph node on the same side of the neck as the original tumor and the lymph node is 3 centimeters or smaller. In stage III cancer of the subglottis: - cancer is in the larynx and the vocal cords cannot move; cancer may have spread to one lymph node on the same side of the neck as the original tumor and the lymph node is 3 centimeters or smaller; or - cancer is in the subglottis and in one lymph node on the same side of the neck as the original tumor; the lymph node is 3 centimeters or smaller; or - cancer has spread to one or both vocal cords, which may not move normally. Cancer has also spread to one lymph node on the same side of the neck as the original tumor and the lymph node is 3 centimeters or smaller. Stage IV Stage IV is divided into stage IVA, stage IVB, and stage IVC. Each substage is the same for cancer in the supraglottis, glottis, or subglottis. - In stage IVA: - cancer has spread through the thyroid cartilage and/or has spread to tissues beyond the larynx such as the neck, trachea, thyroid, or esophagus. Cancer may have spread to one lymph node on the same side of the neck as the original tumor and the lymph node is 3 centimeters or smaller; or - cancer has spread to one lymph node on the same side of the neck as the original tumor and the lymph node is larger than 3 centimeters but not larger than 6 centimeters, or has spread to more than one lymph node anywhere in the neck with none larger than 6 centimeters. Cancer may have spread to tissues beyond the larynx, such as the neck, trachea, thyroid, or esophagus. The vocal cords may not move normally. - In stage IVB: - cancer has spread to the space in front of the spinal column, surrounds the carotid artery, or has spread to parts of the chest. Cancer may have spread to one or more lymph nodes anywhere in the neck and the lymph nodes may be any size; or - cancer has spread to a lymph node that is larger than 6 centimeters and may have spread as far as the space in front of the spinal column, around the carotid artery, or to parts of the chest. The vocal cords may not move normally. - In stage IVC, cancer has spread to other parts of the body, such as the lungs, liver, or bone.",Laryngeal Cancer,0000024_2,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/laryngeal-treatment-pdq,C0595989,T191,Disorders What are the treatments for Laryngeal Cancer ?,0000024_2-7,treatment,"Key Points - There are different types of treatment for patients with laryngeal cancer. - Three types of standard treatment are used: - Radiation therapy - Surgery - Chemotherapy - New types of treatment are being tested in clinical trials. - Chemoprevention - Radiosensitizers - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with laryngeal cancer. Different types of treatment are available for patients with laryngeal cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Three types of standard treatment are used: Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. Radiation therapy may work better in patients who have stopped smoking before beginning treatment. External radiation therapy to the thyroid or the pituitary gland may change the way the thyroid gland works. The doctor may test the thyroid gland before and after therapy to make sure it is working properly. Hyperfractionated radiation therapy and new types of radiation therapy are being studied in the treatment of laryngeal cancer. Surgery Surgery (removing the cancer in an operation) is a common treatment for all stages of laryngeal cancer. The following surgical procedures may be used: - Cordectomy: Surgery to remove the vocal cords only. - Supraglottic laryngectomy: Surgery to remove the supraglottis only. - Hemilaryngectomy: Surgery to remove half of the larynx (voice box). A hemilaryngectomy saves the voice. - Partial laryngectomy: Surgery to remove part of the larynx (voice box). A partial laryngectomy helps keep the patient's ability to talk. - Total laryngectomy: Surgery to remove the whole larynx. During this operation, a hole is made in the front of the neck to allow the patient to breathe. This is called a tracheostomy. - Thyroidectomy: The removal of all or part of the thyroid gland. - Laser surgery: A surgical procedure that uses a laser beam (a narrow beam of intense light) as a knife to make bloodless cuts in tissue or to remove a surface lesion such as a tumor Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. See Drugs Approved for Head and Neck Cancer for more information. (Laryngeal cancer is a type of head and neck cancer.) New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI Web site. Chemoprevention Chemoprevention is the use of drugs, vitamins, or other substances to reduce the risk of developing cancer or to reduce the risk cancer will recur (come back). The drug isotretinoin is being studied to prevent the development of a second cancer in patients who have had cancer of the head or neck. Radiosensitizers Radiosensitizers are drugs that make tumor cells more sensitive to radiation therapy. Combining radiation therapy with radiosensitizers may kill more tumor cells. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options by Stage Stage I Laryngeal Cancer Treatment of stage I laryngeal cancer depends on where cancer is found in the larynx. If cancer is in the supraglottis, treatment may include the following: - Radiation therapy. - Supraglottic laryngectomy. If cancer is in the glottis, treatment may include the following: - Radiation therapy. - Cordectomy. - Partial laryngectomy, hemilaryngectomy, or total laryngectomy. - Laser surgery. If cancer is in the subglottis, treatment may include the following: - Radiation therapy with or without surgery. - Surgery alone. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I laryngeal cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage II Laryngeal Cancer Treatment of stage II laryngeal cancer depends on where cancer is found in the larynx. If cancer is in the supraglottis, treatment may include the following: - Radiation therapy - Supraglottic laryngectomy or total laryngectomy with or without radiation therapy. - A clinical trial of radiation therapy. - A clinical trial of chemoprevention. If cancer is in the glottis, treatment may include the following: - Radiation therapy. - Partial laryngectomy, hemilaryngectomy, or total laryngectomy. - Laser surgery. - A clinical trial of radiation therapy. - A clinical trial of chemoprevention. If cancer is in the subglottis, treatment may include the following: - Radiation therapy with or without surgery. - Surgery alone. - A clinical trial of radiation therapy. - A clinical trial of chemoprevention. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage II laryngeal cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage III Laryngeal Cancer Treatment of stage III laryngeal cancer depends on where cancer is found in the larynx. If cancer is in the supraglottis or glottis, treatment may include the following: - Chemotherapy and radiation therapy given together. - Chemotherapy followed by chemotherapy and radiation therapy given together. Laryngectomy may be done if cancer remains. - Radiation therapy for patients who cannot be treated with chemotherapy and surgery.For tumors that do not respond to radiation, total laryngectomy may be done. - Surgery, which may be followed by radiation therapy. - A clinical trial of radiation therapy. - A clinical trial of chemotherapy, radiosensitizers, or radiation therapy. - A clinical trial of chemoprevention. If cancer is in the subglottis, treatment may include the following: - Laryngectomy plus total thyroidectomy and removal of lymph nodes in the throat, usually followed by radiation therapy. - Radiation therapy with or without surgery. - A clinical trial of chemotherapy, radiosensitizers, or radiation therapy. - A clinical trial of chemoprevention. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III laryngeal cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage IV Laryngeal Cancer Treatment of stage IV laryngeal cancer depends on where cancer is found in the larynx. If cancer is in the supraglottis or glottis, treatment may include the following: - Chemotherapy and radiation therapy given together. - Chemotherapy followed by chemotherapy and radiation therapy given together. Laryngectomy may be done if cancer remains. - Radiation therapy for patients who cannot be treated with chemotherapy and surgery. For tumors that do not respond to radiation, total laryngectomy may be done. - Surgery followed by radiation therapy. Chemotherapy may be given with the radiation therapy. - A clinical trial of radiation therapy. - A clinical trial of chemotherapy, radiosensitizers, or radiation therapy. - A clinical trial of chemoprevention. If cancer is in the subglottis, treatment may include the following: - Laryngectomy plus total thyroidectomy and removal of lymph nodes in the throat, usually with radiation therapy. - Radiation therapy. - A clinical trial of radiation therapy. - A clinical trial of chemotherapy, radiosensitizers, or radiation therapy. - A clinical trial of chemoprevention. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage IV laryngeal cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Laryngeal Cancer,0000024_2,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/laryngeal-treatment-pdq,C0595989,T191,Disorders what research (or clinical trials) is being done for Laryngeal Cancer ?,0000024_2-8,research,"New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI Web site. Chemoprevention Chemoprevention is the use of drugs, vitamins, or other substances to reduce the risk of developing cancer or to reduce the risk cancer will recur (come back). The drug isotretinoin is being studied to prevent the development of a second cancer in patients who have had cancer of the head or neck. Radiosensitizers Radiosensitizers are drugs that make tumor cells more sensitive to radiation therapy. Combining radiation therapy with radiosensitizers may kill more tumor cells. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Laryngeal Cancer,0000024_2,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/laryngeal-treatment-pdq,C0595989,T191,Disorders What is (are) Lip and Oral Cavity Cancer ?,0000024_3-1,information,"Key Points - Lip and oral cavity cancer is a disease in which malignant (cancer) cells form in the lips or mouth. - Tobacco and alcohol use can affect the risk of lip and oral cavity cancer. - Signs of lip and oral cavity cancer include a sore or lump on the lips or in the mouth. - Tests that examine the mouth and throat are used to detect (find), diagnose, and stage lip and oral cavity cancer. - Certain factors affect prognosis (chance of recovery) and treatment options. Lip and oral cavity cancer is a disease in which malignant (cancer) cells form in the lips or mouth. The oral cavity includes the following: - The front two thirds of the tongue. - The gingiva (gums). - The buccal mucosa (the lining of the inside of the cheeks). - The floor (bottom) of the mouth under the tongue. - The hard palate (the roof of the mouth). - The retromolar trigone (the small area behind the wisdom teeth). Most lip and oral cavity cancers start in squamous cells, the thin, flat cells that line the lips and oral cavity. These are called squamous cell carcinomas. Cancer cells may spread into deeper tissue as the cancer grows. Squamous cell carcinoma usually develops in areas of leukoplakia (white patches of cells that do not rub off). Lip and oral cavity cancer is a type of head and neck cancer.",Lip and Oral Cavity Cancer,0000024_3,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/lip-mouth-treatment-pdq,C0153381,T191,Disorders Who is at risk for Lip and Oral Cavity Cancer? ?,0000024_3-2,susceptibility,Tobacco and alcohol use can affect the risk of lip and oral cavity cancer. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for lip and oral cavity cancer include the following: - Using tobacco products. - Heavy alcohol use. - Being exposed to natural sunlight or artificial sunlight (such as from tanning beds) over long periods of time. - Being male.,Lip and Oral Cavity Cancer,0000024_3,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/lip-mouth-treatment-pdq,C0153381,T191,Disorders What are the symptoms of Lip and Oral Cavity Cancer ?,0000024_3-3,symptoms,"Signs of lip and oral cavity cancer include a sore or lump on the lips or in the mouth. These and other signs and symptoms may be caused by lip and oral cavity cancer or by other conditions. Check with your doctor if you have any of the following: - A sore on the lip or in the mouth that does not heal. - A lump or thickening on the lips or gums or in the mouth. - A white or red patch on the gums, tongue, or lining of the mouth. - Bleeding, pain, or numbness in the lip or mouth. - Change in voice. - Loose teeth or dentures that no longer fit well. - Trouble chewing or swallowing or moving the tongue or jaw. - Swelling of jaw. - Sore throat or feeling that something is caught in the throat. Lip and oral cavity cancer may not have any symptoms and is sometimes found during a regular dental exam.",Lip and Oral Cavity Cancer,0000024_3,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/lip-mouth-treatment-pdq,C0153381,T191,Disorders How to diagnose Lip and Oral Cavity Cancer ?,0000024_3-4,exams and tests,"Tests that examine the mouth and throat are used to detect (find), diagnose, and stage lip and oral cavity cancer. The following tests and procedures may be used: - Physical exam of the lips and oral cavity: An exam to check the lips and oral cavity for abnormal areas. The medical doctor or dentist will feel the entire inside of the mouth with a gloved finger and examine the oral cavity with a small long-handled mirror and lights. This will include checking the insides of the cheeks and lips; the gums; the roof and floor of the mouth; and the top, bottom, and sides of the tongue. The neck will be felt for swollen lymph nodes. A history of the patients health habits and past illnesses and medical and dental treatments will also be taken. - Endoscopy : A procedure to look at organs and tissues inside the body to check for abnormal areas. An endoscope is inserted through an incision (cut) in the skin or opening in the body, such as the mouth. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of disease. - Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist. If leukoplakia is found, cells taken from the patches are also checked under the microscope for signs of cancer. - Exfoliative cytology : A procedure to collect cells from the lip or oral cavity. A piece of cotton, a brush, or a small wooden stick is used to gently scrape cells from the lips, tongue, mouth, or throat. The cells are viewed under a microscope to find out if they are abnormal. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - Barium swallow : A series of x-rays of the esophagus and stomach. The patient drinks a liquid that contains barium (a silver-white metallic compound). The liquid coats the esophagus and x-rays are taken. This procedure is also called an upper GI series. - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. - Bone scan : A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones and is detected by a scanner.",Lip and Oral Cavity Cancer,0000024_3,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/lip-mouth-treatment-pdq,C0153381,T191,Disorders What is the outlook for Lip and Oral Cavity Cancer ?,0000024_3-5,outlook,"Certain factors affect prognosis (chance of recovery) and treatment options. Prognosis (chance of recovery) depends on the following: - The stage of the cancer. - Where the tumor is in the lip or oral cavity. - Whether the cancer has spread to blood vessels. For patients who smoke, the chance of recovery is better if they stop smoking before beginning radiation therapy. Treatment options depend on the following: - The stage of the cancer. - The size of the tumor and where it is in the lip or oral cavity. - Whether the patient's appearance and ability to talk and eat can stay the same. - The patient's age and general health. Patients who have had lip and oral cavity cancer have an increased risk of developing a second cancer in the head or neck. Frequent and careful follow-up is important. Clinical trials are studying the use of retinoid drugs to reduce the risk of a second head and neck cancer. Information about ongoing clinical trials is available from the NCI website.",Lip and Oral Cavity Cancer,0000024_3,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/lip-mouth-treatment-pdq,C0153381,T191,Disorders What are the stages of Lip and Oral Cavity Cancer ?,0000024_3-6,stages,"Key Points - After lip and oral cavity cancer has been diagnosed, tests are done to find out if cancer cells have spread within the lip and oral cavity or to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - The following stages are used for lip and oral cavity cancer: - Stage 0 (Carcinoma in Situ) - Stage I - Stage II - Stage III - Stage IV After lip and oral cavity cancer has been diagnosed, tests are done to find out if cancer cells have spread within the lip and oral cavity or to other parts of the body. The process used to find out if cancer has spread within the lip and oral cavity or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The results of the tests used to diagnose lip and oral cavity cancer are also used to stage the disease. (See the General Information section.) There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if lip cancer spreads to the lung, the cancer cells in the lung are actually lip cancer cells. The disease is metastatic lip cancer, not lung cancer. The following stages are used for lip and oral cavity cancer: Stage 0 (Carcinoma in Situ) In stage 0, abnormal cells are found in the lining of the lips and oral cavity. These abnormal cells may become cancer and spread into nearby normal tissue. Stage 0 is also called carcinoma in situ. Stage I In stage I, cancer has formed and the tumor is 2 centimeters or smaller. Cancer has not spread to the lymph nodes. Stage II In stage II, the tumor is larger than 2 centimeters but not larger than 4 centimeters, and cancer has not spread to the lymph nodes. Stage III In stage III, the tumor: - may be any size and has spread to one lymph node that is 3 centimeters or smaller, on the same side of the neck as the tumor; or - is larger than 4 centimeters. Stage IV Stage IV is divided into stages IVA, IVB, and IVC. - In stage IVA, the tumor: - has spread through tissue in the lip or oral cavity into nearby tissue and/or bone (jaw, tongue, floor of mouth, maxillary sinus, or skin on the chin or nose); cancer may have spread to one lymph node that is 3 centimeters or smaller, on the same side of the neck as the tumor; or - is any size or has spread through tissue in the lip or oral cavity into nearby tissue and/or bone (jaw, tongue, floor of mouth, maxillary sinus, or skin on the chin or nose), and cancer has spread: - to one lymph node on the same side of the neck as the tumor and the lymph node is larger than 3 centimeters but not larger than 6 centimeters; or - to more than one lymph node on the same side of the neck as the tumor and the lymph nodes are not larger than 6 centimeters; or - to lymph nodes on the opposite side of the neck as the tumor or on both sides of the neck, and the lymph nodes are not larger than 6 centimeters. - In stage IVB, the tumor: - may be any size and has spread to one or more lymph nodes that are larger than 6 centimeters; or - has spread further into the muscles or bones in the oral cavity, or to the base of the skull and/or the carotid artery. Cancer may have spread to one or more lymph nodes anywhere in the neck. - In stage IVC, the tumor has spread beyond the lip or oral cavity to distant parts of the body, such as the lungs. The tumor may be any size and may have spread to the lymph nodes.",Lip and Oral Cavity Cancer,0000024_3,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/lip-mouth-treatment-pdq,C0153381,T191,Disorders What are the treatments for Lip and Oral Cavity Cancer ?,0000024_3-7,treatment,"Key Points - There are different types of treatment for patients with lip and oral cavity cancer. - Patients with lip and oral cavity cancer should have their treatment planned by a team of doctors who are expert in treating head and neck cancer. - Two types of standard treatment are used: - Surgery - Radiation therapy - New types of treatment are being tested in clinical trials. - Chemotherapy - Hyperfractionated radiation therapy - Hyperthermia therapy - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with lip and oral cavity cancer. Different types of treatment are available for patients with lip and oral cavity cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Patients with lip and oral cavity cancer should have their treatment planned by a team of doctors who are expert in treating head and neck cancer. Treatment will be overseen by a medical oncologist, a doctor who specializes in treating people with cancer. Because the lips and oral cavity are important for breathing, eating, and talking, patients may need special help adjusting to the side effects of the cancer and its treatment. The medical oncologist may refer the patient to other health professionals with special training in the treatment of patients with head and neck cancer. These include the following: - Head and neck surgeon. - Radiation oncologist. - Dentist. - Speech therapist. - Dietitian. - Psychologist. - Rehabilitation specialist. - Plastic surgeon. Two types of standard treatment are used: Surgery Surgery (removing the cancer in an operation) is a common treatment for all stages of lip and oral cavity cancer. Surgery may include the following: - Wide local excision: Removal of the cancer and some of the healthy tissue around it. If cancer has spread into bone, surgery may include removal of the involved bone tissue. - Neck dissection: Removal of lymph nodes and other tissues in the neck. This is done when cancer may have spread from the lip and oral cavity. - Plastic surgery: An operation that restores or improves the appearance of parts of the body. Dental implants, a skin graft, or other plastic surgery may be needed to repair parts of the mouth, throat, or neck after removal of large tumors. Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External and internal radiation therapy are used to treat lip and oral cavity cancer. Radiation therapy may work better in patients who have stopped smoking before beginning treatment. It is also important for patients to have a dental exam before radiation therapy begins, so that existing problems can be treated. New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. Hyperfractionated radiation therapy Hyperfractionated radiation therapy is radiation treatment in which the total dose of radiation is divided into small doses and the treatments are given more than once a day. Hyperthermia therapy Hyperthermia therapy is a treatment in which body tissue is heated above normal temperature to damage and kill cancer cells or to make cancer cells more sensitive to the effects of radiation and certain anticancer drugs. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options by Stage Stage I Lip and Oral Cavity Cancer Treatment of stage I lip and oral cavity cancer depends on where cancer is found in the lip and oral cavity. Lip If cancer is in the lip, treatment may include the following: - Surgery (wide local excision). - Internal radiation therapy with or without external radiation therapy. Front of the tongue If cancer is in the front of the tongue, treatment may include the following: - Surgery (wide local excision). - Internal radiation therapy with or without external radiation therapy. - Radiation therapy to lymph nodes in the neck. Buccal mucosa If cancer is in the buccal mucosa (the lining of the inside of the cheeks), treatment may include the following: - Surgery (wide local excision) for tumors smaller than 1 centimeter, with or without internal and/or external radiation therapy. - Surgery (wide local excision with skin graft) or radiation therapy for larger tumors. Floor of the mouth If cancer is in the floor (bottom) of the mouth, treatment may include the following: - Surgery (wide local excision) for tumors smaller than centimeter. - Surgery (wide local excision) or radiation therapy for larger tumors. Lower gingiva If cancer is in the lower gingiva (gums), treatment may include the following: - Surgery (wide local excision, which may include removing part of the jawbone, and skin graft). - Radiation therapy with or without surgery. Retromolar trigone If cancer is in the retromolar trigone (the small area behind the wisdom teeth), treatment may include the following: - Surgery (wide local excision, which may include removing part of the jawbone.) - Radiation therapy with or without surgery. Upper gingiva or hard palate If cancer is in the upper gingiva (gums) or the hard palate (the roof of the mouth), treatment is usually surgery (wide local excision) with or without radiation therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I lip and oral cavity cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage II Lip and Oral Cavity Cancer Treatment of stage II lip and oral cavity cancer depends on where cancer is found in the lip and oral cavity. Lip If cancer is in the lip, treatment may include the following: - Surgery (wide local excision). - External radiation therapy and/or internal radiation therapy. Front of the tongue If cancer is in the front of the tongue, treatment may include the following: - Radiation therapy and/or surgery (wide local excision). - Internal radiation therapy with surgery (neck dissection). Buccal mucosa If cancer is in the buccal mucosa (the lining of the inside of the cheeks), treatment may include the following: - Radiation therapy for tumors that are 3 centimeters or smaller. - Surgery (wide local excision) and/or radiation therapy for larger tumors. Floor of the mouth If cancer is in the floor (bottom) of the mouth, treatment may include the following: - Surgery (wide local excision). - Radiation therapy. - Surgery (wide local excision) followed by external radiation therapy, with or without internal radiation therapy, for large tumors. Lower gingiva If cancer is in the lower gingiva (gums), treatment may include the following: - Surgery (wide local excision, which may include removing part of the jawbone, and a skin graft). - Radiation therapy alone or after surgery. Retromolar trigone If cancer is in the retromolar trigone (the small area behind the wisdom teeth), treatment may include the following: - Surgery (wide local excision, which includes removing part of the jawbone). - Radiation therapy with or without surgery. Upper gingiva or hard palate If cancer is in the upper gingiva (gums) or the hard palate (the roof of the mouth), treatment may include the following: - Surgery (wide local excision) with or without radiation therapy. - Radiation therapy alone. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage II lip and oral cavity cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage III Lip and Oral Cavity Cancer Treatment of stage III lip and oral cavity cancer depends on where cancer is found in the lip and oral cavity. Lip If cancer is in the lip, treatment may include the following: - Surgery and external radiation therapy with or without internal radiation therapy. - A clinical trial of chemotherapy before or after surgery. - A clinical trial of chemotherapy and radiation therapy. - A clinical trial of hyperfractionated radiation therapy. Front of the tongue If cancer is in the front of the tongue, treatment may include the following: - External radiation therapy with or without internal radiation therapy. - Surgery (wide local excision) followed by radiation therapy. - A clinical trial of chemotherapy and radiation therapy. - A clinical trial of hyperfractionated radiation therapy. Buccal mucosa If cancer is in the buccal mucosa (the lining of the inside of the cheeks), treatment may include the following: - Surgery (wide local excision) with or without radiation therapy. - Radiation therapy. - A clinical trial of chemotherapy before or after surgery. - A clinical trial of chemotherapy and radiation therapy. - A clinical trial of hyperfractionated radiation therapy. Floor of the mouth If cancer is in the floor (bottom) of the mouth, treatment may include the following: - Surgery (wide local excision, which may include removing part of the jawbone, with or without neck dissection). - External radiation therapy with or without internal radiation therapy. - A clinical trial of chemotherapy before or after surgery. - A clinical trial of chemotherapy and radiation therapy. - A clinical trial of hyperfractionated radiation therapy. Lower gingiva If cancer is in the lower gingiva (gums), treatment may include the following: - Surgery (wide local excision) with or without radiation therapy. Radiation may be given before or after surgery. - A clinical trial of chemotherapy and radiation therapy. - A clinical trial of hyperfractionated radiation therapy. Retromolar trigone If cancer is in the retromolar trigone (the small area behind the wisdom teeth), treatment may include the following: - Surgery to remove the tumor, lymph nodes, and part of the jawbone, with or without radiation therapy. - A clinical trial of chemotherapy before or after surgery. - A clinical trial of chemotherapy and radiation therapy. - A clinical trial of hyperfractionated radiation therapy. Upper gingiva If cancer is in the upper gingiva (gums), treatment may include the following: - Radiation therapy. - Surgery (wide local excision) and radiation therapy. - A clinical trial of chemotherapy and radiation therapy. - A clinical trial of hyperfractionated radiation therapy. Hard palate If cancer is in the hard palate (the roof of the mouth), treatment may include the following: - Radiation therapy. - Surgery (wide local excision) with or without radiation therapy. - A clinical trial of chemotherapy and radiation therapy. - A clinical trial of hyperfractionated radiation therapy. Lymph nodes For cancer that may have spread to lymph nodes, treatment may include the following: - Radiation therapy and/or surgery (neck dissection). - A clinical trial of chemotherapy and radiation therapy. - A clinical trial of hyperfractionated radiation therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III lip and oral cavity cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage IV Lip and Oral Cavity Cancer Treatment of stage IV lip and oral cavity cancer depends on where cancer is found in the lip and oral cavity. Lip If cancer is in the lip, treatment may include the following: - Surgery and external radiation therapy with or without internal radiation therapy. - A clinical trial of chemotherapy and radiation therapy. - A clinical trial of chemotherapy before or after surgery. - A clinical trial of hyperfractionated radiation therapy. Front of the tongue If cancer is in the front of the tongue, treatment may include the following: - Surgery to remove the tongue and sometimes the larynx (voice box) with or without radiation therapy. - Radiation therapy as palliative therapy to relieve symptoms and improve quality of life. - A clinical trial of chemotherapy and radiation therapy. - A clinical trial of chemotherapy before or after surgery. - A clinical trial of hyperfractionated radiation therapy. Buccal mucosa If cancer is in the buccal mucosa (the lining of the inside of the cheeks), treatment may include the following: - Surgery (wide local excision) and/or radiation therapy. - A clinical trial of chemotherapy and radiation therapy. - A clinical trial of chemotherapy before or after surgery. - A clinical trial of hyperfractionated radiation therapy. Floor of the mouth If cancer is in the floor (bottom) of the mouth, treatment may include the following: - Surgery before or after radiation therapy. - A clinical trial of chemotherapy and radiation therapy. - A clinical trial of chemotherapy before or after surgery. - A clinical trial of hyperfractionated radiation therapy. Lower gingiva If cancer is in the lower gingiva (gums), treatment may include the following: - Surgery and/or radiation therapy. - A clinical trial of chemotherapy and radiation therapy. - A clinical trial of chemotherapy before or after surgery. - A clinical trial of hyperfractionated radiation therapy. Retromolar trigone If cancer is in the retromolar trigone (the small area behind the wisdom teeth), treatment may include the following: - Surgery to remove the tumor, lymph nodes, and part of the jawbone, followed by radiation therapy. - A clinical trial of chemotherapy and radiation therapy. - A clinical trial of chemotherapy before or after surgery. - A clinical trial of hyperfractionated radiation therapy. Upper gingiva or hard palate If cancer is in the upper gingiva (gums) or hard palate (the roof of the mouth), treatment may include the following: - Surgery with radiation therapy. - A clinical trial of chemotherapy and radiation therapy. - A clinical trial of chemotherapy before or after surgery. - A clinical trial of hyperfractionated radiation therapy. Lymph nodes For cancer that may have spread to lymph nodes, treatment may include the following: - Radiation therapy and/or surgery (neck dissection). - A clinical trial of chemotherapy and radiation therapy. - A clinical trial of chemotherapy before or after surgery. - A clinical trial of hyperfractionated radiation therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage IV lip and oral cavity cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Lip and Oral Cavity Cancer,0000024_3,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/lip-mouth-treatment-pdq,C0153381,T191,Disorders what research (or clinical trials) is being done for Lip and Oral Cavity Cancer ?,0000024_3-8,research,"New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. Hyperfractionated radiation therapy Hyperfractionated radiation therapy is radiation treatment in which the total dose of radiation is divided into small doses and the treatments are given more than once a day. Hyperthermia therapy Hyperthermia therapy is a treatment in which body tissue is heated above normal temperature to damage and kill cancer cells or to make cancer cells more sensitive to the effects of radiation and certain anticancer drugs. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Lip and Oral Cavity Cancer,0000024_3,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/lip-mouth-treatment-pdq,C0153381,T191,Disorders What is (are) Metastatic Squamous Neck Cancer with Occult Primary ?,0000024_4-1,information,"Key Points - Metastatic squamous neck cancer with occult primary is a disease in which squamous cell cancer spreads to lymph nodes in the neck and it is not known where the cancer first formed in the body. - Signs and symptoms of metastatic squamous neck cancer with occult primary include a lump or pain in the neck or throat. - Tests that examine the tissues of the neck, respiratory tract, and upper part of the digestive tract are used to detect (find) and diagnose metastatic squamous neck cancer and the primary tumor. - Certain factors affect prognosis (chance of recovery) and treatment options. Metastatic squamous neck cancer with occult primary is a disease in which squamous cell cancer spreads to lymph nodes in the neck and it is not known where the cancer first formed in the body. Squamous cells are thin, flat cells found in tissues that form the surface of the skin and the lining of body cavities such as the mouth, hollow organs such as the uterus and blood vessels, and the lining of the respiratory (breathing) and digestive tracts. Some organs with squamous cells are the esophagus, lungs, kidneys, and uterus. Cancer can begin in squamous cells anywhere in the body and metastasize (spread) through the blood or lymph system to other parts of the body. When squamous cell cancer spreads to lymph nodes in the neck or around the collarbone, it is called metastatic squamous neck cancer. The doctor will try to find the primary tumor (the cancer that first formed in the body), because treatment for metastatic cancer is the same as treatment for the primary tumor. For example, when lung cancer spreads to the neck, the cancer cells in the neck are lung cancer cells and they are treated the same as the cancer in the lung. Sometimes doctors cannot find where in the body the cancer first began to grow. When tests cannot find a primary tumor, it is called an occult (hidden) primary tumor. In many cases, the primary tumor is never found.",Metastatic Squamous Neck Cancer with Occult Primary,0000024_4,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/metastatic-squamous-neck-treatment-pdq,C0746787,T191,Disorders What are the symptoms of Metastatic Squamous Neck Cancer with Occult Primary ?,0000024_4-2,symptoms,Signs and symptoms of metastatic squamous neck cancer with occult primary include a lump or pain in the neck or throat. Check with your doctor if you have a lump or pain in your neck or throat that doesn't go away. These and other signs and symptoms may be caused by metastatic squamous neck cancer with occult primary. Other conditions may cause the same signs and symptoms.,Metastatic Squamous Neck Cancer with Occult Primary,0000024_4,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/metastatic-squamous-neck-treatment-pdq,C0746787,T191,Disorders How to diagnose Metastatic Squamous Neck Cancer with Occult Primary ?,0000024_4-3,exams and tests,"Tests that examine the tissues of the neck, respiratory tract, and upper part of the digestive tract are used to detect (find) and diagnose metastatic squamous neck cancer and the primary tumor. Tests will include checking for a primary tumor in the organs and tissues of the respiratory tract (part of the trachea), the upper part of the digestive tract (including the lips, mouth, tongue, nose, throat, vocal cords, and part of the esophagus), and the genitourinary system. The following procedures may be used: - Physical exam and history : An exam of the body, especially the head and neck, to check general signs of health. This includes checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist or tested in the laboratory to check for signs of cancer. Three types of biopsy may be done: - Fine-needle aspiration (FNA) biopsy : The removal of tissue or fluid using a thin needle. - Core needle biopsy : The removal of tissue using a wide needle. - Excisional biopsy : The removal of an entire lump of tissue. The following procedures are used to remove samples of cells or tissue: - Tonsillectomy: Surgery to remove both tonsils. - Endoscopy : A procedure to look at organs and tissues inside the body to check for abnormal areas. An endoscope is inserted through an incision (cut) in the skin or opening in the body, such as the mouth or nose. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove abnormal tissue or lymph node samples, which are checked under a microscope for signs of disease. The nose, throat, back of the tongue, esophagus, stomach, voice box, windpipe, and large airways will be checked. One or more of the following laboratory tests may be done to study the tissue samples: - Immunohistochemistry : A test that uses antibodies to check for certain antigens in a sample of blood or bone marrow. The antibody is usually linked to a radioactive substance or a dye that causes the tissue to light up under a microscope. This type of test may be used to tell the difference between different types of cancer. - Light and electron microscopy : A test in which cells in a sample of tissue are viewed under regular and high-powered microscopes to look for certain changes in the cells. - Epstein-Barr virus (EBV) and human papillomavirus (HPV) test: A test that checks the cells in a sample of tissue for EBV and HPV DNA. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. A whole body PET scan and a CT scan are done at the same time to look for where the cancer first formed. If there is any cancer, this increases the chance that it will be found. A diagnosis of occult primary tumor is made if the primary tumor is not found during testing or treatment.",Metastatic Squamous Neck Cancer with Occult Primary,0000024_4,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/metastatic-squamous-neck-treatment-pdq,C0746787,T191,Disorders What is the outlook for Metastatic Squamous Neck Cancer with Occult Primary ?,0000024_4-4,outlook,Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - The number and size of lymph nodes that have cancer in them. - Whether the cancer has responded to treatment or has recurred (come back). - How different from normal the cancer cells look under a microscope. - The patient's age and general health. Treatment options also depend on the following: - Which part of the neck the cancer is in. - Whether certain tumor markers are found.,Metastatic Squamous Neck Cancer with Occult Primary,0000024_4,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/metastatic-squamous-neck-treatment-pdq,C0746787,T191,Disorders What are the stages of Metastatic Squamous Neck Cancer with Occult Primary ?,0000024_4-5,stages,"Key Points - After metastatic squamous neck cancer with occult primary has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body. - There are three ways that cancer spreads in the body. After metastatic squamous neck cancer with occult primary has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body. The process used to find out if cancer has spread to other parts of the body is called staging. The results from tests and procedures used to detect and diagnose the primary tumor are also used to find out if cancer has spread to other parts of the body. There is no standard staging system for metastatic squamous neck cancer with occult primary. The tumors are described as untreated or recurrent. Untreated metastatic squamous neck cancer with occult primary is cancer that is newly diagnosed and has not been treated, except to relieve signs and symptoms caused by the cancer. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.",Metastatic Squamous Neck Cancer with Occult Primary,0000024_4,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/metastatic-squamous-neck-treatment-pdq,C0746787,T191,Disorders What are the treatments for Metastatic Squamous Neck Cancer with Occult Primary ?,0000024_4-6,treatment,"Key Points - There are different types of treatment for patients with metastatic squamous neck cancer with occult primary. - Two types of standard treatment are used: - Surgery - Radiation therapy - New types of treatment are being tested in clinical trials. - Chemotherapy - Hyperfractionated radiation therapy - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with metastatic squamous neck cancer with occult primary. Different types of treatment are available for patients with metastatic squamous neck cancer with occult primary. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Two types of standard treatment are used: Surgery Surgery may include neck dissection. There are different types of neck dissection, based on the amount of tissue that is removed. - Radical neck dissection: Surgery to remove tissues in one or both sides of the neck between the jawbone and the collarbone, including the following: - All lymph nodes. - The jugular vein. - Muscles and nerves that are used for face, neck, and shoulder movement, speech, and swallowing. The patient may need physical therapy of the throat, neck, shoulder, and/or arm after radical neck dissection. Radical neck dissection may be used when cancer has spread widely in the neck. - Modified radical neck dissection: Surgery to remove all the lymph nodes in one or both sides of the neck without removing the neck muscles. The nerves and/or the jugular vein may be removed. - Partial neck dissection: Surgery to remove some of the lymph nodes in the neck. This is also called selective neck dissection. Even if the doctor removes all the cancer that can be seen at the time of surgery, some patients may be given radiation therapy after surgery to kill any cancer cells that are left. Treatment given after surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. Certain ways of giving radiation therapy can help keep radiation from damaging nearby healthy tissue. This type of radiation therapy may include the following: - Intensity-modulated radiation therapy (IMRT): IMRT is a type of 3-dimensional (3-D) radiation therapy that uses a computer to make pictures of the size and shape of the tumor. Thin beams of radiation of different intensities (strengths) are aimed at the tumor from many angles. This type of radiation therapy is less likely to cause dry mouth, trouble swallowing, and damage to the skin. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type of cancer being treated. External radiation therapy is used to treat metastatic squamous neck cancer with occult primary. Radiation therapy to the neck may change the way the thyroid gland works. Blood tests may be done to check the thyroid hormone level in the body before treatment and at regular checkups after treatment. New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Hyperfractionated radiation therapy Hyperfractionated radiation therapy is a type of external radiation treatment in which a smaller than usual total daily dose of radiation is divided into two doses and the treatments are given twice a day. Hyperfractionated radiation therapy is given over the same period of time (days or weeks) as standard radiation therapy. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Metastatic Squamous Neck Cancer with Occult Primary Untreated Metastatic Squamous Neck Cancer with Occult Primary Treatment of untreated metastatic squamous neck cancer with occult primary may include the following: - Radiation therapy. - Surgery. - Radiation therapy followed by surgery. - A clinical trial of chemotherapy followed by radiation therapy. - A clinical trial of chemotherapy given at the same time as hyperfractionated radiation therapy. - Clinical trials of new treatments. Check the list of NCI-supported cancer clinical trials that are now accepting patients with untreated metastatic squamous neck cancer with occult primary. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Recurrent Metastatic Squamous Neck Cancer with Occult Primary Treatment of recurrent metastatic squamous neck cancer with occult primary is usually within a clinical trial. Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent metastatic squamous neck cancer with occult primary. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Metastatic Squamous Neck Cancer with Occult Primary,0000024_4,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/metastatic-squamous-neck-treatment-pdq,C0746787,T191,Disorders what research (or clinical trials) is being done for Metastatic Squamous Neck Cancer with Occult Primary ?,0000024_4-7,research,"New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Hyperfractionated radiation therapy Hyperfractionated radiation therapy is a type of external radiation treatment in which a smaller than usual total daily dose of radiation is divided into two doses and the treatments are given twice a day. Hyperfractionated radiation therapy is given over the same period of time (days or weeks) as standard radiation therapy. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Metastatic Squamous Neck Cancer with Occult Primary,0000024_4,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/metastatic-squamous-neck-treatment-pdq,C0746787,T191,Disorders What is (are) Nasopharyngeal Cancer ?,0000024_5-1,information,"Key Points - Nasopharyngeal cancer is a disease in which malignant (cancer) cells form in the tissues of the nasopharynx. - Ethnic background and being exposed to the Epstein-Barr virus can affect the risk of nasopharyngeal cancer. - Signs of nasopharyngeal cancer include trouble breathing, speaking, or hearing. - Tests that examine the nose and throat are used to detect (find) and diagnose nasopharyngeal cancer. - Certain factors affect prognosis (chance of recovery) and treatment options. Nasopharyngeal cancer is a disease in which malignant (cancer) cells form in the tissues of the nasopharynx. The nasopharynx is the upper part of the pharynx (throat) behind the nose. The pharynx is a hollow tube about 5 inches long that starts behind the nose and ends at the top of the trachea (windpipe) and esophagus (the tube that goes from the throat to the stomach). Air and food pass through the pharynx on the way to the trachea or the esophagus. The nostrils lead into the nasopharynx. An opening on each side of the nasopharynx leads into an ear. Nasopharyngeal cancer most commonly starts in the squamous cells that line the nasopharynx. Nasopharyngeal cancer is a type of head and neck cancer.",Nasopharyngeal Cancer,0000024_5,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/nasopharyngeal-treatment-pdq,C0153392,T191,Disorders Who is at risk for Nasopharyngeal Cancer? ?,0000024_5-2,susceptibility,"Ethnic background and being exposed to the Epstein-Barr virus can affect the risk of nasopharyngeal cancer. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for nasopharyngeal cancer include the following: - Having Chinese or Asian ancestry. - Being exposed to the Epstein-Barr virus: The Epstein-Barr virus has been associated with certain cancers, including nasopharyngeal cancer and some lymphomas. - Drinking large amounts of alcohol.",Nasopharyngeal Cancer,0000024_5,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/nasopharyngeal-treatment-pdq,C0153392,T191,Disorders What are the symptoms of Nasopharyngeal Cancer ?,0000024_5-3,symptoms,"Signs of nasopharyngeal cancer include trouble breathing, speaking, or hearing. These and other signs and symptoms may be caused by nasopharyngeal cancer or by other conditions. Check with your doctor if you have any of the following: - A lump in the nose or neck. - A sore throat. - Trouble breathing or speaking. - Nosebleeds. - Trouble hearing. - Pain or ringing in the ear. - Headaches.",Nasopharyngeal Cancer,0000024_5,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/nasopharyngeal-treatment-pdq,C0153392,T191,Disorders How to diagnose Nasopharyngeal Cancer ?,0000024_5-4,exams and tests,"Tests that examine the nose and throat are used to detect (find) and diagnose nasopharyngeal cancer. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as swollen lymph nodes in the neck or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Neurological exam : A series of questions and tests to check the brain, spinal cord, and nerve function. The exam checks a persons mental status, coordination, and ability to walk normally, and how well the muscles, senses, and reflexes work. This may also be called a neuro exam or a neurologic exam. - Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. The tissue sample is removed during one of the following procedures: - Nasoscopy : A procedure to look inside the nose for abnormal areas. A nasoscope is inserted through the nose. A nasoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer. - Upper endoscopy : A procedure to look at the inside of the nose, throat, esophagus, stomach, and duodenum (first part of the small intestine, near the stomach). An endoscope is inserted through the mouth and into the esophagus, stomach, and duodenum. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples. The tissue samples are checked under a microscope for signs of cancer. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. PET scans may be used to find nasopharyngeal cancers that have spread to the bone. Sometimes a PET scan and a CT scan are done at the same time. If there is any cancer, this increases the chance that it will be found. - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. - Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following: - The number of red blood cells, white blood cells, and platelets. - The amount of hemoglobin (the protein that carries oxygen) in the red blood cells. - The portion of the blood sample made up of red blood cells. - Epstein-Barr virus (EBV) test: A blood test to check for antibodies to the Epstein-Barr virus and DNA markers of the Epstein-Barr virus. These are found in the blood of patients who have been infected with EBV. - Hearing test: A procedure to check whether soft and loud sounds and low- and high-pitched sounds can be heard. Each ear is checked separately.",Nasopharyngeal Cancer,0000024_5,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/nasopharyngeal-treatment-pdq,C0153392,T191,Disorders What is the outlook for Nasopharyngeal Cancer ?,0000024_5-5,outlook,"Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - The stage of the cancer (whether it affects part of the nasopharynx, involves the whole nasopharynx, or has spread to other places in the body). - The type of nasopharyngeal cancer. - The size of the tumor. - The patients age and general health.",Nasopharyngeal Cancer,0000024_5,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/nasopharyngeal-treatment-pdq,C0153392,T191,Disorders What are the stages of Nasopharyngeal Cancer ?,0000024_5-6,stages,"Key Points - After nasopharyngeal cancer has been diagnosed, tests are done to find out if cancer cells have spread within the nasopharynx or to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - The following stages are used for nasopharyngeal cancer: - Stage 0 (Carcinoma in Situ) - Stage I - Stage II - Stage III - Stage IV After nasopharyngeal cancer has been diagnosed, tests are done to find out if cancer cells have spread within the nasopharynx or to other parts of the body. The process used to find out whether cancer has spread within the nasopharynx or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The results of the tests used to diagnose nasopharyngeal cancer are often also used to stage the disease. (See the General Information section.) There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if nasopharyngeal cancer spreads to the lung, the cancer cells in the lung are actually nasopharyngeal cancer cells. The disease is metastatic nasopharyngeal cancer, not lung cancer. The following stages are used for nasopharyngeal cancer: Stage 0 (Carcinoma in Situ) In stage 0, abnormal cells are found in the lining of the nasopharynx. These abnormal cells may become cancer and spread into nearby normal tissue. Stage 0 is also called carcinoma in situ. Stage I In stage I, cancer has formed and the cancer: - is found in the nasopharynx only; or - has spread from the nasopharynx to the oropharynx and/or to the nasal cavity. The oropharynx is the middle part of the throat and includes the soft palate, the base of the tongue, and the tonsils. Stage II In stage II nasopharyngeal cancer, the cancer: - is found in the nasopharynx only or has spread from the nasopharynx to the oropharynx and/or to the nasal cavity. Cancer has spread to one or more lymph nodes on one side of the neck and/or to lymph nodes behind the pharynx. The affected lymph nodes are 6 centimeters or smaller; or - is found in the parapharyngeal space. Cancer may have spread to one or more lymph nodes on one side of the neck and/or to lymph nodes behind the pharynx. The affected lymph nodes are 6 centimeters or smaller. The oropharynx is the middle part of the throat and includes the soft palate, the base of the tongue, and the tonsils. The parapharyngeal space is a fat-filled, triangular area near the pharynx, between the base of the skull and the lower jaw. Stage III In stage III nasopharyngeal cancer, the cancer: - is found in the nasopharynx only or has spread from the nasopharynx to the oropharynx and/or to the nasal cavity. Cancer has spread to one or more lymph nodes on both sides of the neck. The affected lymph nodes are 6 centimeters or smaller; or - is found in the parapharyngeal space. Cancer has spread to one or more lymph nodes on both sides of the neck. The affected lymph nodes are 6 centimeters or smaller; or - has spread to nearby bones or sinuses. Cancer may have spread to one or more lymph nodes on one or both sides of the neck and/or to lymph nodes behind the pharynx. The affected lymph nodes are 6 centimeters or smaller. The oropharynx is the middle part of the throat and includes the soft palate, the base of the tongue, and the tonsils. The parapharyngeal space is a fat-filled, triangular area near the pharynx, between the base of the skull and the lower jaw. Stage IV Stage IV nasopharyngeal cancer is divided into stages IVA, IVB, and IVC. - Stage IVA: Cancer has spread beyond the nasopharynx and may have spread to the cranial nerves, the hypopharynx (bottom part of the throat), areas in and around the side of the skull or jawbone, and/or the bone around the eye. Cancer may also have spread to one or more lymph nodes on one or both sides of the neck and/or to lymph nodes behind the pharynx. The affected lymph nodes are 6 centimeters or smaller. - Stage IVB: Cancer has spread to lymph nodes between the collarbone and the top of the shoulder and/or the affected lymph nodes are larger than 6 centimeters. - Stage IVC: Cancer has spread beyond nearby lymph nodes to other parts of the body.",Nasopharyngeal Cancer,0000024_5,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/nasopharyngeal-treatment-pdq,C0153392,T191,Disorders What are the treatments for Nasopharyngeal Cancer ?,0000024_5-7,treatment,"Key Points - There are different types of treatment for patients with nasopharyngeal cancer. - Three types of standard treatment are used: - Radiation therapy - Chemotherapy - Surgery - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with nasopharyngeal cancer. Different types of treatment are available for patients with nasopharyngeal cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Three types of standard treatment are used: Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. Certain ways of giving radiation therapy can help keep radiation from damaging nearby healthy tissue. These types of radiation therapy include the following: - Intensity-modulated radiation therapy (IMRT): IMRT is a type of 3-dimensional (3-D) radiation therapy that uses a computer to make pictures of the size and shape of the tumor. Thin beams of radiation of different intensities (strengths) are aimed at the tumor from many angles. Compared to standard radiation therapy, intensity-modulated radiation therapy may be less likely to cause dry mouth. - Stereotactic radiation therapy: A rigid head frame is attached to the skull to keep the head still during the radiation treatment. A machine aims radiation directly at the tumor. The total dose of radiation is divided into several smaller doses given over several days. This procedure is also called stereotactic external-beam radiation therapy and stereotaxic radiation therapy. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External and internal radiation therapy are used to treat nasopharyngeal cancer. External radiation therapy to the thyroid or the pituitary gland may change the way the thyroid gland works. A blood test to check the thyroid hormone level in the blood is done before and after therapy to make sure the thyroid gland is working properly. It is also important that a dentist check the patients teeth, gums, and mouth, and fix any existing problems before radiation therapy begins. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. Chemotherapy may be given after radiation therapy to kill any cancer cells that are left. Treatment given after radiation therapy, to lower the risk that the cancer will come back, is called adjuvant therapy. See Drugs Approved for Head and Neck Cancer for more information. (Nasopharyngeal cancer is a type of head and neck cancer.) Surgery Surgery is a procedure to find out whether cancer is present, to remove cancer from the body, or to repair a body part. Also called an operation. Surgery is sometimes used for nasopharyngeal cancer that does not respond to radiation therapy. If cancer has spread to the lymph nodes, the doctor may remove lymph nodes and other tissues in the neck. New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options by Stage Stage I Nasopharyngeal Cancer Treatment of stage I nasopharyngeal cancer is usually radiation therapy to the tumor and lymph nodes in the neck. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I nasopharyngeal cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage II Nasopharyngeal Cancer Treatment of stage II nasopharyngeal cancer may include the following: - Chemotherapy given with radiation therapy, followed by more chemotherapy. - Radiation therapy to the tumor and lymph nodes in the neck. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage II nasopharyngeal cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage III Nasopharyngeal Cancer Treatment of stage III nasopharyngeal cancer may include the following: - Chemotherapy given with radiation therapy, which may be followed by more chemotherapy. - Radiation therapy. - Radiation therapy followed by surgery to remove cancer -containing lymph nodes in the neck that remain or come back after radiation therapy. - A clinical trial of chemotherapy given before, with, or after radiation therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III nasopharyngeal cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage IV Nasopharyngeal Cancer Treatment of stage IV nasopharyngeal cancer may include the following: - Chemotherapy given with radiation therapy, followed by more chemotherapy. - Radiation therapy. - Radiation therapy followed by surgery to remove cancer -containing lymph nodes in the neck that remain or come back after radiation therapy. - Chemotherapy for cancer that has metastasized (spread) to other parts of the body. - A clinical trial of chemotherapy given before, with, or after radiation therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage IV nasopharyngeal cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Nasopharyngeal Cancer,0000024_5,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/nasopharyngeal-treatment-pdq,C0153392,T191,Disorders what research (or clinical trials) is being done for Nasopharyngeal Cancer ?,0000024_5-8,research,"New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Nasopharyngeal Cancer,0000024_5,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/nasopharyngeal-treatment-pdq,C0153392,T191,Disorders What is (are) Oropharyngeal Cancer ?,0000024_6-1,information,"Key Points - Oropharyngeal cancer is a disease in which malignant (cancer) cells form in the tissues of the oropharynx. - Smoking or being infected with human papillomavirus can increase the risk of oropharyngeal cancer. - Signs and symptoms of oropharyngeal cancer include a lump in the neck and a sore throat. - Tests that examine the mouth and throat are used to help detect (find), diagnose, and stage oropharyngeal cancer. - Certain factors affect prognosis (chance of recovery) and treatment options. Oropharyngeal cancer is a disease in which malignant (cancer) cells form in the tissues of the oropharynx. The oropharynx is the middle part of the pharynx (throat), behind the mouth. The pharynx is a hollow tube about 5 inches long that starts behind the nose and ends where the trachea (windpipe) and esophagus (tube from the throat to the stomach) begin. Air and food pass through the pharynx on the way to the trachea or the esophagus. The oropharynx includes the following: - Soft palate. - Side and back walls of the throat. - Tonsils. - Back one-third of the tongue. Oropharyngeal cancer is a type of head and neck cancer. Sometimes more than one cancer can occur in the oropharynx and in other parts of the oral cavity, nose, pharynx, larynx (voice box), trachea, or esophagus at the same time. Most oropharyngeal cancers are squamous cell carcinomas. Squamous cells are the thin, flat cells that line the inside of the oropharynx. See the following PDQ summaries for more information about other types of head and neck cancers: - Hypopharyngeal Cancer Treatment - Lip and Oral Cavity Cancer Treatment - Oral Cavity and Oropharyngeal Cancer Prevention - Oral Cavity and Oropharyngeal Cancer Screening",Oropharyngeal Cancer,0000024_6,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/oropharyngeal-treatment-pdq,C0153382,T191,Disorders Who is at risk for Oropharyngeal Cancer? ?,0000024_6-2,susceptibility,"Smoking or being infected with human papillomavirus can increase the risk of oropharyngeal cancer. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. The most common risk factors for oropharyngeal cancer include the following: - A history of smoking cigarettes for more than 10 pack years and other tobacco use. - Personal history of head and neck cancer. - Heavy alcohol use. - Being infected with human papillomavirus (HPV), especially HPV type 16. The number of cases of oropharyngeal cancers linked to HPV infection is increasing. - Chewing betel quid, a stimulant commonly used in parts of Asia.",Oropharyngeal Cancer,0000024_6,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/oropharyngeal-treatment-pdq,C0153382,T191,Disorders What are the symptoms of Oropharyngeal Cancer ?,0000024_6-3,symptoms,"Signs and symptoms of oropharyngeal cancer include a lump in the neck and a sore throat. These and other signs and symptoms may be caused by oropharyngeal cancer or by other conditions. Check with your doctor if you have any of the following: - A sore throat that does not go away. - Trouble swallowing. - Trouble opening the mouth fully. - Trouble moving the tongue. - Weight loss for no known reason. - Ear pain. - A lump in the back of the mouth, throat, or neck. - A white patch on the tongue or lining of the mouth that does not go away. - Coughing up blood. Sometimes oropharyngeal cancer does not cause early signs or symptoms.",Oropharyngeal Cancer,0000024_6,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/oropharyngeal-treatment-pdq,C0153382,T191,Disorders How to diagnose Oropharyngeal Cancer ?,0000024_6-4,exams and tests,"Tests that examine the mouth and throat are used to help detect (find), diagnose, and stage oropharyngeal cancer. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as swollen lymph nodes in the neck or anything else that seems unusual. The medical doctor or dentist does a complete exam of the mouth and neck and looks under the tongue and down the throat with a small, long-handled mirror to check for abnormal areas. An exam of the eyes may be done to check for vision problems that are caused by nerves in the head and neck. A history of the patients health habits and past illnesses and treatments will also be taken. - PET-CT scan : A procedure that combines the pictures from a positron emission tomography (PET) scan and a computed tomography (CT) scan. The PET and CT scans are done at the same time with the same machine. The combined scans give more detailed pictures of areas inside the body than either scan gives by itself. A PET-CT scan may be used to help diagnose disease, such as cancer, plan treatment, or find out how well treatment is working. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the head and neck, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye is injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. A fine-needle biopsy is usually done to remove a sample of tissue using a thin needle. The following procedures may be used to remove samples of cells or tissue: - Endoscopy : A procedure to look at organs and tissues inside the body to check for abnormal areas. An endoscope is inserted through an incision (cut) in the skin or opening in the body, such as the mouth or nose. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove abnormal tissue or lymph node samples, which are checked under a microscope for signs of disease. The nose, throat, back of the tongue, esophagus, stomach, larynx, windpipe, and large airways will be checked. The type of endoscopy is named for the part of the body that is being examined. For example, pharyngoscopy is an exam to check the pharynx. - Laryngoscopy : A procedure in which the doctor checks the larynx with a mirror or with a laryngoscope. A laryngoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove abnormal tissue or lymph node samples, which are checked under a microscope for signs of disease. If cancer is found, the following test may be done to study the cancer cells: - HPV test (human papillomavirus test): A laboratory test used to check the sample of tissue for certain types of HPV infection. This test is done because oropharyngeal cancer can be caused by HPV.",Oropharyngeal Cancer,0000024_6,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/oropharyngeal-treatment-pdq,C0153382,T191,Disorders What is the outlook for Oropharyngeal Cancer ?,0000024_6-5,outlook,Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) depends on the following: - Whether the patient has HPV infection of the oropharynx. - Whether the patient has a history of smoking cigarettes for ten or more pack years. - The stage of the cancer. - The number and size of lymph nodes with cancer. Oropharyngeal tumors related to HPV infection have a better prognosis and are less likely to recur than tumors not linked to HPV infection. Treatment options depend on the following: - The stage of the cancer. - Keeping the patient's ability to speak and swallow as normal as possible. - The patient's general health. Patients with oropharyngeal cancer have an increased risk of another cancer in the head or neck. This risk is increased in patients who continue to smoke or drink alcohol after treatment. See the PDQ summary Cigarette Smoking: Health Risks and How to Quit for more information.,Oropharyngeal Cancer,0000024_6,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/oropharyngeal-treatment-pdq,C0153382,T191,Disorders What are the stages of Oropharyngeal Cancer ?,0000024_6-6,stages,"Key Points - After oropharyngeal cancer has been diagnosed, tests are done to find out if cancer cells have spread within the oropharynx or to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - The following stages are used for oropharyngeal cancer: - Stage 0 (Carcinoma in Situ) - Stage I - Stage II - Stage III - Stage IV After oropharyngeal cancer has been diagnosed, tests are done to find out if cancer cells have spread within the oropharynx or to other parts of the body. The process used to find out if cancer has spread within the oropharynx or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The results of some of the tests used to diagnose oropharyngeal cancer are often used to stage the disease. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if oropharyngeal cancer spreads to the lung, the cancer cells in the lung are actually oropharyngeal cancer cells. The disease is metastatic oropharyngeal cancer, not lung cancer. The following stages are used for oropharyngeal cancer: Stage 0 (Carcinoma in Situ) In stage 0, abnormal cells are found in the lining of the oropharynx. These abnormal cells may become cancer and spread into nearby normal tissue. Stage 0 is also called carcinoma in situ. Stage I In stage I, cancer has formed and is 2 centimeters or smaller and is found in the oropharynx only. Stage II In stage II, the cancer is larger than 2 centimeters but not larger than 4 centimeters and is found in the oropharynx only. Stage III In stage III, the cancer is either: - 4 centimeters or smaller; cancer has spread to one lymph node on the same side of the neck as the tumor and the lymph node is 3 centimeters or smaller; or - larger than 4 centimeters or has spread to the epiglottis (the flap that covers the trachea during swallowing). Cancer may have spread to one lymph node on the same side of the neck as the tumor and the lymph node is 3 centimeters or smaller. Stage IV Stage IV is divided into stage IVA, IVB, and IVC: - In stage IVA, cancer: - has spread to the larynx, front part of the roof of the mouth, lower jaw, or muscles that move the tongue or are used for chewing. Cancer may have spread to one lymph node on the same side of the neck as the tumor and the lymph node is 3 centimeters or smaller; or - has spread to one lymph node on the same side of the neck as the tumor (the lymph node is larger than 3 centimeters but not larger than 6 centimeters) or to more than one lymph node anywhere in the neck (the lymph nodes are 6 centimeters or smaller), and one of the following is true: - tumor in the oropharynx is any size and may have spread to the epiglottis (the flap that covers the trachea during swallowing); or - tumor has spread to the larynx, front part of the roof of the mouth, lower jaw, or muscles that move the tongue or are used for chewing. - In stage IVB, the tumor: - surrounds the carotid artery or has spread to the muscle that opens the jaw, the bone attached to the muscles that move the jaw, nasopharynx, or base of the skull. Cancer may have spread to one or more lymph nodes which can be any size; or - may be any size and has spread to one or more lymph nodes that are larger than 6 centimeters. - In stage IVC, the tumor may be any size and has spread beyond the oropharynx to other parts of the body, such as the lung, bone, or liver.",Oropharyngeal Cancer,0000024_6,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/oropharyngeal-treatment-pdq,C0153382,T191,Disorders What are the treatments for Oropharyngeal Cancer ?,0000024_6-7,treatment,"Key Points - There are different types of treatment for patients with oropharyngeal cancer. - Patients with oropharyngeal cancer should have their treatment planned by a team of doctors with expertise in treating head and neck cancer. - Four types of standard treatment are used: - Surgery - Radiation therapy - Chemotherapy - Targeted therapy - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with oropharyngeal cancer. Different types of treatment are available for patients with oropharyngeal cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Patients with oropharyngeal cancer should have their treatment planned by a team of doctors with expertise in treating head and neck cancer. The patient's treatment will be overseen by a medical oncologist, a doctor who specializes in treating people with cancer. Because the oropharynx helps in breathing, eating, and talking, patients may need special help adjusting to the side effects of the cancer and its treatment. The medical oncologist may refer the patient to other health professionals with special training in the treatment of patients with head and neck cancer. These may include the following specialists: - Head and neck surgeon. - Radiation oncologist. - Plastic surgeon. - Dentist. - Dietitian. - Psychologist. - Rehabilitation specialist. - Speech therapist. Four types of standard treatment are used: Surgery Surgery (removing the cancer in an operation) is a common treatment of all stages of oropharyngeal cancer. A surgeon may remove the cancer and some of the healthy tissue around the cancer. Even if the surgeon removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. New types of surgery, including transoral robotic surgery, are being studied for the treatment of oropharyngeal cancer. Transoral robotic surgery may be used to remove cancer from hard-to-reach areas of the mouth and throat. Cameras attached to a robot give a 3-dimensional (3D) image that a surgeon can see. Using a computer, the surgeon guides very small tools at the ends of the robot arms to remove the cancer. This procedure may also be done using an endoscope. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. Certain ways of giving radiation therapy can help keep radiation from damaging nearby healthy tissue. These types of radiation therapy include the following: - Intensity-modulated radiation therapy (IMRT): IMRT is a type of 3-dimensional (3-D) radiation therapy that uses a computer to make pictures of the size and shape of the tumor. Thin beams of radiation of different intensities (strengths) are aimed at the tumor from many angles. - Stereotactic body radiation therapy: Stereotactic body radiation therapy is a type of external radiation therapy. Special equipment is used to place the patient in the same position for each radiation treatment. Once a day for several days, a radiation machine aims a larger than usual dose of radiation directly at the tumor. By having the patient in the same position for each treatment, there is less damage to nearby healthy tissue. This procedure is also called stereotactic external-beam radiation therapy and stereotaxic radiation therapy. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. In advanced oropharyngeal cancer, dividing the daily dose of radiation into smaller-dose treatments improves the way the tumor responds to treatment. This is called hyperfractionated radiation therapy. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External radiation therapy is used to treat oropharyngeal cancer. Radiation therapy may work better in patients who have stopped smoking before beginning treatment. If the thyroid or pituitary gland are part of the radiation treatment area, the patient has an increased risk of hypothyroidism (too little thyroid hormone). A blood test to check the thyroid hormone level in the body should be done before and after treatment. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. Systemic chemotherapy is used to treat oropharyngeal cancer. See Drugs Approved for Head and Neck Cancer for more information. (Oropharyngeal cancer is a type of head and neck cancer.) Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. Monoclonal antibodies are a type of targeted therapy being used in the treatment of oropharyngeal cancer. Monoclonal antibody therapy is a cancer treatment that uses antibodies made in the laboratory from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances in the blood or tissues that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Cetuximab is a type of monoclonal antibody that works by binding to a protein on the surface of the cancer cells and stops the cells from growing and dividing. It is used in the treatment of recurrent oropharyngeal cancer. Other types of monoclonal antibody therapy are being studied in the treatment of oropharyngeal cancer. Nivolumab is being studied in the treatment of stage III and IV oropharyngeal cancer. See Drugs Approved for Head and Neck Cancer for more information. (Oropharyngeal cancer is a type of head and neck cancer.) New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Following treatment, it is important to have careful head and neck exams to look for signs that the cancer has come back. Check-ups will be done every 6 to 12 weeks in the first year, every 3 months in the second year, every 3 to 4 months in the third year, and every 6 months thereafter. Treatment Options by Stage Stage I and Stage II Oropharyngeal Cancer Treatment of stage I and stage II oropharyngeal cancer may include the following: - Radiation therapy. - Surgery. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I oropharyngeal cancer and stage II oropharyngeal cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage III and Stage IV Oropharyngeal Cancer Treatment of stage III oropharyngeal cancer and stage IV oropharyngeal cancer may include the following: - For patients with locally advanced cancer, surgery followed by radiation therapy. Chemotherapy also may be given at the same time as radiation therapy. - Radiation therapy alone for patients who cannot have chemotherapy. - Chemotherapy given at the same time as radiation therapy. - Chemotherapy followed by radiation therapy given at the same time as more chemotherapy. - A clinical trial of chemotherapy followed by surgery or radiation therapy. - A clinical trial of targeted therapy (nivolumab) with chemotherapy given at the same time as radiation therapy in patients with advanced HPV -positive oropharyngeal cancer. - A clinical trial of radiation therapy with or without chemotherapy. - A clinical trial of transoral surgery followed by standard - or low-dose radiation therapy with or without chemotherapy in patients with HPV-positive oropharyngeal cancer. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III oropharyngeal cancer and stage IV oropharyngeal cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Recurrent Oropharyngeal Cancer Treatment of recurrent oropharyngeal cancer may include the following: - Surgery, if the tumor does not respond to radiation therapy. - Radiation therapy, if the tumor was not completely removed by surgery and previous radiation has not been given. - Second surgery, if the tumor was not completely removed by the first surgery. - Chemotherapy for patients with recurrent cancer that cannot be removed by surgery. - Radiation therapy given at the same time as chemotherapy. - Stereotactic body radiation therapy given at the same time as targeted therapy (cetuximab). - Clinical trials of targeted therapy, stereotactic body radiation therapy, or hyperfractionated radiation therapy given at the same time as chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent oropharyngeal cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Oropharyngeal Cancer,0000024_6,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/oropharyngeal-treatment-pdq,C0153382,T191,Disorders what research (or clinical trials) is being done for Oropharyngeal Cancer ?,0000024_6-8,research,"New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Oropharyngeal Cancer,0000024_6,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/oropharyngeal-treatment-pdq,C0153382,T191,Disorders What is (are) Paranasal Sinus and Nasal Cavity Cancer ?,0000024_7-1,information,"Key Points - Paranasal sinus and nasal cavity cancer is a disease in which malignant (cancer) cells form in the tissues of the paranasal sinuses and nasal cavity. - Different types of cells in the paranasal sinus and nasal cavity may become malignant. - Being exposed to certain chemicals or dust in the workplace can increase the risk of paranasal sinus and nasal cavity cancer. - Signs of paranasal sinus and nasal cavity cancer include sinus problems and nosebleeds. - Tests that examine the sinuses and nasal cavity are used to detect (find) and diagnose paranasal sinus and nasal cavity cancer. - Certain factors affect prognosis (chance of recovery) and treatment options. Paranasal sinus and nasal cavity cancer is a disease in which malignant (cancer) cells form in the tissues of the paranasal sinuses and nasal cavity. Paranasal sinuses ""Paranasal"" means near the nose. The paranasal sinuses are hollow, air-filled spaces in the bones around the nose. The sinuses are lined with cells that make mucus, which keeps the inside of the nose from drying out during breathing. There are several paranasal sinuses named after the bones that surround them: - The frontal sinuses are in the lower forehead above the nose. - The maxillary sinuses are in the cheekbones on either side of the nose. - The ethmoid sinuses are beside the upper nose, between the eyes. - The sphenoid sinuses are behind the nose, in the center of the skull. Nasal cavity The nose opens into the nasal cavity, which is divided into two nasal passages. Air moves through these passages during breathing. The nasal cavity lies above the bone that forms the roof of the mouth and curves down at the back to join the throat. The area just inside the nostrils is called the nasal vestibule. A small area of special cells in the roof of each nasal passage sends signals to the brain to give the sense of smell. Together the paranasal sinuses and the nasal cavity filter and warm the air, and make it moist before it goes into the lungs. The movement of air through the sinuses and other parts of the respiratory system help make sounds for talking. Paranasal sinus and nasal cavity cancer is a type of head and neck cancer. Different types of cells in the paranasal sinus and nasal cavity may become malignant. The most common type of paranasal sinus and nasal cavity cancer is squamous cell carcinoma. This type of cancer forms in the squamous cells (thin, flat cells) lining the inside of the paranasal sinuses and the nasal cavity. Other types of paranasal sinus and nasal cavity cancer include the following: - Melanoma: Cancer that starts in cells called melanocytes, the cells that give skin its natural color. - Sarcoma: Cancer that starts in muscle or connective tissue. - Inverting papilloma: Benign tumors that form inside the nose. A small number of these change into cancer. - Midline granulomas: Cancer of tissues in the middle part of the face.",Paranasal Sinus and Nasal Cavity Cancer,0000024_7,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/paranasal-sinus-treatment-pdq,C1710095,T191,Disorders Who is at risk for Paranasal Sinus and Nasal Cavity Cancer? ?,0000024_7-2,susceptibility,"Being exposed to certain chemicals or dust in the workplace can increase the risk of paranasal sinus and nasal cavity cancer. Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesnt mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for paranasal sinus and nasal cavity cancer include the following: - Being exposed to certain workplace chemicals or dust, such as those found in the following jobs: - Furniture-making. - Sawmill work. - Woodworking (carpentry). - Shoemaking. - Metal-plating. - Flour mill or bakery work. - Being infected with human papillomavirus (HPV). - Being male and older than 40 years. - Smoking.",Paranasal Sinus and Nasal Cavity Cancer,0000024_7,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/paranasal-sinus-treatment-pdq,C1710095,T191,Disorders What are the symptoms of Paranasal Sinus and Nasal Cavity Cancer ?,0000024_7-3,symptoms,"Signs of paranasal sinus and nasal cavity cancer include sinus problems and nosebleeds. These and other signs and symptoms may be caused by paranasal sinus and nasal cavity cancer or by other conditions. There may be no signs or symptoms in the early stages. Signs and symptoms may appear as the tumor grows. Check with your doctor if you have any of the following: - Blocked sinuses that do not clear, or sinus pressure. - Headaches or pain in the sinus areas. - A runny nose. - Nosebleeds. - A lump or sore inside the nose that does not heal. - A lump on the face or roof of the mouth. - Numbness or tingling in the face. - Swelling or other trouble with the eyes, such as double vision or the eyes pointing in different directions. - Pain in the upper teeth, loose teeth, or dentures that no longer fit well. - Pain or pressure in the ear.",Paranasal Sinus and Nasal Cavity Cancer,0000024_7,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/paranasal-sinus-treatment-pdq,C1710095,T191,Disorders How to diagnose Paranasal Sinus and Nasal Cavity Cancer ?,0000024_7-4,exams and tests,"Tests that examine the sinuses and nasal cavity are used to detect (find) and diagnose paranasal sinus and nasal cavity cancer. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Physical exam of the nose, face, and neck: An exam in which the doctor looks into the nose with a small, long-handled mirror to check for abnormal areas and checks the face and neck for lumps or swollen lymph nodes. - X-rays of the head and neck: An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. There are three types of biopsy: - Fine-needle aspiration (FNA) biopsy : The removal of tissue or fluid using a thin needle. - Incisional biopsy : The removal of part of an area of tissue that doesnt look normal. - Excisional biopsy : The removal of an entire area of tissue that doesnt look normal. - Nasoscopy : A procedure to look inside the nose for abnormal areas. A nasoscope is inserted into the nose. A nasoscope is a thin, tube-like instrument with a light and a lens for viewing. A special tool on the nasoscope may be used to remove samples of tissue. The tissues samples are viewed under a microscope by a pathologist to check for signs of cancer. - Laryngoscopy : A procedure to look at the larynx (voice box) for abnormal areas. A mirror or a laryngoscope (a thin, tube-like instrument with a light and a lens for viewing) is inserted through the mouth to see the larynx. A special tool on the laryngoscope may be used to remove samples of tissue. The tissue samples are viewed under a microscope by a pathologist to check for signs of cancer.",Paranasal Sinus and Nasal Cavity Cancer,0000024_7,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/paranasal-sinus-treatment-pdq,C1710095,T191,Disorders What is the outlook for Paranasal Sinus and Nasal Cavity Cancer ?,0000024_7-5,outlook,"Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - Where the tumor is in the paranasal sinus or nasal cavity and whether it has spread. - The size of the tumor. - The type of cancer. - The patient's age and general health. - Whether the cancer has just been diagnosed or has recurred (come back). Paranasal sinus and nasal cavity cancers often have spread by the time they are diagnosed and are hard to cure. After treatment, a lifetime of frequent and careful follow-up is important because there is an increased risk of developing a second kind of cancer in the head or neck.",Paranasal Sinus and Nasal Cavity Cancer,0000024_7,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/paranasal-sinus-treatment-pdq,C1710095,T191,Disorders What are the stages of Paranasal Sinus and Nasal Cavity Cancer ?,0000024_7-6,stages,"Key Points - After paranasal sinus and nasal cavity cancer has been diagnosed, tests are done to find out if cancer cells have spread within the paranasal sinuses and nasal cavity or to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - There is no standard staging system for cancer of the sphenoid and frontal sinuses. - The following stages are used for maxillary sinus cancer: - Stage 0 (Carcinoma in Situ) - Stage I - Stage II - Stage III - Stage IV - The following stages are used for nasal cavity and ethmoid sinus cancer: - Stage 0 (Carcinoma in Situ) - Stage I - Stage II - Stage III - Stage IV After paranasal sinus and nasal cavity cancer has been diagnosed, tests are done to find out if cancer cells have spread within the paranasal sinuses and nasal cavity or to other parts of the body. The process used to find out if cancer has spread within the paranasal sinuses and nasal cavity or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following tests and procedures may be used in the staging process: - Endoscopy : A procedure to look at organs and tissues inside the body to check for abnormal areas. An endoscope is inserted through an opening in the body, such as the nose or mouth. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of disease. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - Chest x-ray : An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. - MRI (magnetic resonance imaging) with gadolinium : A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. Sometimes a substance called gadolinium is injected into a vein. The gadolinium collects around the cancer cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI). - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. - Bone scan : A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones and is detected by a scanner. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if nasal cavity cancer spreads to the lung, the cancer cells in the lung are actually nasal cavity cancer cells. The disease is metastatic nasal cavity cancer, not lung cancer. There is no standard staging system for cancer of the sphenoid and frontal sinuses. The following stages are used for maxillary sinus cancer: Stage 0 (Carcinoma in Situ) In stage 0, abnormal cells are found in the innermost lining of the maxillary sinus. These abnormal cells may become cancer and spread into nearby normal tissue. Stage 0 is also called carcinoma in situ. Stage I In stage I, cancer has formed in the mucous membranes of the maxillary sinus. Stage II In stage II, cancer has spread to bone around the maxillary sinus, including the roof of the mouth and the nose, but not to bone at the back of the maxillary sinus or the base of the skull. Stage III In stage III, cancer has spread to any of the following: - Bone at the back of the maxillary sinus. - Tissues under the skin. - The eye socket. - The base of the skull. - The ethmoid sinuses. or Cancer has spread to one lymph node on the same side of the neck as the cancer and the lymph node is 3 centimeters or smaller. Cancer has also spread to any of the following: - The lining of the maxillary sinus. - Bones around the maxillary sinus, including the roof of the mouth and the nose. - Tissues under the skin. - The eye socket. - The base of the skull. - The ethmoid sinuses. Stage IV Stage IV is divided into stage IVA, IVB, and IVC. Stage IVA In stage IVA, cancer has spread: - to one lymph node on the same side of the neck as the cancer and the lymph node is larger than 3 centimeters but not larger than 6 centimeters; or - to more than one lymph node on the same side of the neck as the original tumor and the lymph nodes are not larger than 6 centimeters; or - to lymph nodes on the opposite side of the neck as the original tumor or on both sides of the neck, and the lymph nodes are not larger than 6 centimeters. and cancer has spread to any of the following: - The lining of the maxillary sinus. - Bones around the maxillary sinus, including the roof of the mouth and the nose. - Tissues under the skin. - The eye socket. - The base of the skull. - The ethmoid sinuses. or Cancer has spread to any of the following: - The front of the eye. - The skin of the cheek. - The base of the skull. - Behind the jaw. - The bone between the eyes. - The sphenoid or frontal sinuses. and cancer may also have spread to one or more lymph nodes 6 centimeters or smaller, anywhere in the neck. Stage IVB In stage IVB, cancer has spread to any of the following: - The back of the eye. - The brain. - The middle parts of the skull. - The nerves in the head that go to the brain. - The upper part of the throat behind the nose. - The base of the skull. and cancer may be found in one or more lymph nodes of any size, anywhere in the neck. or Cancer is found in a lymph node larger than 6 centimeters. Cancer may also be found anywhere in or near the maxillary sinus. Stage IVC In stage IVC, cancer may be anywhere in or near the maxillary sinus, may have spread to lymph nodes, and has spread to organs far away from the maxillary sinus, such as the lungs. The following stages are used for nasal cavity and ethmoid sinus cancer: Stage 0 (Carcinoma in Situ) In stage 0, abnormal cells are found in the innermost lining of the nasal cavity or ethmoid sinus. These abnormal cells may become cancer and spread into nearby normal tissue. Stage 0 is also called carcinoma in situ. Stage I In stage I, cancer has formed and is found in only one area (of either the nasal cavity or the ethmoid sinus) and may have spread into bone. Stage II In stage II, cancer is found in two areas (of either the nasal cavity or the ethmoid sinus) that are near each other or has spread to an area next to the sinuses. Cancer may also have spread into bone. Stage III In stage III, cancer has spread to any of the following: - The eye socket. - The maxillary sinus. - The roof of the mouth. - The bone between the eyes. or Cancer has spread to one lymph node on the same side of the neck as the cancer and the lymph node is 3 centimeters or smaller. Cancer has also spread to any of the following: - The nasal cavity. - The ethmoid sinus. - The eye socket. - The maxillary sinus. - The roof of the mouth. - The bone between the eyes. Stage IV Stage IV is divided into stage IVA, IVB, and IVC. Stage IVA In stage IVA, cancer has spread: - to one lymph node on the same side of the neck as the cancer and the lymph node is larger than 3 centimeters but not larger than 6 centimeters; or - to more than one lymph node on the same side of the neck as the original tumor and the lymph nodes are not larger than 6 centimeters; or - to lymph nodes on the opposite side of the neck as the original tumor or on both sides of the neck, and the lymph nodes are not larger than 6 centimeters. and cancer has spread to any of the following: - The nasal cavity. - The ethmoid sinus. - The eye socket. - The maxillary sinus. - The roof of the mouth. - The bone between the eyes. or Cancer has spread to any of the following: - The front of the eye. - The skin of the nose or cheek. - Front parts of the skull. - The base of the skull. - The sphenoid or frontal sinuses. and cancer may have spread to one or more lymph nodes 6 centimeters or smaller, anywhere in the neck. Stage IVB In stage IVB, cancer has spread to any of the following: - The back of the eye. - The brain. - The middle parts of the skull. - The nerves in the head that go to the brain. - The upper part of the throat behind the nose. - The base of the skull. and cancer may be found in one or more lymph nodes of any size, anywhere in the neck. or Cancer is found in a lymph node larger than 6 centimeters. Cancer may also be found anywhere in or near the nasal cavity and ethmoid sinus. Stage IVC In stage IVC, cancer may be anywhere in or near the nasal cavity and ethmoid sinus, may have spread to lymph nodes, and has spread to organs far away from the nasal cavity and ethmoid sinus, such as the lungs.",Paranasal Sinus and Nasal Cavity Cancer,0000024_7,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/paranasal-sinus-treatment-pdq,C1710095,T191,Disorders What are the treatments for Paranasal Sinus and Nasal Cavity Cancer ?,0000024_7-7,treatment,"Key Points - There are different types of treatment for patients with paranasal sinus and nasal cavity cancer. - Patients with paranasal sinus and nasal cavity cancer should have their treatment planned by a team of doctors with expertise in treating head and neck cancer. - Three types of standard treatment are used: - Surgery - Radiation therapy - Chemotherapy - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with paranasal sinus and nasal cavity cancer. Different types of treatment are available for patients with paranasal sinus and nasal cavity cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Patients with paranasal sinus and nasal cavity cancer should have their treatment planned by a team of doctors with expertise in treating head and neck cancer. Treatment will be overseen by a medical oncologist, a doctor who specializes in treating people with cancer. The medical oncologist works with other doctors who are experts in treating patients with head and neck cancer and who specialize in certain areas of medicine and rehabilitation. Patients who have paranasal sinus and nasal cavity cancer may need special help adjusting to breathing problems or other side effects of the cancer and its treatment. If a large amount of tissue or bone around the paranasal sinuses or nasal cavity is taken out, plastic surgery may be done to repair or rebuild the area. The treatment team may include the following specialists: - Radiation oncologist. - Neurologist. - Oral surgeon or head and neck surgeon. - Plastic surgeon. - Dentist. - Nutritionist. - Speech and language pathologist. - Rehabilitation specialist. Three types of standard treatment are used: Surgery Surgery (removing the cancer in an operation) is a common treatment for all stages of paranasal sinus and nasal cavity cancer. A doctor may remove the cancer and some of the healthy tissue and bone around the cancer. If the cancer has spread, the doctor may remove lymph nodes and other tissues in the neck. Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. The total dose of radiation therapy is sometimes divided into several smaller, equal doses delivered over a period of several days. This is called fractionation. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External and internal radiation therapy are used to treat paranasal sinus and nasal cavity cancer. External radiation therapy to the thyroid or the pituitary gland may change the way the thyroid gland works. The thyroid hormone levels in the blood may be tested before and after treatment. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is treatment using more than one anticancer drug. The way the chemotherapy is given depends on the type and stage of the cancer being treated. See Drugs Approved for Head and Neck Cancer for more information. (Paranasal sinus and nasal cavity cancer is a type of head and neck cancer.) New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options by Stage Stage I Paranasal Sinus and Nasal Cavity Cancer Treatment of stage I paranasal sinus and nasal cavity cancer depends on where cancer is found in the paranasal sinuses and nasal cavity: - If cancer is in the maxillary sinus, treatment is usually surgery with or without radiation therapy. - If cancer is in the ethmoid sinus, treatment is usually radiation therapy and/or surgery. - If cancer is in the sphenoid sinus, treatment is the same as for nasopharyngeal cancer, usually radiation therapy. (See the PDQ summary on Nasopharyngeal Cancer Treatment for more information.) - If cancer is in the nasal cavity, treatment is usually surgery and/or radiation therapy. - If cancer is in the nasal vestibule, treatment is usually surgery or radiation therapy. - For inverting papilloma, treatment is usually surgery with or without radiation therapy. - For melanoma and sarcoma, treatment is usually surgery with or without radiation therapy and chemotherapy. - For midline granuloma, treatment is usually radiation therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I paranasal sinus and nasal cavity cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage II Paranasal Sinus and Nasal Cavity Cancer Treatment of stage II paranasal sinus and nasal cavity cancer depends on where cancer is found in the paranasal sinuses and nasal cavity: - If cancer is in the maxillary sinus, treatment is usually high-dose radiation therapy before or after surgery. - If cancer is in the ethmoid sinus, treatment is usually radiation therapy and/or surgery. - If cancer is in the sphenoid sinus, treatment is the same as for nasopharyngeal cancer, usually radiation therapy with or without chemotherapy. (See the PDQ summary on Nasopharyngeal Cancer Treatment for more information.) - If cancer is in the nasal cavity, treatment is usually surgery and/or radiation therapy. - If cancer is in the nasal vestibule, treatment is usually surgery or radiation therapy. - For inverting papilloma, treatment is usually surgery with or without radiation therapy. - For melanoma and sarcoma, treatment is usually surgery with or without radiation therapy and chemotherapy. - For midline granuloma, treatment is usually radiation therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage II paranasal sinus and nasal cavity cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage III Paranasal Sinus and Nasal Cavity Cancer Treatment of stage III paranasal sinus and nasal cavity cancer depends on where cancer is found in the paranasal sinuses and nasal cavity. If cancer is in the maxillary sinus, treatment may include the following: - High-dose radiation therapy before or after surgery. - A clinical trial of fractionated radiation therapy before or after surgery. If cancer is in the ethmoid sinus, treatment may include the following: - Surgery followed by radiation therapy. - A clinical trial of combination chemotherapy before surgery or radiation therapy. - A clinical trial of combination chemotherapy after surgery or other cancer treatment. If cancer is in the sphenoid sinus, treatment is the same as for nasopharyngeal cancer, usually radiation therapy with or without chemotherapy. (See the PDQ summary on Nasopharyngeal Cancer Treatment for more information.) If cancer is in the nasal cavity, treatment may include the following: - Surgery and/or radiation therapy. - Chemotherapy and radiation therapy. - A clinical trial of combination chemotherapy before surgery or radiation therapy. - A clinical trial of combination chemotherapy after surgery or other cancer treatment. For inverting papilloma, treatment is usually surgery with or without radiation therapy. For melanoma and sarcoma, treatment may include the following: - Surgery. - Radiation therapy. - Surgery, radiation therapy, and chemotherapy. For midline granuloma, treatment is usually radiation therapy. If cancer is in the nasal vestibule, treatment may include the following: - External radiation therapy and/or internal radiation therapy with or without surgery. - A clinical trial of combination chemotherapy before surgery or radiation therapy. - A clinical trial of combination chemotherapy after surgery or other cancer treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III paranasal sinus and nasal cavity cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage IV Paranasal Sinus and Nasal Cavity Cancer Treatment of stage IV paranasal sinus and nasal cavity cancer depends on where cancer is found in the paranasal sinuses and nasal cavity. If cancer is in the maxillary sinus, treatment may include the following: - High-dose radiation therapy with or without surgery. - A clinical trial of fractionated radiation therapy. - A clinical trial of chemotherapy before surgery or radiation therapy. - A clinical trial of chemotherapy after surgery or other cancer treatment. - A clinical trial of chemotherapy and radiation therapy. If cancer is in the ethmoid sinus, treatment may include the following: - Radiation therapy before or after surgery. - Chemotherapy and radiation therapy. - A clinical trial of chemotherapy before surgery or radiation therapy. - A clinical trial of chemotherapy after surgery or other cancer treatment. - A clinical trial of chemotherapy and radiation therapy. If cancer is in the sphenoid sinus, treatment is the same as for nasopharyngeal cancer, usually radiation therapy with or without chemotherapy. (See the PDQ summary on Nasopharyngeal Cancer Treatment for more information.) If cancer is in the nasal cavity, treatment may include the following: - Surgery and/or radiation therapy. - Chemotherapy and radiation therapy. - A clinical trial of chemotherapy before surgery or radiation therapy. - A clinical trial of chemotherapy after surgery or other cancer treatment. - A clinical trial of chemotherapy and radiation therapy. For inverting papilloma, treatment is usually surgery with or without radiation therapy. For melanoma and sarcoma, treatment may include the following: - Surgery. - Radiation therapy. - Chemotherapy. For midline granuloma, treatment is usually radiation therapy. If cancer is in the nasal vestibule, treatment may include the following: - External radiation therapy and/or internal radiation therapy with or without surgery. - A clinical trial of chemotherapy before surgery or radiation therapy. - A clinical trial of chemotherapy after surgery or other cancer treatment. - A clinical trial of chemotherapy and radiation therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage IV paranasal sinus and nasal cavity cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Paranasal Sinus and Nasal Cavity Cancer,0000024_7,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/paranasal-sinus-treatment-pdq,C1710095,T191,Disorders what research (or clinical trials) is being done for Paranasal Sinus and Nasal Cavity Cancer ?,0000024_7-8,research,"New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Paranasal Sinus and Nasal Cavity Cancer,0000024_7,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/paranasal-sinus-treatment-pdq,C1710095,T191,Disorders What is (are) Salivary Gland Cancer ?,0000024_8-1,information,"Key Points - Salivary gland cancer is a rare disease in which malignant (cancer) cells form in the tissues of the salivary glands. - Being exposed to certain types of radiation may increase the risk of salivary cancer. - Signs of salivary gland cancer include a lump or trouble swallowing. - Tests that examine the head, neck, and the inside of the mouth are used to detect (find) and diagnose salivary gland cancer. - Certain factors affect treatment options and prognosis (chance of recovery). Salivary gland cancer is a rare disease in which malignant (cancer) cells form in the tissues of the salivary glands. The salivary glands make saliva and release it into the mouth. Saliva has enzymes that help digest food and antibodies that help protect against infections of the mouth and throat. There are 3 pairs of major salivary glands: - Parotid glands: These are the largest salivary glands and are found in front of and just below each ear. Most major salivary gland tumors begin in this gland. - Sublingual glands: These glands are found under the tongue in the floor of the mouth. - Submandibular glands: These glands are found below the jawbone. There are also hundreds of small (minor) salivary glands lining parts of the mouth, nose, and larynx that can be seen only with a microscope. Most small salivary gland tumors begin in the palate (roof of the mouth). More than half of all salivary gland tumors are benign (not cancerous) and do not spread to other tissues. Salivary gland cancer is a type of head and neck cancer.",Salivary Gland Cancer,0000024_8,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/salivary-gland-treatment-pdq,C0220636,T191,Disorders Who is at risk for Salivary Gland Cancer? ?,0000024_8-2,susceptibility,"Being exposed to certain types of radiation may increase the risk of salivary cancer. Anything that increases the chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesnt mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Although the cause of most salivary gland cancers is not known, risk factors include the following: - Older age. - Treatment with radiation therapy to the head and neck. - Being exposed to certain substances at work.",Salivary Gland Cancer,0000024_8,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/salivary-gland-treatment-pdq,C0220636,T191,Disorders What are the symptoms of Salivary Gland Cancer ?,0000024_8-3,symptoms,"Signs of salivary gland cancer include a lump or trouble swallowing. Salivary gland cancer may not cause any symptoms. It may be found during a regular dental check-up or physical exam. Signs and symptoms may be caused by salivary gland cancer or by other conditions. Check with your doctor if you have any of the following: - A lump (usually painless) in the area of the ear, cheek, jaw, lip, or inside the mouth. - Fluid draining from the ear. - Trouble swallowing or opening the mouth widely. - Numbness or weakness in the face. - Pain in the face that does not go away.",Salivary Gland Cancer,0000024_8,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/salivary-gland-treatment-pdq,C0220636,T191,Disorders How to diagnose Salivary Gland Cancer ?,0000024_8-4,exams and tests,"Tests that examine the head, neck, and the inside of the mouth are used to detect (find) and diagnose salivary gland cancer. The following procedures may be used: - Physical exam and history : An exam of the body to check general signs of health. The head, neck, mouth, and throat will be checked for signs of disease, such as lumps or anything else that seems unusual. A history of the patient's health habits and past illnesses and treatments will also be taken. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. - Endoscopy : A procedure to look at organs and tissues inside the body to check for abnormal areas. For salivary gland cancer, an endoscope is inserted into the mouth to look at the mouth, throat, and larynx. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. - Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. - Fine needle aspiration (FNA) biopsy : The removal of tissue or fluid using a thin needle. An FNA is the most common type of biopsy used for salivary gland cancer. - Incisional biopsy : The removal of part of a lump or a sample of tissue that doesnt look normal. - Surgery : If cancer cannot be diagnosed from the sample of tissue removed during an FNA biopsy or an incisional biopsy, the mass may be removed and checked for signs of cancer. Because salivary gland cancer can be hard to diagnose, patients should ask to have the tissue samples checked by a pathologist who has experience in diagnosing salivary gland cancer.",Salivary Gland Cancer,0000024_8,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/salivary-gland-treatment-pdq,C0220636,T191,Disorders What is the outlook for Salivary Gland Cancer ?,0000024_8-5,outlook,Certain factors affect treatment options and prognosis (chance of recovery). The treatment options and prognosis (chance of recovery) depend on the following: - The stage of the cancer (especially the size of the tumor). - The type of salivary gland the cancer is in. - The type of cancer cells (how they look under a microscope). - The patient's age and general health.,Salivary Gland Cancer,0000024_8,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/salivary-gland-treatment-pdq,C0220636,T191,Disorders What are the stages of Salivary Gland Cancer ?,0000024_8-6,stages,"Key Points - After salivary gland cancer has been diagnosed, tests are done to find out if cancer cells have spread within the salivary gland or to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - The following stages are used for major salivary gland cancers: - Stage I - Stage II - Stage III - Stage IV After salivary gland cancer has been diagnosed, tests are done to find out if cancer cells have spread within the salivary gland or to other parts of the body. The process used to find out if cancer has spread within the salivary glands or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following procedures may be used in the staging process: - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if salivary gland cancer spreads to the lung, the cancer cells in the lung are actually salivary gland cancer cells. The disease is metastatic salivary gland cancer, not lung cancer. The following stages are used for major salivary gland cancers: Stage I In stage I, the tumor is in the salivary gland only and is 2 centimeters or smaller. Stage II In stage II, the tumor is in the salivary gland only and is larger than 2 centimeters but not larger than 4 centimeters. Stage III In stage III, one of the following is true: - The tumor is not larger than 4 centimeters and has spread to a single lymph node on the same side as the tumor and the lymph node is 3 centimeters or smaller. - The tumor is larger than 4 centimeters and/or has spread to soft tissue around the affected gland. Cancer may have spread to a single lymph node on the same side as the tumor and the lymph node is 3 centimeters or smaller. Stage IV Stage IV is divided into stages IVA, IVB, and IVC as follows: - Stage IVA: - The tumor may be any size and may have spread to soft tissue around the affected gland. Cancer has spread to a single lymph node on the same side as the tumor and the lymph node is larger than 3 centimeters but not larger than 6 centimeters, or has spread to more than one lymph node on either or both sides of the body and the lymph nodes are not larger than 6 centimeters; or - Cancer has spread to the skin, jawbone, ear canal, and/or facial nerve, and may have spread to one or more lymph nodes on either or both sides of the body. The lymph nodes are not larger than 6 centimeters. - Stage IVB: - The tumor may be any size and may have spread to soft tissue around the affected gland. Cancer has spread to a lymph node larger than 6 centimeters; or - Cancer has spread to the base of the skull and/or the carotid artery, and may have spread to one or more lymph nodes of any size on either or both sides of the body. - Stage IVC: - The tumor may be any size and may have spread to soft tissue around the affected gland, to the skin, jawbone, ear canal, facial nerve, base of the skull, or carotid artery, or to one or more lymph nodes on either or both sides of the body. Cancer has spread to distant parts of the body. Salivary gland cancers are also grouped by grade. The grade of a tumor tells how fast the cancer cells are growing, based on how the cells look under a microscope. Low-grade cancers grow more slowly than high-grade cancers. Minor salivary gland cancers are staged according to where they were first found in the body.",Salivary Gland Cancer,0000024_8,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/salivary-gland-treatment-pdq,C0220636,T191,Disorders What are the treatments for Salivary Gland Cancer ?,0000024_8-7,treatment,"Key Points - There are different types of treatment for patients with salivary gland cancer. - Patients with salivary gland cancer should have their treatment planned by a team of doctors who are experts in treating head and neck cancer. - Three types of standard treatment are used: - Surgery - Radiation therapy - Chemotherapy - New types of treatment are being tested in clinical trials. - Radiosensitizers - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with salivary gland cancer. Different types of treatment are available for patients with salivary gland cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Patients with salivary gland cancer should have their treatment planned by a team of doctors who are experts in treating head and neck cancer. Your treatment will be overseen by a medical oncologist, a doctor who specializes in treating people with cancer. Because the salivary glands help in eating and digesting food, patients may need special help adjusting to the side effects of the cancer and its treatment. The medical oncologist may refer you to other doctors who have experience and expertise in treating patients with head and neck cancer and who specialize in certain areas of medicine. These include the following: - Head and neck surgeon. - Radiation oncologist. - Dentist. - Speech therapist. - Dietitian. - Psychologist. - Rehabilitation specialist. - Plastic surgeon. Three types of standard treatment are used: Surgery Surgery (removing the cancer in an operation) is a common treatment for salivary gland cancer. A doctor may remove the cancer and some of the healthy tissue around the cancer. In some cases, a lymphadenectomy (surgery in which lymph nodes are removed) will also be done. Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given radiation therapy after surgery to kill any cancer cells that are left. Treatment given after surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. Special types of external radiation may be used to treat some salivary gland tumors. These include: - Fast neutron radiation therapy: Fast neutron radiation therapy is a type of high-energy external radiation therapy. A radiation therapy machine aims neutrons (tiny, invisible particles) at the cancer cells to kill them. Fast neutron radiation therapy uses a higher-energy radiation than the x-ray type of radiation therapy. This allows the radiation therapy to be given in fewer treatments. - Photon-beam radiation therapy: Photon-beam radiation therapy is a type of external radiation therapy that reaches deep tumors with high-energy x-rays made by a machine called a linear accelerator. This can be delivered as hyperfractionated radiation therapy, in which the total dose of radiation is divided into small doses and the treatments are given more than once a day. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External radiation therapy is used to treat salivary gland cancer, and may also be used as palliative therapy to relieve symptoms and improve quality of life. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. See Drugs Approved for Head and Neck Cancer for more information. (Salivary gland cancer is a type of head and neck cancer.) New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Radiosensitizers Radiosensitizers are drugs that make tumor cells more sensitive to radiation therapy. Combining radiation therapy with radiosensitizers may kill more tumor cells. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options by Stage Stage I Salivary Gland Cancer Treatment for stage I salivary gland cancer depends on whether the cancer is low-grade (slow growing) or high-grade (fast growing). If the cancer is low-grade, treatment may include the following: - Surgery with or without radiation therapy. - Fast neutron radiation therapy. If the cancer is high-grade, treatment may include the following: - Surgery with or without radiation therapy. - A clinical trial of chemotherapy. - A clinical trial of a new local therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I salivary gland cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage II Salivary Gland Cancer Treatment for stage II salivary gland cancer depends on whether the cancer is low-grade (slow growing) or high-grade (fast growing). If the cancer is low-grade, treatment may include the following: - Surgery with or without radiation therapy. - Radiation therapy. - Chemotherapy. If the cancer is high-grade, treatment may include the following: - Surgery with or without radiation therapy. - Fast neutron or photon-beam radiation therapy. - A clinical trial of radiation therapy and/or radiosensitizers. - A clinical trial of chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage II salivary gland cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage III Salivary Gland Cancer Treatment for stage III salivary gland cancer depends on whether the cancer is low-grade (slow growing) or high-grade (fast growing). If the cancer is low-grade, treatment may include the following: - Surgery with or without lymphadenectomy. Radiation therapy may also be given after surgery. - Radiation therapy. - Fast neutron radiation therapy to lymph nodes with cancer. - Chemotherapy. - A clinical trial of fast neutron radiation therapy to the tumor. - A clinical trial of chemotherapy. If the cancer is high-grade, treatment may include the following: - Surgery with or without lymphadenectomy. Radiation therapy may also be given after surgery. - Fast neutron radiation therapy. - Radiation therapy as palliative therapy to relieve symptoms and improve quality of life. - A clinical trial of radiation therapy and/or radiosensitizers. - A clinical trial of chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III salivary gland cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage IV Salivary Gland Cancer Treatment of stage IV salivary gland cancer may include the following: - Fast neutron or photon-beam radiation therapy. - A clinical trial of chemotherapy with or without radiation therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage IV salivary gland cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Salivary Gland Cancer,0000024_8,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/salivary-gland-treatment-pdq,C0220636,T191,Disorders what research (or clinical trials) is being done for Salivary Gland Cancer ?,0000024_8-8,research,"New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Radiosensitizers Radiosensitizers are drugs that make tumor cells more sensitive to radiation therapy. Combining radiation therapy with radiosensitizers may kill more tumor cells. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Salivary Gland Cancer,0000024_8,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/salivary-gland-treatment-pdq,C0220636,T191,Disorders How to prevent Oral Cavity and Oropharyngeal Cancer ?,0000024_9-1,prevention,"Key Points - Avoiding risk factors and increasing protective factors may help prevent cancer. - The following are risk factors for oral cavity cancer and oropharyngeal cancer: - Tobacco use - Alcohol use - Tobacco and alcohol use - Betel quid or gutka chewing - Personal history of head and neck cancer - The following is a risk factor for oropharyngeal cancer: - HPV infection - The following is a protective factor for oral cavity cancer and oropharyngeal cancer: - Quitting smoking - It is not clear whether avoiding certain risk factors will decrease the risk of oral cavity cancer or oropharyngeal cancer. - Cancer prevention clinical trials are used to study ways to prevent cancer. - New ways to prevent oral cavity cancer and oropharyngeal cancer are being studied in clinical trials. Avoiding risk factors and increasing protective factors may help prevent cancer. Avoiding cancer risk factors may help prevent certain cancers. Risk factors include smoking, being overweight, and not getting enough exercise. Increasing protective factors such as quitting smoking and exercising may also help prevent some cancers. Talk to your doctor or other health care professional about how you might lower your risk of cancer. Oral cavity cancer and oropharyngeal cancer are two different diseases, but they have some risk factors in common. The following are risk factors for oral cavity cancer and oropharyngeal cancer: Tobacco use Using tobacco is the most common cause of oral cavity cancer and oropharyngeal cancer. The risk of these cancers is about 5 to 10 times higher for current smokers than for people who have never smoked. The use of all types of tobacco, including cigarettes, pipes, cigars, and smokeless tobacco (snuff and chewing tobacco) can cause cancer of the oral cavity and oropharynx. For cigarette smokers, the risk of oral cavity cancer and oropharyngeal cancer increases with the number of cigarettes smoked per day. Alcohol use Using alcohol is also an important risk factor for oral cavity cancer and oropharyngeal cancer. The risk of oral cavity cancer and oropharyngeal cancer increases with the number of alcoholic drinks consumed per day. The risk of oral cavity cancer and oropharyngeal cancer is about twice as high in people who have 3 to 4 alcoholic drinks per day and 5 times higher in people who have 5 or more alcoholic drinks per day compared with those who don't drink alcohol. Tobacco and alcohol use The risk of oral cavity cancer and oropharyngeal cancer is 2 to 3 times higher in people who use both tobacco and alcohol than it is in people who use only tobacco or only alcohol. The risk of oral cavity cancer and oropharyngeal cancer is about 35 times higher in people who smoke 2 or more packs of cigarettes per day and have more than 4 alcoholic drinks per day than it is in people who have never smoked cigarettes or consumed alcohol. Betel quid or gutka chewing Chewing betel quid or gutka (betel quid mixed with tobacco) has been shown to increase the risk of oral cavity cancer and oropharyngeal cancer. Betel quid contains areca nut, which is a cancer-causing substance. The risk of oral cavity cancer and oropharyngeal cancer increases with how long and how often betel quid or gutka are chewed. The risk for oral cavity cancer and oropharyngeal cancer is higher when chewing gutka than when chewing betel quid alone. Betel quid and gutka chewing is common in many countries in South Asia and Southeast Asia, including China and India. Personal history of head and neck cancer A personal history of head and neck cancer increases the risk of oral cavity cancer and oropharyngeal cancer. The following is a risk factor for oropharyngeal cancer: HPV infection Being infected with certain types of HPV, especially HPV type 16, increases the risk of oropharyngeal cancer. HPV infection is spread mainly through sexual contact. The risk of oropharyngeal cancer is about 15 times higher in people who have oral HPV 16 infection compared with people who do not have oral HPV 16 infection. The following is a protective factor for oral cavity cancer and oropharyngeal cancer: Quitting smoking Studies have shown that when people stop smoking cigarettes, their risk of oral cavity cancer and oropharyngeal cancer decreases by one half (50%) within 5 years. Within 20 years of quitting, their risk of oral cavity cancer and oropharyngeal cancer is the same as for a person who never smoked cigarettes. It is not clear whether avoiding certain risk factors will decrease the risk of oral cavity cancer or oropharyngeal cancer. It has not been proven that stopping alcohol use will decrease the risk of oral cavity cancer or oropharyngeal cancer. Getting an HPV vaccination greatly lessens the risk of oral HPV infection. It is not yet known whether getting an HPV vaccination at any age will decrease the risk of oropharyngeal cancer from HPV infection. Cancer prevention clinical trials are used to study ways to prevent cancer. Cancer prevention clinical trials are used to study ways to lower the risk of certain types of cancer. Some cancer prevention trials are done with healthy people who have not had cancer but who have an increased risk for cancer. Other prevention trials are done with people who have had cancer and are trying to prevent another cancer of the same type or to lower their chance of developing a new type of cancer. Other trials are done with healthy volunteers who are not known to have any risk factors for cancer. The purpose of some cancer prevention clinical trials is to find out whether actions people take can prevent cancer. These may include eating fruits and vegetables, exercising, quitting smoking, or taking certain medicines, vitamins, minerals, or food supplements. New ways to prevent oral cavity cancer and oropharyngeal cancer are being studied in clinical trials. Clinical trials are taking place in many parts of the country. Information about clinical trials can be found in the Clinical Trials section of the NCI website. Check NCI's list of cancer clinical trials for oral cavity cancer prevention trials and oropharyngeal cancer prevention trials that are now accepting patients.",Oral Cavity and Oropharyngeal Cancer,0000024_9,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/oral-prevention-pdq,C0153382,T191,Disorders Who is at risk for Oral Cavity and Oropharyngeal Cancer? ?,0000024_9-2,susceptibility,"Key Points - Avoiding risk factors and increasing protective factors may help prevent cancer. - The following are risk factors for oral cavity cancer and oropharyngeal cancer: - Tobacco use - Alcohol use - Tobacco and alcohol use - Betel quid or gutka chewing - Personal history of head and neck cancer - The following is a risk factor for oropharyngeal cancer: - HPV infection - The following is a protective factor for oral cavity cancer and oropharyngeal cancer: - Quitting smoking - It is not clear whether avoiding certain risk factors will decrease the risk of oral cavity cancer or oropharyngeal cancer. - Cancer prevention clinical trials are used to study ways to prevent cancer. - New ways to prevent oral cavity cancer and oropharyngeal cancer are being studied in clinical trials. Avoiding risk factors and increasing protective factors may help prevent cancer. Avoiding cancer risk factors may help prevent certain cancers. Risk factors include smoking, being overweight, and not getting enough exercise. Increasing protective factors such as quitting smoking and exercising may also help prevent some cancers. Talk to your doctor or other health care professional about how you might lower your risk of cancer. Oral cavity cancer and oropharyngeal cancer are two different diseases, but they have some risk factors in common. The following are risk factors for oral cavity cancer and oropharyngeal cancer: Tobacco use Using tobacco is the most common cause of oral cavity cancer and oropharyngeal cancer. The risk of these cancers is about 5 to 10 times higher for current smokers than for people who have never smoked. The use of all types of tobacco, including cigarettes, pipes, cigars, and smokeless tobacco (snuff and chewing tobacco) can cause cancer of the oral cavity and oropharynx. For cigarette smokers, the risk of oral cavity cancer and oropharyngeal cancer increases with the number of cigarettes smoked per day. Alcohol use Using alcohol is also an important risk factor for oral cavity cancer and oropharyngeal cancer. The risk of oral cavity cancer and oropharyngeal cancer increases with the number of alcoholic drinks consumed per day. The risk of oral cavity cancer and oropharyngeal cancer is about twice as high in people who have 3 to 4 alcoholic drinks per day and 5 times higher in people who have 5 or more alcoholic drinks per day compared with those who don't drink alcohol. Tobacco and alcohol use The risk of oral cavity cancer and oropharyngeal cancer is 2 to 3 times higher in people who use both tobacco and alcohol than it is in people who use only tobacco or only alcohol. The risk of oral cavity cancer and oropharyngeal cancer is about 35 times higher in people who smoke 2 or more packs of cigarettes per day and have more than 4 alcoholic drinks per day than it is in people who have never smoked cigarettes or consumed alcohol. Betel quid or gutka chewing Chewing betel quid or gutka (betel quid mixed with tobacco) has been shown to increase the risk of oral cavity cancer and oropharyngeal cancer. Betel quid contains areca nut, which is a cancer-causing substance. The risk of oral cavity cancer and oropharyngeal cancer increases with how long and how often betel quid or gutka are chewed. The risk for oral cavity cancer and oropharyngeal cancer is higher when chewing gutka than when chewing betel quid alone. Betel quid and gutka chewing is common in many countries in South Asia and Southeast Asia, including China and India. Personal history of head and neck cancer A personal history of head and neck cancer increases the risk of oral cavity cancer and oropharyngeal cancer. The following is a risk factor for oropharyngeal cancer: HPV infection Being infected with certain types of HPV, especially HPV type 16, increases the risk of oropharyngeal cancer. HPV infection is spread mainly through sexual contact. The risk of oropharyngeal cancer is about 15 times higher in people who have oral HPV 16 infection compared with people who do not have oral HPV 16 infection. It is not clear whether avoiding certain risk factors will decrease the risk of oral cavity cancer or oropharyngeal cancer. It has not been proven that stopping alcohol use will decrease the risk of oral cavity cancer or oropharyngeal cancer. Getting an HPV vaccination greatly lessens the risk of oral HPV infection. It is not yet known whether getting an HPV vaccination at any age will decrease the risk of oropharyngeal cancer from HPV infection.",Oral Cavity and Oropharyngeal Cancer,0000024_9,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/oral-prevention-pdq,C0153382,T191,Disorders what research (or clinical trials) is being done for Oral Cavity and Oropharyngeal Cancer ?,0000024_9-3,research,"Cancer prevention clinical trials are used to study ways to prevent cancer. Cancer prevention clinical trials are used to study ways to lower the risk of certain types of cancer. Some cancer prevention trials are done with healthy people who have not had cancer but who have an increased risk for cancer. Other prevention trials are done with people who have had cancer and are trying to prevent another cancer of the same type or to lower their chance of developing a new type of cancer. Other trials are done with healthy volunteers who are not known to have any risk factors for cancer. The purpose of some cancer prevention clinical trials is to find out whether actions people take can prevent cancer. These may include eating fruits and vegetables, exercising, quitting smoking, or taking certain medicines, vitamins, minerals, or food supplements. New ways to prevent oral cavity cancer and oropharyngeal cancer are being studied in clinical trials. Clinical trials are taking place in many parts of the country. Information about clinical trials can be found in the Clinical Trials section of the NCI website. Check NCI's list of cancer clinical trials for oral cavity cancer prevention trials and oropharyngeal cancer prevention trials that are now accepting patients.",Oral Cavity and Oropharyngeal Cancer,0000024_9,CancerGov,https://www.cancer.gov/types/head-and-neck/patient/oral-prevention-pdq,C0153382,T191,Disorders What is (are) Pancreatic Cancer ?,0000025_1-1,information,"Key Points - Pancreatic cancer is a disease in which malignant (cancer) cells form in the tissues of the pancreas. - Smoking and health history can affect the risk of pancreatic cancer. - Signs and symptoms of pancreatic cancer include jaundice, pain, and weight loss. - Pancreatic cancer is difficult to detect (find) and diagnose early. - Tests that examine the pancreas are used to detect (find), diagnose, and stage pancreatic cancer. - Certain factors affect prognosis (chance of recovery) and treatment options. Pancreatic cancer is a disease in which malignant (cancer) cells form in the tissues of the pancreas. The pancreas is a gland about 6 inches long that is shaped like a thin pear lying on its side. The wider end of the pancreas is called the head, the middle section is called the body, and the narrow end is called the tail. The pancreas lies between the stomach and the spine. The pancreas has two main jobs in the body: - To make juices that help digest (break down) food. - To make hormones, such as insulin and glucagon, that help control blood sugar levels. Both of these hormones help the body use and store the energy it gets from food. The digestive juices are made by exocrine pancreas cells and the hormones are made by endocrine pancreas cells. About 95% of pancreatic cancers begin in exocrine cells. This summary is about exocrine pancreatic cancer. For information on endocrine pancreatic cancer, see the PDQ summary on Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment. For information on pancreatic cancer in children, see the PDQ summary on Unusual Cancers of Childhood Treatment.",Pancreatic Cancer,0000025_1,CancerGov,https://www.cancer.gov/types/pancreatic/patient/pancreatic-treatment-pdq,C0346647,T191,Disorders Who is at risk for Pancreatic Cancer? ?,0000025_1-2,susceptibility,"Smoking and health history can affect the risk of pancreatic cancer. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesnt mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for pancreatic cancer include the following: - Smoking. - Being very overweight. - Having a personal history of diabetes or chronic pancreatitis. - Having a family history of pancreatic cancer or pancreatitis. - Having certain hereditary conditions, such as: - Multiple endocrine neoplasia type 1 (MEN1) syndrome. - Hereditary nonpolyposis colon cancer (HNPCC; Lynch syndrome). - von Hippel-Lindau syndrome. - Peutz-Jeghers syndrome. - Hereditary breast and ovarian cancer syndrome. - Familial atypical multiple mole melanoma (FAMMM) syndrome.",Pancreatic Cancer,0000025_1,CancerGov,https://www.cancer.gov/types/pancreatic/patient/pancreatic-treatment-pdq,C0346647,T191,Disorders What are the symptoms of Pancreatic Cancer ?,0000025_1-3,symptoms,"Signs and symptoms of pancreatic cancer include jaundice, pain, and weight loss. Pancreatic cancer may not cause early signs or symptoms. Signs and symptoms may be caused by pancreatic cancer or by other conditions. Check with your doctor if you have any of the following: - Jaundice (yellowing of the skin and whites of the eyes). - Light-colored stools. - Dark urine. - Pain in the upper or middle abdomen and back. - Weight loss for no known reason. - Loss of appetite. - Feeling very tired. Pancreatic cancer is difficult to detect (find) and diagnose early. Pancreatic cancer is difficult to detect and diagnose for the following reasons: - There arent any noticeable signs or symptoms in the early stages of pancreatic cancer. - The signs and symptoms of pancreatic cancer, when present, are like the signs and symptoms of many other illnesses. - The pancreas is hidden behind other organs such as the stomach, small intestine, liver, gallbladder, spleen, and bile ducts.",Pancreatic Cancer,0000025_1,CancerGov,https://www.cancer.gov/types/pancreatic/patient/pancreatic-treatment-pdq,C0346647,T191,Disorders How to diagnose Pancreatic Cancer ?,0000025_1-4,exams and tests,"Tests that examine the pancreas are used to detect (find), diagnose, and stage pancreatic cancer. Pancreatic cancer is usually diagnosed with tests and procedures that make pictures of the pancreas and the area around it. The process used to find out if cancer cells have spread within and around the pancreas is called staging. Tests and procedures to detect, diagnose, and stage pancreatic cancer are usually done at the same time. In order to plan treatment, it is important to know the stage of the disease and whether or not the pancreatic cancer can be removed by surgery. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances, such as bilirubin, released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. - Tumor marker test : A procedure in which a sample of blood, urine, or tissue is checked to measure the amounts of certain substances, such as CA 19-9, and carcinoembryonic antigen (CEA), made by organs, tissues, or tumor cells in the body. Certain substances are linked to specific types of cancer when found in increased levels in the body. These are called tumor markers. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. A spiral or helical CT scan makes a series of very detailed pictures of areas inside the body using an x-ray machine that scans the body in a spiral path. - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. A PET scan and CT scan may be done at the same time. This is called a PET-CT. - Abdominal ultrasound : An ultrasound exam used to make pictures of the inside of the abdomen. The ultrasound transducer is pressed against the skin of the abdomen and directs high-energy sound waves (ultrasound) into the abdomen. The sound waves bounce off the internal tissues and organs and make echoes. The transducer receives the echoes and sends them to a computer, which uses the echoes to make pictures called sonograms. The picture can be printed to be looked at later. - Endoscopic ultrasound (EUS): A procedure in which an endoscope is inserted into the body, usually through the mouth or rectum. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. A probe at the end of the endoscope is used to bounce high-energy sound waves (ultrasound) off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. This procedure is also called endosonography. - Endoscopic retrograde cholangiopancreatography (ERCP): A procedure used to x-ray the ducts (tubes) that carry bile from the liver to the gallbladder and from the gallbladder to the small intestine. Sometimes pancreatic cancer causes these ducts to narrow and block or slow the flow of bile, causing jaundice. An endoscope (a thin, lighted tube) is passed through the mouth, esophagus, and stomach into the first part of the small intestine. A catheter (a smaller tube) is then inserted through the endoscope into the pancreatic ducts. A dye is injected through the catheter into the ducts and an x-ray is taken. If the ducts are blocked by a tumor, a fine tube may be inserted into the duct to unblock it. This tube (or stent) may be left in place to keep the duct open. Tissue samples may also be taken. - Percutaneous transhepatic cholangiography (PTC): A procedure used to x-ray the liver and bile ducts. A thin needle is inserted through the skin below the ribs and into the liver. Dye is injected into the liver or bile ducts and an x-ray is taken. If a blockage is found, a thin, flexible tube called a stent is sometimes left in the liver to drain bile into the small intestine or a collection bag outside the body. This test is done only if ERCP cannot be done. - Laparoscopy : A surgical procedure to look at the organs inside the abdomen to check for signs of disease. Small incisions (cuts) are made in the wall of the abdomen and a laparoscope (a thin, lighted tube) is inserted into one of the incisions. The laparoscope may have an ultrasound probe at the end in order to bounce high-energy sound waves off internal organs, such as the pancreas. This is called laparoscopic ultrasound. Other instruments may be inserted through the same or other incisions to perform procedures such as taking tissue samples from the pancreas or a sample of fluid from the abdomen to check for cancer. - Biopsy: The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. There are several ways to do a biopsy for pancreatic cancer. A fine needle or a core needle may be inserted into the pancreas during an x-ray or ultrasound to remove cells. Tissue may also be removed during a laparoscopy.",Pancreatic Cancer,0000025_1,CancerGov,https://www.cancer.gov/types/pancreatic/patient/pancreatic-treatment-pdq,C0346647,T191,Disorders What is the outlook for Pancreatic Cancer ?,0000025_1-5,outlook,"Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - Whether or not the tumor can be removed by surgery. - The stage of the cancer (the size of the tumor and whether the cancer has spread outside the pancreas to nearby tissues or lymph nodes or to other places in the body). - The patients general health. - Whether the cancer has just been diagnosed or has recurred (come back). Pancreatic cancer can be controlled only if it is found before it has spread, when it can be completely removed by surgery. If the cancer has spread, palliative treatment can improve the patient's quality of life by controlling the symptoms and complications of this disease.",Pancreatic Cancer,0000025_1,CancerGov,https://www.cancer.gov/types/pancreatic/patient/pancreatic-treatment-pdq,C0346647,T191,Disorders What are the stages of Pancreatic Cancer ?,0000025_1-6,stages,"Key Points - Tests and procedures to stage pancreatic cancer are usually done at the same time as diagnosis. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - The following stages are used for pancreatic cancer: - Stage 0 (Carcinoma in Situ) - Stage I - Stage II - Stage III - Stage IV Tests and procedures to stage pancreatic cancer are usually done at the same time as diagnosis. The process used to find out if cancer has spread within the pancreas or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage of the disease in order to plan treatment. The results of some of the tests used to diagnose pancreatic cancer are often also used to stage the disease. See the General Information section for more information. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if pancreatic cancer spreads to the liver, the cancer cells in the liver are actually pancreatic cancer cells. The disease is metastatic pancreatic cancer, not liver cancer. The following stages are used for pancreatic cancer: Stage 0 (Carcinoma in Situ) In stage 0, abnormal cells are found in the lining of the pancreas. These abnormal cells may become cancer and spread into nearby normal tissue. Stage 0 is also called carcinoma in situ. Stage I In stage I, cancer has formed and is found in the pancreas only. Stage I is divided into stage IA and stage IB, based on the size of the tumor. - Stage IA: The tumor is 2 centimeters or smaller. - Stage IB: The tumor is larger than 2 centimeters. Stage II In stage II, cancer may have spread to nearby tissue and organs, and may have spread to lymph nodes near the pancreas. Stage II is divided into stage IIA and stage IIB, based on where the cancer has spread. - Stage IIA: Cancer has spread to nearby tissue and organs but has not spread to nearby lymph nodes. - Stage IIB: Cancer has spread to nearby lymph nodes and may have spread to nearby tissue and organs. Stage III In stage III, cancer has spread to the major blood vessels near the pancreas and may have spread to nearby lymph nodes. Stage IV In stage IV, cancer may be of any size and has spread to distant organs, such as the liver, lung, and peritoneal cavity. It may have also spread to organs and tissues near the pancreas or to lymph nodes.",Pancreatic Cancer,0000025_1,CancerGov,https://www.cancer.gov/types/pancreatic/patient/pancreatic-treatment-pdq,C0346647,T191,Disorders What are the treatments for Pancreatic Cancer ?,0000025_1-7,treatment,"Key Points - There are different types of treatment for patients with pancreatic cancer. - Five types of standard treatment are used: - Surgery - Radiation therapy - Chemotherapy - Chemoradiation therapy - Targeted therapy - There are treatments for pain caused by pancreatic cancer. - Patients with pancreatic cancer have special nutritional needs. - New types of treatment are being tested in clinical trials. - Biologic therapy - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed There are different types of treatment for patients with pancreatic cancer. Different types of treatment are available for patients with pancreatic cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Five types of standard treatment are used: Surgery One of the following types of surgery may be used to take out the tumor: - Whipple procedure: A surgical procedure in which the head of the pancreas, the gallbladder, part of the stomach, part of the small intestine, and the bile duct are removed. Enough of the pancreas is left to produce digestive juices and insulin. - Total pancreatectomy: This operation removes the whole pancreas, part of the stomach, part of the small intestine, the common bile duct, the gallbladder, the spleen, and nearby lymph nodes. - Distal pancreatectomy: The body and the tail of the pancreas and usually the spleen are removed. If the cancer has spread and cannot be removed, the following types of palliative surgery may be done to relieve symptoms and improve quality of life: - Surgical biliary bypass: If cancer is blocking the small intestine and bile is building up in the gallbladder, a biliary bypass may be done. During this operation, the doctor will cut the gallbladder or bile duct and sew it to the small intestine to create a new pathway around the blocked area. - Endoscopic stent placement: If the tumor is blocking the bile duct, surgery may be done to put in a stent (a thin tube) to drain bile that has built up in the area. The doctor may place the stent through a catheter that drains to the outside of the body or the stent may go around the blocked area and drain the bile into the small intestine. - Gastric bypass: If the tumor is blocking the flow of food from the stomach, the stomach may be sewn directly to the small intestine so the patient can continue to eat normally. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External radiation therapy is used to treat pancreatic cancer. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is treatment using more than one anticancer drug. The way the chemotherapy is given depends on the type and stage of the cancer being treated. See Drugs Approved for Pancreatic Cancer for more information. Chemoradiation therapy Chemoradiation therapy combines chemotherapy and radiation therapy to increase the effects of both. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Tyrosine kinase inhibitors (TKIs) are targeted therapy drugs that block signals needed for tumors to grow. Erlotinib is a type of TKI used to treat pancreatic cancer. See Drugs Approved for Pancreatic Cancer for more information. There are treatments for pain caused by pancreatic cancer. Pain can occur when the tumor presses on nerves or other organs near the pancreas. When pain medicine is not enough, there are treatments that act on nerves in the abdomen to relieve the pain. The doctor may inject medicine into the area around affected nerves or may cut the nerves to block the feeling of pain. Radiation therapy with or without chemotherapy can also help relieve pain by shrinking the tumor. See the PDQ summary on Cancer Pain for more information. Patients with pancreatic cancer have special nutritional needs. Surgery to remove the pancreas may affect its ability to make pancreatic enzymes that help to digest food. As a result, patients may have problems digesting food and absorbing nutrients into the body. To prevent malnutrition, the doctor may prescribe medicines that replace these enzymes. See the PDQ summary on Nutrition in Cancer Care for more information. New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Biologic therapy Biologic therapy is a treatment that uses the patients immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the bodys natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options by Stage Stages I and II Pancreatic Cancer Treatment of stage I and stage II pancreatic cancer may include the following: - Surgery. - Surgery followed by chemotherapy. - Surgery followed by chemoradiation. - A clinical trial of combination chemotherapy. - A clinical trial of chemotherapy and targeted therapy, with or without chemoradiation. - A clinical trial of chemotherapy and/or radiation therapy before surgery. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I pancreatic cancer and stage II pancreatic cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage III Pancreatic Cancer Treatment of stage III pancreatic cancer may include the following: - Palliative surgery or stent placement to bypass blocked areas in ducts or the small intestine. - Chemotherapy followed by chemoradiation. - Chemoradiation followed by chemotherapy. - Chemotherapy with or without targeted therapy. - A clinical trial of new anticancer therapies together with chemotherapy or chemoradiation. - A clinical trial of radiation therapy given during surgery or internal radiation therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III pancreatic cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage IV Pancreatic Cancer Treatment of stage IV pancreatic cancer may include the following: - Palliative treatments to relieve pain, such as nerve blocks, and other supportive care. - Palliative surgery or stent placement to bypass blocked areas in ducts or the small intestine. - Chemotherapy with or without targeted therapy. - Clinical trials of new anticancer agents with or without chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage IV pancreatic cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Pancreatic Cancer,0000025_1,CancerGov,https://www.cancer.gov/types/pancreatic/patient/pancreatic-treatment-pdq,C0346647,T191,Disorders what research (or clinical trials) is being done for Pancreatic Cancer ?,0000025_1-8,research,"New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Biologic therapy Biologic therapy is a treatment that uses the patients immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the bodys natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Pancreatic Cancer,0000025_1,CancerGov,https://www.cancer.gov/types/pancreatic/patient/pancreatic-treatment-pdq,C0346647,T191,Disorders What is (are) Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) ?,0000025_2-1,information,"Key Points - Pancreatic neuroendocrine tumors form in hormone-making cells (islet cells) of the pancreas. - Pancreatic NETs may or may not cause signs or symptoms. - There are different kinds of functional pancreatic NETs. - Having certain syndromes can increase the risk of pancreatic NETs. - Different types of pancreatic NETs have different signs and symptoms. - Lab tests and imaging tests are used to detect (find) and diagnose pancreatic NETs. - Other kinds of lab tests are used to check for the specific type of pancreatic NETs. - Certain factors affect prognosis (chance of recovery) and treatment options. Pancreatic neuroendocrine tumors form in hormone-making cells (islet cells) of the pancreas. The pancreas is a gland about 6 inches long that is shaped like a thin pear lying on its side. The wider end of the pancreas is called the head, the middle section is called the body, and the narrow end is called the tail. The pancreas lies behind the stomach and in front of the spine. There are two kinds of cells in the pancreas: - Endocrine pancreas cells make several kinds of hormones (chemicals that control the actions of certain cells or organs in the body), such as insulin to control blood sugar. They cluster together in many small groups (islets) throughout the pancreas. Endocrine pancreas cells are also called islet cells or islets of Langerhans. Tumors that form in islet cells are called islet cell tumors, pancreatic endocrine tumors, or pancreatic neuroendocrine tumors (pancreatic NETs). - Exocrine pancreas cells make enzymes that are released into the small intestine to help the body digest food. Most of the pancreas is made of ducts with small sacs at the end of the ducts, which are lined with exocrine cells. This summary discusses islet cell tumors of the endocrine pancreas. See the PDQ summary on Pancreatic Cancer Treatment for information on exocrine pancreatic cancer. Pancreatic neuroendocrine tumors (NETs) may be benign (not cancer) or malignant (cancer). When pancreatic NETs are malignant, they are called pancreatic endocrine cancer or islet cell carcinoma. Pancreatic NETs are much less common than pancreatic exocrine tumors and have a better prognosis. There are different kinds of functional pancreatic NETs. Pancreatic NETs make different kinds of hormones such as gastrin, insulin, and glucagon. Functional pancreatic NETs include the following: - Gastrinoma: A tumor that forms in cells that make gastrin. Gastrin is a hormone that causes the stomach to release an acid that helps digest food. Both gastrin and stomach acid are increased by gastrinomas. When increased stomach acid, stomach ulcers, and diarrhea are caused by a tumor that makes gastrin, it is called Zollinger-Ellison syndrome. A gastrinoma usually forms in the head of the pancreas and sometimes forms in the small intestine. Most gastrinomas are malignant (cancer). - Insulinoma: A tumor that forms in cells that make insulin. Insulin is a hormone that controls the amount of glucose (sugar) in the blood. It moves glucose into the cells, where it can be used by the body for energy. Insulinomas are usually slow-growing tumors that rarely spread. An insulinoma forms in the head, body, or tail of the pancreas. Insulinomas are usually benign (not cancer). - Glucagonoma: A tumor that forms in cells that make glucagon. Glucagon is a hormone that increases the amount of glucose in the blood. It causes the liver to break down glycogen. Too much glucagon causes hyperglycemia (high blood sugar). A glucagonoma usually forms in the tail of the pancreas. Most glucagonomas are malignant (cancer). - Other types of tumors: There are other rare types of functional pancreatic NETs that make hormones, including hormones that control the balance of sugar, salt, and water in the body. These tumors include: - VIPomas, which make vasoactive intestinal peptide. VIPoma may also be called Verner-Morrison syndrome. - Somatostatinomas, which make somatostatin. These other types of tumors are grouped together because they are treated in much the same way.",Pancreatic Neuroendocrine Tumors (Islet Cell Tumors),0000025_2,CancerGov,https://www.cancer.gov/types/pancreatic/patient/pnet-treatment-pdq,C0242363,T191,Disorders Who is at risk for Pancreatic Neuroendocrine Tumors (Islet Cell Tumors)? ?,0000025_2-2,susceptibility,Having certain syndromes can increase the risk of pancreatic NETs. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Multiple endocrine neoplasia type 1 (MEN1) syndrome is a risk factor for pancreatic NETs.,Pancreatic Neuroendocrine Tumors (Islet Cell Tumors),0000025_2,CancerGov,https://www.cancer.gov/types/pancreatic/patient/pnet-treatment-pdq,C0242363,T191,Disorders How to diagnose Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) ?,0000025_2-4,exams and tests,"Lab tests and imaging tests are used to detect (find) and diagnose pancreatic NETs. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances, such as glucose (sugar), released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. - Chromogranin A test: A test in which a blood sample is checked to measure the amount of chromogranin A in the blood. A higher than normal amount of chromogranin A and normal amounts of hormones such as gastrin, insulin, and glucagon can be a sign of a non-functional pancreatic NET. - Abdominal CT scan (CAT scan): A procedure that makes a series of detailed pictures of the abdomen, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Somatostatin receptor scintigraphy : A type of radionuclide scan that may be used to find small pancreatic NETs. A small amount of radioactive octreotide (a hormone that attaches to tumors) is injected into a vein and travels through the blood. The radioactive octreotide attaches to the tumor and a special camera that detects radioactivity is used to show where the tumors are in the body. This procedure is also called octreotide scan and SRS. - Endoscopic ultrasound (EUS): A procedure in which an endoscope is inserted into the body, usually through the mouth or rectum. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. A probe at the end of the endoscope is used to bounce high-energy sound waves (ultrasound) off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. This procedure is also called endosonography. - Endoscopic retrograde cholangiopancreatography (ERCP): A procedure used to x-ray the ducts (tubes) that carry bile from the liver to the gallbladder and from the gallbladder to the small intestine. Sometimes pancreatic cancer causes these ducts to narrow and block or slow the flow of bile, causing jaundice. An endoscope is passed through the mouth, esophagus, and stomach into the first part of the small intestine. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. A catheter (a smaller tube) is then inserted through the endoscope into the pancreatic ducts. A dye is injected through the catheter into the ducts and an x-ray is taken. If the ducts are blocked by a tumor, a fine tube may be inserted into the duct to unblock it. This tube (or stent) may be left in place to keep the duct open. Tissue samples may also be taken and checked under a microscope for signs of cancer. - Angiogram : A procedure to look at blood vessels and the flow of blood. A contrast dye is injected into the blood vessel. As the contrast dye moves through the blood vessel, x-rays are taken to see if there are any blockages. - Laparotomy : A surgical procedure in which an incision (cut) is made in the wall of the abdomen to check the inside of the abdomen for signs of disease. The size of the incision depends on the reason the laparotomy is being done. Sometimes organs are removed or tissue samples are taken and checked under a microscope for signs of disease. - Intraoperative ultrasound : A procedure that uses high-energy sound waves (ultrasound) to create images of internal organs or tissues during surgery. A transducer placed directly on the organ or tissue is used to make the sound waves, which create echoes. The transducer receives the echoes and sends them to a computer, which uses the echoes to make pictures called sonograms. - Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. There are several ways to do a biopsy for pancreatic NETs. Cells may be removed using a fine or wide needle inserted into the pancreas during an x-ray or ultrasound. Tissue may also be removed during a laparoscopy (a surgical incision made in the wall of the abdomen). - Bone scan : A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the blood. The radioactive material collects in bones where cancer cells have spread and is detected by a scanner. Other kinds of lab tests are used to check for the specific type of pancreatic NETs. The following tests and procedures may be used: Gastrinoma - Fasting serum gastrin test: A test in which a blood sample is checked to measure the amount of gastrin in the blood. This test is done after the patient has had nothing to eat or drink for at least 8 hours. Conditions other than gastrinoma can cause an increase in the amount of gastrin in the blood. - Basal acid output test: A test to measure the amount of acid made by the stomach. The test is done after the patient has had nothing to eat or drink for at least 8 hours. A tube is inserted through the nose or throat, into the stomach. The stomach contents are removed and four samples of gastric acid are removed through the tube. These samples are used to find out the amount of gastric acid made during the test and the pH level of the gastric secretions. - Secretin stimulation test : If the basal acid output test result is not normal, a secretin stimulation test may be done. The tube is moved into the small intestine and samples are taken from the small intestine after a drug called secretin is injected. Secretin causes the small intestine to make acid. When there is a gastrinoma, the secretin causes an increase in how much gastric acid is made and the level of gastrin in the blood. - Somatostatin receptor scintigraphy: A type of radionuclide scan that may be used to find small pancreatic NETs. A small amount of radioactive octreotide (a hormone that attaches to tumors) is injected into a vein and travels through the blood. The radioactive octreotide attaches to the tumor and a special camera that detects radioactivity is used to show where the tumors are in the body. This procedure is also called octreotide scan and SRS. Insulinoma - Fasting serum glucose and insulin test: A test in which a blood sample is checked to measure the amounts of glucose (sugar) and insulin in the blood. The test is done after the patient has had nothing to eat or drink for at least 24 hours. Glucagonoma - Fasting serum glucagon test: A test in which a blood sample is checked to measure the amount of glucagon in the blood. The test is done after the patient has had nothing to eat or drink for at least 8 hours. Other tumor types - VIPoma - Serum VIP (vasoactive intestinal peptide) test: A test in which a blood sample is checked to measure the amount of VIP. - Blood chemistry studies: A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. In VIPoma, there is a lower than normal amount of potassium. - Stool analysis : A stool sample is checked for a higher than normal sodium (salt) and potassium levels. - Somatostatinoma - Fasting serum somatostatin test: A test in which a blood sample is checked to measure the amount of somatostatin in the blood. The test is done after the patient has had nothing to eat or drink for at least 8 hours. - Somatostatin receptor scintigraphy: A type of radionuclide scan that may be used to find small pancreatic NETs. A small amount of radioactive octreotide (a hormone that attaches to tumors) is injected into a vein and travels through the blood. The radioactive octreotide attaches to the tumor and a special camera that detects radioactivity is used to show where the tumors are in the body. This procedure is also called octreotide scan and SRS.",Pancreatic Neuroendocrine Tumors (Islet Cell Tumors),0000025_2,CancerGov,https://www.cancer.gov/types/pancreatic/patient/pnet-treatment-pdq,C0242363,T191,Disorders What is the outlook for Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) ?,0000025_2-7,outlook,Certain factors affect prognosis (chance of recovery) and treatment options. Pancreatic NETs can often be cured. The prognosis (chance of recovery) and treatment options depend on the following: - The type of cancer cell. - Where the tumor is found in the pancreas. - Whether the tumor has spread to more than one place in the pancreas or to other parts of the body. - Whether the patient has MEN1 syndrome. - The patient's age and general health. - Whether the cancer has just been diagnosed or has recurred (come back).,Pancreatic Neuroendocrine Tumors (Islet Cell Tumors),0000025_2,CancerGov,https://www.cancer.gov/types/pancreatic/patient/pnet-treatment-pdq,C0242363,T191,Disorders What are the stages of Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) ?,0000025_2-8,stages,"Key Points - The plan for cancer treatment depends on where the NET is found in the pancreas and whether it has spread. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. The plan for cancer treatment depends on where the NET is found in the pancreas and whether it has spread. The process used to find out if cancer has spread within the pancreas or to other parts of the body is called staging. The results of the tests and procedures used to diagnose pancreatic neuroendocrine tumors (NETs) are also used to find out whether the cancer has spread. See the General Information section for a description of these tests and procedures. Although there is a standard staging system for pancreatic NETs, it is not used to plan treatment. Treatment of pancreatic NETs is based on the following: - Whether the cancer is found in one place in the pancreas. - Whether the cancer is found in several places in the pancreas. - Whether the cancer has spread to lymph nodes near the pancreas or to other parts of the body such as the liver, lung, peritoneum, or bone. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of tumor as the primary tumor. For example, if a pancreatic neuroendocrine tumor spreads to the liver, the tumor cells in the liver are actually neuroendocrine tumor cells. The disease is metastatic pancreatic neuroendocrine tumor, not liver cancer.",Pancreatic Neuroendocrine Tumors (Islet Cell Tumors),0000025_2,CancerGov,https://www.cancer.gov/types/pancreatic/patient/pnet-treatment-pdq,C0242363,T191,Disorders What are the treatments for Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) ?,0000025_2-9,treatment,"Key Points - There are different types of treatment for patients with pancreatic NETs. - Six types of standard treatment are used: - Surgery - Chemotherapy - Hormone therapy - Hepatic arterial occlusion or chemoembolization - Targeted therapy - Supportive care - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with pancreatic NETs. Different types of treatments are available for patients with pancreatic neuroendocrine tumors (NETs). Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Six types of standard treatment are used: Surgery An operation may be done to remove the tumor. One of the following types of surgery may be used: - Enucleation: Surgery to remove the tumor only. This may be done when cancer occurs in one place in the pancreas. - Pancreatoduodenectomy: A surgical procedure in which the head of the pancreas, the gallbladder, nearby lymph nodes and part of the stomach, small intestine, and bile duct are removed. Enough of the pancreas is left to make digestive juices and insulin. The organs removed during this procedure depend on the patient's condition. This is also called the Whipple procedure. - Distal pancreatectomy: Surgery to remove the body and tail of the pancreas. The spleen may also be removed. - Total gastrectomy: Surgery to remove the whole stomach. - Parietal cell vagotomy: Surgery to cut the nerve that causes stomach cells to make acid. - Liver resection: Surgery to remove part or all of the liver. - Radiofrequency ablation: The use of a special probe with tiny electrodes that kill cancer cells. Sometimes the probe is inserted directly through the skin and only local anesthesia is needed. In other cases, the probe is inserted through an incision in the abdomen. This is done in the hospital with general anesthesia. - Cryosurgical ablation: A procedure in which tissue is frozen to destroy abnormal cells. This is usually done with a special instrument that contains liquid nitrogen or liquid carbon dioxide. The instrument may be used during surgery or laparoscopy or inserted through the skin. This procedure is also called cryoablation. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is the use of more than one anticancer drug. The way the chemotherapy is given depends on the type of the cancer being treated. Hormone therapy Hormone therapy is a cancer treatment that removes hormones or blocks their action and stops cancer cells from growing. Hormones are substances made by glands in the body and circulated in the bloodstream. Some hormones can cause certain cancers to grow. If tests show that the cancer cells have places where hormones can attach (receptors), drugs, surgery, or radiation therapy is used to reduce the production of hormones or block them from working. Hepatic arterial occlusion or chemoembolization Hepatic arterial occlusion uses drugs, small particles, or other agents to block or reduce the flow of blood to the liver through the hepatic artery (the major blood vessel that carries blood to the liver). This is done to kill cancer cells growing in the liver. The tumor is prevented from getting the oxygen and nutrients it needs to grow. The liver continues to receive blood from the hepatic portal vein, which carries blood from the stomach and intestine. Chemotherapy delivered during hepatic arterial occlusion is called chemoembolization. The anticancer drug is injected into the hepatic artery through a catheter (thin tube). The drug is mixed with the substance that blocks the artery and cuts off blood flow to the tumor. Most of the anticancer drug is trapped near the tumor and only a small amount of the drug reaches other parts of the body. The blockage may be temporary or permanent, depending on the substance used to block the artery. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Certain types of targeted therapies are being studied in the treatment of pancreatic NETs. Supportive care Supportive care is given to lessen the problems caused by the disease or its treatment. Supportive care for pancreatic NETs may include treatment for the following: - Stomach ulcers may be treated with drug therapy such as: - Proton pump inhibitor drugs such as omeprazole, lansoprazole, or pantoprazole. - Histamine blocking drugs such as cimetidine, ranitidine, or famotidine. - Somatostatin-type drugs such as octreotide. - Diarrhea may be treated with: - Intravenous (IV) fluids with electrolytes such as potassium or chloride. - Somatostatin-type drugs such as octreotide. - Low blood sugar may be treated by having small, frequent meals or with drug therapy to maintain a normal blood sugar level. - High blood sugar may be treated with drugs taken by mouth or insulin by injection. New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Pancreatic Neuroendocrine Tumors Gastrinoma Treatment of gastrinoma may include supportive care and the following: - For symptoms caused by too much stomach acid, treatment may be a drug that decreases the amount of acid made by the stomach. - For a single tumor in the head of the pancreas: - Surgery to remove the tumor. - Surgery to cut the nerve that causes stomach cells to make acid and treatment with a drug that decreases stomach acid. - Surgery to remove the whole stomach (rare). - For a single tumor in the body or tail of the pancreas, treatment is usually surgery to remove the body or tail of the pancreas. - For several tumors in the pancreas, treatment is usually surgery to remove the body or tail of the pancreas. If tumor remains after surgery, treatment may include either: - Surgery to cut the nerve that causes stomach cells to make acid and treatment with a drug that decreases stomach acid; or - Surgery to remove the whole stomach (rare). - For one or more tumors in the duodenum (the part of the small intestine that connects to the stomach), treatment is usually pancreatoduodenectomy (surgery to remove the head of the pancreas, the gallbladder, nearby lymph nodes and part of the stomach, small intestine, and bile duct). - If no tumor is found, treatment may include the following: - Surgery to cut the nerve that causes stomach cells to make acid and treatment with a drug that decreases stomach acid. - Surgery to remove the whole stomach (rare). - If the cancer has spread to the liver, treatment may include: - Surgery to remove part or all of the liver. - Radiofrequency ablation or cryosurgical ablation. - Chemoembolization. - If cancer has spread to other parts of the body or does not get better with surgery or drugs to decrease stomach acid, treatment may include: - Chemotherapy. - Hormone therapy. - If the cancer mostly affects the liver and the patient has severe symptoms from hormones or from the size of tumor, treatment may include: - Hepatic arterial occlusion, with or without systemic chemotherapy. - Chemoembolization, with or without systemic chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with gastrinoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Insulinoma Treatment of insulinoma may include the following: - For one small tumor in the head or tail of the pancreas, treatment is usually surgery to remove the tumor. - For one large tumor in the head of the pancreas that cannot be removed by surgery, treatment is usually pancreatoduodenectomy (surgery to remove the head of the pancreas, the gallbladder, nearby lymph nodes and part of the stomach, small intestine, and bile duct). - For one large tumor in the body or tail of the pancreas, treatment is usually a distal pancreatectomy (surgery to remove the body and tail of the pancreas). - For more than one tumor in the pancreas, treatment is usually surgery to remove any tumors in the head of the pancreas and the body and tail of the pancreas. - For tumors that cannot be removed by surgery, treatment may include the following: - Combination chemotherapy. - Palliative drug therapy to decrease the amount of insulin made by the pancreas. - Hormone therapy. - Radiofrequency ablation or cryosurgical ablation. - For cancer that has spread to lymph nodes or other parts of the body, treatment may include the following: - Surgery to remove the cancer. - Radiofrequency ablation or cryosurgical ablation, if the cancer cannot be removed by surgery. - If the cancer mostly affects the liver and the patient has severe symptoms from hormones or from the size of tumor, treatment may include: - Hepatic arterial occlusion, with or without systemic chemotherapy. - Chemoembolization, with or without systemic chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with insulinoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Glucagonoma Treatment may include the following: - For one small tumor in the head or tail of the pancreas, treatment is usually surgery to remove the tumor. - For one large tumor in the head of the pancreas that cannot be removed by surgery, treatment is usually pancreatoduodenectomy (surgery to remove the head of the pancreas, the gallbladder, nearby lymph nodes and part of the stomach, small intestine, and bile duct). - For more than one tumor in the pancreas, treatment is usually surgery to remove the tumor or surgery to remove the body and tail of the pancreas. - For tumors that cannot be removed by surgery, treatment may include the following: - Combination chemotherapy. - Hormone therapy. - Radiofrequency ablation or cryosurgical ablation. - For cancer that has spread to lymph nodes or other parts of the body, treatment may include the following: - Surgery to remove the cancer. - Radiofrequency ablation or cryosurgical ablation, if the cancer cannot be removed by surgery. - If the cancer mostly affects the liver and the patient has severe symptoms from hormones or from the size of tumor, treatment may include: - Hepatic arterial occlusion, with or without systemic chemotherapy. - Chemoembolization, with or without systemic chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with glucagonoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Other Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) For VIPoma, treatment may include the following: - Fluids and hormone therapy to replace fluids and electrolytes that have been lost from the body. - Surgery to remove the tumor and nearby lymph nodes. - Surgery to remove as much of the tumor as possible when the tumor cannot be completely removed or has spread to distant parts of the body. This is palliative therapy to relieve symptoms and improve the quality of life. - For tumors that have spread to lymph nodes or other parts of the body, treatment may include the following: - Surgery to remove the tumor. - Radiofrequency ablation or cryosurgical ablation, if the tumor cannot be removed by surgery. - For tumors that continue to grow during treatment or have spread to other parts of the body, treatment may include the following: - Chemotherapy. - Targeted therapy. For somatostatinoma, treatment may include the following: - Surgery to remove the tumor. - For cancer that has spread to distant parts of the body, surgery to remove as much of the cancer as possible to relieve symptoms and improve quality of life. - For tumors that continue to grow during treatment or have spread to other parts of the body, treatment may include the following: - Chemotherapy. - Targeted therapy. Treatment of other types of pancreatic neuroendocrine tumors (NETs) may include the following: - Surgery to remove the tumor. - For cancer that has spread to distant parts of the body, surgery to remove as much of the cancer as possible or hormone therapy to relieve symptoms and improve quality of life. - For tumors that continue to grow during treatment or have spread to other parts of the body, treatment may include the following: - Chemotherapy. - Targeted therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with islet cell tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Recurrent or Progressive Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment of pancreatic neuroendocrine tumors (NETs) that continue to grow during treatment or recur (come back) may include the following: - Surgery to remove the tumor. - Chemotherapy. - Hormone therapy. - Targeted therapy. - For liver metastases: - Regional chemotherapy. - Hepatic arterial occlusion or chemoembolization, with or without systemic chemotherapy. - A clinical trial of a new therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent islet cell carcinoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Pancreatic Neuroendocrine Tumors (Islet Cell Tumors),0000025_2,CancerGov,https://www.cancer.gov/types/pancreatic/patient/pnet-treatment-pdq,C0242363,T191,Disorders what research (or clinical trials) is being done for Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) ?,0000025_2-10,research,"New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Pancreatic Neuroendocrine Tumors (Islet Cell Tumors),0000025_2,CancerGov,https://www.cancer.gov/types/pancreatic/patient/pnet-treatment-pdq,C0242363,T191,Disorders What is (are) Renal Cell Cancer ?,0000026_1-1,information,"Key Points - Renal cell cancer is a disease in which malignant (cancer) cells form in tubules of the kidney. - Smoking and misuse of certain pain medicines can affect the risk of renal cell cancer. - Signs of renal cell cancer include blood in the urine and a lump in the abdomen. - Tests that examine the abdomen and kidneys are used to detect (find) and diagnose renal cell cancer. - Certain factors affect prognosis (chance of recovery) and treatment options. Renal cell cancer is a disease in which malignant (cancer) cells form in tubules of the kidney. Renal cell cancer (also called kidney cancer or renal adenocarcinoma) is a disease in which malignant (cancer) cells are found in the lining of tubules (very small tubes) in the kidney. There are 2 kidneys, one on each side of the backbone, above the waist. Tiny tubules in the kidneys filter and clean the blood. They take out waste products and make urine. The urine passes from each kidney through a long tube called a ureter into the bladder. The bladder holds the urine until it passes through the urethra and leaves the body. Cancer that starts in the ureters or the renal pelvis (the part of the kidney that collects urine and drains it to the ureters) is different from renal cell cancer. (See the PDQ summary about Transitional Cell Cancer of the Renal Pelvis and Ureter Treatment for more information).",Renal Cell Cancer,0000026_1,CancerGov,https://www.cancer.gov/types/kidney/patient/kidney-treatment-pdq,C0007134,T191,Disorders What are the stages of Renal Cell Cancer ?,0000026_1-6,stages,"Key Points - After renal cell cancer has been diagnosed, tests are done to find out if cancer cells have spread within the kidney or to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - The following stages are used for renal cell cancer: - Stage I - Stage II - Stage III - Stage IV After renal cell cancer has been diagnosed, tests are done to find out if cancer cells have spread within the kidney or to other parts of the body. The process used to find out if cancer has spread within the kidney or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following tests and procedures may be used in the staging process: - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Chest x-ray : An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if renal cell cancer spreads to the bone, the cancer cells in the bone are actually cancerous renal cells. The disease is metastatic renal cell cancer, not bone cancer. The following stages are used for renal cell cancer: Stage I In stage I, the tumor is 7 centimeters or smaller and is found only in the kidney. Stage II In stage II, the tumor is larger than 7 centimeters and is found only in the kidney. Stage III In stage III: - the tumor is any size and cancer is found only in the kidney and in 1 or more nearby lymph nodes; or - cancer is found in the main blood vessels of the kidney or in the layer of fatty tissue around the kidney. Cancer may be found in 1 or more nearby lymph nodes. Stage IV In stage IV, cancer has spread: - beyond the layer of fatty tissue around the kidney and may be found in the adrenal gland above the kidney with cancer, or in nearby lymph nodes; or - to other organs, such as the lungs, liver, bones, or brain, and may have spread to lymph nodes.",Renal Cell Cancer,0000026_1,CancerGov,https://www.cancer.gov/types/kidney/patient/kidney-treatment-pdq,C0007134,T191,Disorders What are the treatments for Renal Cell Cancer ?,0000026_1-7,treatment,"Key Points - There are different types of treatment for patients with renal cell cancer. - Five types of standard treatment are used: - Surgery - Radiation therapy - Chemotherapy - Biologic therapy - Targeted therapy - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with renal cell cancer. Different types of treatments are available for patients with renal cell cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Five types of standard treatment are used: Surgery Surgery to remove part or all of the kidney is often used to treat renal cell cancer. The following types of surgery may be used: - Partial nephrectomy: A surgical procedure to remove the cancer within the kidney and some of the tissue around it. A partial nephrectomy may be done to prevent loss of kidney function when the other kidney is damaged or has already been removed. - Simple nephrectomy: A surgical procedure to remove the kidney only. - Radical nephrectomy: A surgical procedure to remove the kidney, the adrenal gland, surrounding tissue, and, usually, nearby lymph nodes. A person can live with part of 1 working kidney, but if both kidneys are removed or not working, the person will need dialysis (a procedure to clean the blood using a machine outside of the body) or a kidney transplant (replacement with a healthy donated kidney). A kidney transplant may be done when the disease is in the kidney only and a donated kidney can be found. If the patient has to wait for a donated kidney, other treatment is given as needed. When surgery to remove the cancer is not possible, a treatment called arterial embolization may be used to shrink the tumor. A small incision is made and a catheter (thin tube) is inserted into the main blood vessel that flows to the kidney. Small pieces of a special gelatin sponge are injected through the catheter into the blood vessel. The sponges block the blood flow to the kidney and prevent the cancer cells from getting oxygen and other substances they need to grow. Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External radiation therapy is used to treat renal cell cancer, and may also be used as palliative therapy to relieve symptoms and improve quality of life. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. See Drugs Approved for Kidney (Renal Cell) Cancer for more information. Biologic therapy Biologic therapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy. The following types of biologic therapy are being used or studied in the treatment of renal cell cancer: - Nivolumab: Nivolumab is a monoclonal antibody that boosts the bodys immune response against renal cell cancer cells. - Interferon: Interferon affects the division of cancer cells and can slow tumor growth. - Interleukin-2 (IL-2): IL-2 boosts the growth and activity of many immune cells, especially lymphocytes (a type of white blood cell). Lymphocytes can attack and kill cancer cells. See Drugs Approved for Kidney (Renal Cell) Cancer for more information. Targeted therapy Targeted therapy uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Targeted therapy with antiangiogenic agents are used to treat advanced renal cell cancer. Antiangiogenic agents keep blood vessels from forming in a tumor, causing the tumor to starve and stop growing or to shrink. Monoclonal antibodies and kinase inhibitors are two types of antiangiogenic agents used to treat renal cell cancer. Monoclonal antibody therapy uses antibodies made in the laboratory, from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Monoclonal antibodies used to treat renal cell cancer attach to and block substances that cause new blood vessels to form in tumors. Kinase inhibitors stop cells from dividing and may prevent the growth of new blood vessels that tumors need to grow. An mTOR inhibitor is a type of kinase inhibitor. Everolimus and temsirolimus are mTOR inhibitors used to treat advanced renal cell cancer. See Drugs Approved for Kidney (Renal Cell) Cancer for more information. New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Renal Cell Cancer Stage I Renal Cell Cancer Treatment of stage I renal cell cancer may include the following: - Surgery (radical nephrectomy, simple nephrectomy, or partial nephrectomy). - Radiation therapy as palliative therapy to relieve symptoms in patients who cannot have surgery. - Arterial embolization as palliative therapy. - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I renal cell cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage II Renal Cell Cancer Treatment of stage II renal cell cancer may include the following: - Surgery (radical nephrectomy or partial nephrectomy). - Surgery (nephrectomy), before or after radiation therapy. - Radiation therapy as palliative therapy to relieve symptoms in patients who cannot have surgery. - Arterial embolization as palliative therapy. - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage II renal cell cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage III Renal Cell Cancer Treatment of stage III renal cell cancer may include the following: - Surgery (radical nephrectomy). Blood vessels of the kidney and some lymph nodes may also be removed. - Arterial embolization followed by surgery (radical nephrectomy). - Radiation therapy as palliative therapy to relieve symptoms and improve the quality of life. - Arterial embolization as palliative therapy. - Surgery (nephrectomy) as palliative therapy. - Radiation therapy before or after surgery (radical nephrectomy). - A clinical trial of biologic therapy following surgery. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III renal cell cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage IV and Recurrent Renal Cell Cancer Treatment of stage IV and recurrent renal cell cancer may include the following: - Surgery (radical nephrectomy). - Surgery (nephrectomy) to reduce the size of the tumor. - Targeted therapy. - Biologic therapy. - Radiation therapy as palliative therapy to relieve symptoms and improve the quality of life. - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage IV renal cell cancer and recurrent renal cell cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Renal Cell Cancer,0000026_1,CancerGov,https://www.cancer.gov/types/kidney/patient/kidney-treatment-pdq,C0007134,T191,Disorders what research (or clinical trials) is being done for Renal Cell Cancer ?,0000026_1-8,research,"New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Renal Cell Cancer,0000026_1,CancerGov,https://www.cancer.gov/types/kidney/patient/kidney-treatment-pdq,C0007134,T191,Disorders What is (are) Transitional Cell Cancer of the Renal Pelvis and Ureter ?,0000026_2-1,information,"Key Points - Transitional cell cancer of the renal pelvis and ureter is a disease in which malignant (cancer) cells form in the renal pelvis and ureter. - Misuse of certain pain medicines can affect the risk of transitional cell cancer of the renal pelvis and ureter. - Signs and symptoms of transitional cell cancer of the renal pelvis and ureter include blood in the urine and back pain. - Tests that examine the abdomen and kidneys are used to detect (find) and diagnose transitional cell cancer of the renal pelvis and ureter. - Certain factors affect prognosis (chance of recovery) and treatment options. Transitional cell cancer of the renal pelvis and ureter is a disease in which malignant (cancer) cells form in the renal pelvis and ureter. The renal pelvis is the top part of the ureter. The ureter is a long tube that connects the kidney to the bladder. There are two kidneys, one on each side of the backbone, above the waist. The kidneys of an adult are about 5 inches long and 3 inches wide and are shaped like a kidney bean. Tiny tubules in the kidneys filter and clean the blood. They take out waste products and make urine. The urine collects in the middle of each kidney in the renal pelvis. Urine passes from the renal pelvis through the ureter into the bladder. The bladder holds the urine until it passes through the urethra and leaves the body. The renal pelvis and ureters are lined with transitional cells. These cells can change shape and stretch without breaking apart. Transitional cell cancer starts in these cells. Transitional cell cancer can form in the renal pelvis or the ureter or both. Renal cell cancer is a more common type of kidney cancer. See the PDQ summary about Renal Cell Cancer Treatment for more information.",Transitional Cell Cancer of the Renal Pelvis and Ureter,0000026_2,CancerGov,https://www.cancer.gov/types/kidney/patient/transitional-cell-treatment-pdq,C0153618,T191,Disorders What are the symptoms of Transitional Cell Cancer of the Renal Pelvis and Ureter ?,0000026_2-3,symptoms,Signs and symptoms of transitional cell cancer of the renal pelvis and ureter include blood in the urine and back pain. These and other signs and symptoms may be caused by transitional cell cancer of the renal pelvis and ureter or by other conditions. There may be no signs or symptoms in the early stages. Signs and symptoms may appear as the tumor grows. Check with your doctor if you have any of the following: - Blood in the urine. - A pain in the back that doesn't go away. - Extreme tiredness. - Weight loss with no known reason. - Painful or frequent urination.,Transitional Cell Cancer of the Renal Pelvis and Ureter,0000026_2,CancerGov,https://www.cancer.gov/types/kidney/patient/transitional-cell-treatment-pdq,C0153618,T191,Disorders How to diagnose Transitional Cell Cancer of the Renal Pelvis and Ureter ?,0000026_2-4,exams and tests,"Tests that examine the abdomen and kidneys are used to detect (find) and diagnose transitional cell cancer of the renal pelvis and ureter. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Urinalysis : A test to check the color of urine and its contents, such as sugar, protein, blood, and bacteria. - Ureteroscopy : A procedure to look inside the ureter and renal pelvis to check for abnormal areas. A ureteroscope is a thin, tube-like instrument with a light and a lens for viewing. The ureteroscope is inserted through the urethra into the bladder, ureter, and renal pelvis. A tool may be inserted through the ureteroscope to take tissue samples to be checked under a microscope for signs of disease. - Urine cytology : A laboratory test in which a sample of urine is checked under a microscope for abnormal cells. Cancer in the kidney, bladder, or ureter may shed cancer cells into the urine. - Intravenous pyelogram (IVP): A series of x-rays of the kidneys, ureters, and bladder to check for cancer. A contrast dye is injected into a vein. As the contrast dye moves through the kidneys, ureters, and bladder, x-rays are taken to see if there are any blockages. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - Ultrasound : A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. An ultrasound of the abdomen may be done to help diagnose cancer of the renal pelvis and ureter. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body, such as the pelvis. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. This may be done during a ureteroscopy or surgery.",Transitional Cell Cancer of the Renal Pelvis and Ureter,0000026_2,CancerGov,https://www.cancer.gov/types/kidney/patient/transitional-cell-treatment-pdq,C0153618,T191,Disorders What is the outlook for Transitional Cell Cancer of the Renal Pelvis and Ureter ?,0000026_2-5,outlook,Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) depends on the stage and grade of the tumor. The treatment options depend on the following: - The stage and grade of the tumor. - Where the tumor is. - Whether the patient's other kidney is healthy. - Whether the cancer has recurred. Most transitional cell cancer of the renal pelvis and ureter can be cured if found early.,Transitional Cell Cancer of the Renal Pelvis and Ureter,0000026_2,CancerGov,https://www.cancer.gov/types/kidney/patient/transitional-cell-treatment-pdq,C0153618,T191,Disorders What are the stages of Transitional Cell Cancer of the Renal Pelvis and Ureter ?,0000026_2-6,stages,"Key Points - After transitional cell cancer of the renal pelvis and ureter has been diagnosed, tests are done to find out if cancer cells have spread within the renal pelvis and ureter or to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - The following stages are used for transitional cell cancer of the renal pelvis and/or ureter: - Stage 0 (Papillary Carcinoma and Carcinoma in Situ) - Stage I - Stage II - Stage III - Stage IV - Transitional cell cancer of the renal pelvis and ureter is also described as localized, regional, or metastatic: - Localized - Regional - Metastatic After transitional cell cancer of the renal pelvis and ureter has been diagnosed, tests are done to find out if cancer cells have spread within the renal pelvis and ureter or to other parts of the body. The process used to find out if cancer has spread within the renal pelvis and ureter or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following tests and procedures may be used in the staging process: - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - Ureteroscopy : A procedure to look inside the ureter and renal pelvis to check for abnormal areas. A ureteroscope is a thin, tube-like instrument with a light and a lens for viewing. The ureteroscope is inserted through the urethra into the bladder, ureter, and renal pelvis. A tool may be inserted through the ureteroscope to take tissue samples to be checked under a microscope for signs of disease. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if transitional cell cancer of the ureter spreads to the lung, the cancer cells in the lung are actually ureter cancer cells. The disease is metastatic cancer of the ureter, not lung cancer. The following stages are used for transitional cell cancer of the renal pelvis and/or ureter: Stage 0 (Papillary Carcinoma and Carcinoma in Situ) In stage 0, abnormal cells are found in tissue lining the inside of the renal pelvis or ureter. These abnormal cells may become cancer and spread into nearby normal tissue. Stage 0 is divided into stage 0a and stage 0is, depending on the type of tumor: - Stage 0a may look like tiny mushrooms growing from the tissue lining the inside of the renal pelvis or ureter. Stage 0a is also called noninvasive papillary carcinoma. - Stage 0is is a flat tumor on the tissue lining the inside of the renal pelvis or ureter. Stage 0is is also called carcinoma in situ. Stage I In stage I, cancer has formed and spread through the lining of the renal pelvis and/or ureter, into the layer of connective tissue. Stage II In stage II, cancer has spread through the layer of connective tissue to the muscle layer of the renal pelvis and/or ureter. Stage III In stage III, cancer has spread: - From the renal pelvis to tissue or fat in the kidney; or - From the ureter to fat that surrounds the ureter. Stage IV In stage IV, cancer has spread to at least one of the following: - A nearby organ. - The layer of fat surrounding the kidney. - One or more lymph nodes. - Distant parts of the body, such as the lung, liver, or bone. Transitional cell cancer of the renal pelvis and ureter is also described as localized, regional, or metastatic: Localized The cancer is found only in the kidney. Regional The cancer has spread to tissues around the kidney and to nearby lymph nodes and blood vessels in the pelvis. Metastatic The cancer has spread to other parts of the body.",Transitional Cell Cancer of the Renal Pelvis and Ureter,0000026_2,CancerGov,https://www.cancer.gov/types/kidney/patient/transitional-cell-treatment-pdq,C0153618,T191,Disorders What are the treatments for Transitional Cell Cancer of the Renal Pelvis and Ureter ?,0000026_2-7,treatment,"Key Points - There are different types of treatment for patients with transitional cell cancer of the renal pelvis and ureter. - One type of standard treatment is used: - Surgery - New types of treatment are being tested in clinical trials. - Fulguration - Segmental resection of the renal pelvis - Laser surgery - Regional chemotherapy and regional biologic therapy - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with transitional cell cancer of the renal pelvis and ureter. Different types of treatments are available for patients with transitional cell cancer of the renal pelvis and ureter. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. One type of standard treatment is used: Surgery One of the following surgical procedures may be used to treat transitional cell cancer of the renal pelvis and ureter: - Nephroureterectomy: Surgery to remove the entire kidney, the ureter, and the bladder cuff (tissue that connects the ureter to the bladder). - Segmental resection of the ureter: A surgical procedure to remove the part of the ureter that contains cancer and some of the healthy tissue around it. The ends of the ureter are then reattached. This treatment is used when the cancer is superficial and in the lower third of the ureter only, near the bladder. New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI Web site. Fulguration Fulguration is a surgical procedure that destroys tissue using an electric current. A tool with a small wire loop on the end is used to remove the cancer or to burn away the tumor with electricity. Segmental resection of the renal pelvis This is a surgical procedure to remove localized cancer from the renal pelvis without removing the entire kidney. Segmental resection may be done to save kidney function when the other kidney is damaged or has already been removed. Laser surgery A laser beam (narrow beam of intense light) is used as a knife to remove the cancer. A laser beam can also be used to kill the cancer cells. This procedure may also be called or laser fulguration. Regional chemotherapy and regional biologic therapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. Biologic therapy is a treatment that uses the patient's immune system to fight cancer; substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. Regional treatment means the anticancer drugs or biologic substances are placed directly into an organ or a body cavity such as the abdomen, so the drugs will affect cancer cells in that area. Clinical trials are studying chemotherapy or biologic therapy using drugs placed directly into the renal pelvis or the ureter. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Transitional Cell Cancer of the Renal Pelvis and Ureter Localized Transitional Cell Cancer of the Renal Pelvis and Ureter Treatment of localized transitional cell cancer of the renal pelvis and ureter may include the following: - Surgery (nephroureterectomy or segmental resection of ureter). - A clinical trial of fulguration. - A clinical trial of laser surgery. - A clinical trial of segmental resection of the renal pelvis. - A clinical trial of regional chemotherapy. - A clinical trial of regional biologic therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with localized transitional cell cancer of the renal pelvis and ureter. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Regional Transitional Cell Cancer of the Renal Pelvis and Ureter Treatment of regional transitional cell cancer of the renal pelvis and ureter is usually done in a clinical trial. Check the list of NCI-supported cancer clinical trials that are now accepting patients with regional transitional cell cancer of the renal pelvis and ureter. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Metastatic Transitional Cell Cancer of the Renal Pelvis and Ureter Treatment of metastatic transitional cell cancer of the renal pelvis and ureter is usually done in a clinical trial, which may include chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with metastatic transitional cell cancer of the renal pelvis and ureter. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Recurrent Transitional Cell Cancer of the Renal Pelvis and Ureter Treatment of recurrent transitional cell cancer of the renal pelvis and ureter is usually done in a clinical trial, which may include chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent transitional cell cancer of the renal pelvis and ureter. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Transitional Cell Cancer of the Renal Pelvis and Ureter,0000026_2,CancerGov,https://www.cancer.gov/types/kidney/patient/transitional-cell-treatment-pdq,C0153618,T191,Disorders what research (or clinical trials) is being done for Transitional Cell Cancer of the Renal Pelvis and Ureter ?,0000026_2-8,research,"New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI Web site. Fulguration Fulguration is a surgical procedure that destroys tissue using an electric current. A tool with a small wire loop on the end is used to remove the cancer or to burn away the tumor with electricity. Segmental resection of the renal pelvis This is a surgical procedure to remove localized cancer from the renal pelvis without removing the entire kidney. Segmental resection may be done to save kidney function when the other kidney is damaged or has already been removed. Laser surgery A laser beam (narrow beam of intense light) is used as a knife to remove the cancer. A laser beam can also be used to kill the cancer cells. This procedure may also be called or laser fulguration. Regional chemotherapy and regional biologic therapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. Biologic therapy is a treatment that uses the patient's immune system to fight cancer; substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. Regional treatment means the anticancer drugs or biologic substances are placed directly into an organ or a body cavity such as the abdomen, so the drugs will affect cancer cells in that area. Clinical trials are studying chemotherapy or biologic therapy using drugs placed directly into the renal pelvis or the ureter. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Transitional Cell Cancer of the Renal Pelvis and Ureter,0000026_2,CancerGov,https://www.cancer.gov/types/kidney/patient/transitional-cell-treatment-pdq,C0153618,T191,Disorders What is (are) Wilms Tumor and Other Childhood Kidney Tumors ?,0000026_3-1,information,"Key Points - Childhood kidney tumors are diseases in which malignant (cancer) cells form in the tissues of the kidney. - There are many types of childhood kidney tumors. - Wilms Tumor - Renal Cell Cancer (RCC) - Rhabdoid Tumor of the Kidney - Clear Cell Sarcoma of the Kidney - Congenital Mesoblastic Nephroma - Ewing Sarcoma of the Kidney - Primary Renal Myoepithelial Carcinoma - Cystic Partially Differentiated Nephroblastoma - Multilocular Cystic Nephroma - Primary Renal Synovial Sarcoma - Anaplastic Sarcoma of the Kidney - Nephroblastomatosis is not cancer but may become Wilms tumor. - Having certain genetic syndromes or other conditions can increase the risk of Wilms tumor. - Tests are used to screen for Wilms tumor. - Having certain conditions may increase the risk of renal cell cancer. - Treatment for Wilms tumor and other childhood kidney tumors may include genetic counseling. - Signs of Wilms tumor and other childhood kidney tumors include a lump in the abdomen and blood in the urine. - Tests that examine the kidney and the blood are used to detect (find) and diagnose Wilms tumor and other childhood kidney tumors. - Certain factors affect prognosis (chance of recovery) and treatment options. Childhood kidney tumors are diseases in which malignant (cancer) cells form in the tissues of the kidney. There are two kidneys, one on each side of the backbone, above the waist. Tiny tubules in the kidneys filter and clean the blood. They take out waste products and make urine. The urine passes from each kidney through a long tube called a ureter into the bladder. The bladder holds the urine until it passes through the urethra and leaves the body. There are many types of childhood kidney tumors. Wilms Tumor In Wilms tumor, one or more tumors may be found in one or both kidneys. Wilms tumor may spread to the lungs, liver, bone, brain, or nearby lymph nodes. In children and adolescents younger than 15 years old, most kidney cancers are Wilms tumors. Renal Cell Cancer (RCC) Renal cell cancer is rare in children and adolescents younger than 15 years old. It is much more common in adolescents between 15 and 19 years old. Children and adolescents are more likely to be diagnosed with a large renal cell tumor or cancer that has spread. Renal cell cancers may spread to the lungs, liver, or lymph nodes. Renal cell cancer may also be called renal cell carcinoma. Rhabdoid Tumor of the Kidney Rhabdoid tumor of the kidney is a type of kidney cancer that occurs mostly in infants and young children. It is often advanced at the time of diagnosis. Rhabdoid tumor of the kidney grows and spreads quickly, often to the lungs or brain. Children with a certain change in the SMARCB1 gene are checked regularly to see if a rhabdoid tumor has formed in the kidney or has spread to the brain: - Children younger than one year old have an ultrasound of the abdomen every two to three months and an ultrasound of the head every month. - Children one to four years old have an ultrasound of the abdomen and an MRI of the brain and spine every three months. Clear Cell Sarcoma of the Kidney Clear cell sarcoma of the kidney is a type of kidney tumor that may spread to the lung, bone, brain, or soft tissue. When it recurs (comes back) after treatment, it often recurs in the brain or lung. Congenital Mesoblastic Nephroma Congenital mesoblastic nephroma is a tumor of the kidney that is often diagnosed during the first year of life. It can usually be cured. Ewing Sarcoma of the Kidney Ewing sarcoma (previously called neuroepithelial tumor) of the kidney is rare and usually occurs in young adults. These tumors grow and spread to other parts of the body quickly. Primary Renal Myoepithelial Carcinoma Primary renal myoepithelial carcinoma is a rare type of cancer that usually affects soft tissues, but sometimes forms in the internal organs (such as the kidney). This type of cancer grows and spreads quickly. Cystic Partially Differentiated Nephroblastoma Cystic partially differentiated nephroblastoma is a very rare type of Wilms tumor made up of cysts. Multilocular Cystic Nephroma Multilocular cystic nephromas are benign tumors made up of cysts and are most common in infants, young children, and adult women. These tumors can occur in one or both kidneys. Children with this type of tumor also may have pleuropulmonary blastoma, so imaging tests that check the lungs for cysts or solid tumors are done. Since multilocular cystic nephroma may be an inherited condition, genetic counseling and genetic testing may be considered. See the PDQ summary about Unusual Cancers of Childhood Treatment for more information about pleuropulmonary blastoma. Primary Renal Synovial Sarcoma Primary renal synovial sarcoma is a cyst-like tumor of the kidney and is most common in young adults. These tumors grow and spread quickly. Anaplastic Sarcoma of the Kidney Anaplastic sarcoma of the kidney is a rare tumor that is most common in children or adolescents younger than 15 years of age. Anaplastic sarcoma of the kidney often spreads to the lungs, liver, or bones. Imaging tests that check the lungs for cysts or solid tumors may be done. Since anaplastic sarcoma may be an inherited condition, genetic counseling and genetic testing may be considered. Nephroblastomatosis is not cancer but may become Wilms tumor. Sometimes, after the kidneys form in the fetus, abnormal groups of kidney cells remain in one or both kidneys. In nephroblastomatosis (diffuse hyperplastic perilobar nephroblastomatosis), these abnormal groups of cells may grow in many places inside the kidney or make a thick layer around the kidney. When these groups of abnormal cells are found in a kidney after it was removed for Wilms tumor, the child has an increased risk of Wilms tumor in the other kidney. Frequent follow-up testing is important at least every 3 months, for at least 7 years after the child is treated. Treatment for Wilms tumor and other childhood kidney tumors may include genetic counseling. Genetic counseling (a discussion with a trained professional about genetic diseases and whether genetic testing is needed) may be needed if the child has one of the following syndromes or conditions: - A genetic syndrome or condition that increases the risk of Wilms tumor. - An inherited condition that increases the risk of renal cell cancer. - Rhabdoid tumor of the kidney. - Multilocular cystic nephroma.",Wilms Tumor and Other Childhood Kidney Tumors,0000026_3,CancerGov,https://www.cancer.gov/types/kidney/patient/wilms-treatment-pdq,C1333003,T191,Disorders Who is at risk for Wilms Tumor and Other Childhood Kidney Tumors? ?,0000026_3-2,susceptibility,"Having certain genetic syndromes or other conditions can increase the risk of Wilms tumor. Anything that increases the risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk to your child's doctor if you think your child may be at risk. Wilms tumor may be part of a genetic syndrome that affects growth or development. A genetic syndrome is a set of signs and symptoms or conditions that occur together and is caused by certain changes in the genes. Certain conditions can also increase a child's risk of developing Wilms tumor. These and other genetic syndromes and conditions have been linked to Wilms tumor: - WAGR syndrome (Wilms tumor, aniridia, abnormal genitourinary system, and mental retardation). - Denys-Drash syndrome (abnormal genitourinary system). - Frasier syndrome (abnormal genitourinary system). - Beckwith-Wiedemann syndrome (abnormally large growth of one side of the body or a body part, large tongue, umbilical hernia at birth, and abnormal genitourinary system). - A family history of Wilms tumor. - Aniridia (the iris, the colored part of the eye, is missing). - Isolated hemihyperplasia (abnormally large growth of one side of the body or a body part). - Urinary tract problems such as cryptorchidism or hypospadias. Having certain conditions may increase the risk of renal cell cancer. Renal cell cancer may be related to the following conditions: - Von Hippel-Lindau disease (an inherited condition that causes abnormal growth of blood vessels). Children with Von Hippel-Lindau disease should be checked yearly for renal cell cancer with an ultrasound of the abdomen or an MRI (magnetic resonance imaging) beginning at age 8 to 11 years. - Tuberous sclerosis (an inherited disease marked by noncancerous fatty cysts in the kidney). - Familial renal cell cancer (an inherited condition that occurs when certain changes in the genes that cause kidney cancer are passed down from the parent to the child). - Renal medullary cancer (a rare kidney cancer that grows and spreads quickly). - Hereditary leiomyomatosis (an inherited disorder that increases the risk of having cancer of the kidney, skin, and uterus). Prior chemotherapy or radiation therapy for a childhood cancer, such as neuroblastoma, soft tissue sarcoma, leukemia, or Wilms tumor may also increase the risk of renal cell cancer. See the Second Cancers section in the PDQ summary about Late Effects of Treatment for Childhood Cancer for more information.",Wilms Tumor and Other Childhood Kidney Tumors,0000026_3,CancerGov,https://www.cancer.gov/types/kidney/patient/wilms-treatment-pdq,C1333003,T191,Disorders What are the symptoms of Wilms Tumor and Other Childhood Kidney Tumors ?,0000026_3-3,symptoms,"Signs of Wilms tumor and other childhood kidney tumors include a lump in the abdomen and blood in the urine. Sometimes childhood kidney tumors do not cause signs and symptoms and the parent finds a mass in the abdomen by chance or the mass is found during a well-child health check up. These and other signs and symptoms may be caused by kidney tumors or by other conditions. Check with your child's doctor if your child has any of the following: - A lump, swelling, or pain in the abdomen. - Blood in the urine. - High blood pressure (headache, feeling very tired, chest pain, or trouble seeing or breathing). - Hypercalcemia (loss of appetite, nausea and vomiting, weakness, or feeling very tired). - Fever for no known reason. - Loss of appetite. - Weight loss for no known reason. Wilms tumor that has spread to the lungs or liver may cause the following signs and symptoms: - Cough. - Blood in the sputum. - Trouble breathing. - Pain in the abdomen.",Wilms Tumor and Other Childhood Kidney Tumors,0000026_3,CancerGov,https://www.cancer.gov/types/kidney/patient/wilms-treatment-pdq,C1333003,T191,Disorders How to diagnose Wilms Tumor and Other Childhood Kidney Tumors ?,0000026_3-4,exams and tests,"Tests are used to screen for Wilms tumor. Screening tests are done in children with an increased risk of Wilms tumor. These tests may help find cancer early and decrease the chance of dying from cancer. In general, children with an increased risk of Wilms tumor should be screened for Wilms tumor every three months until they are at least 8 years old. An ultrasound test of the abdomen is usually used for screening. Small Wilms tumors may be found and removed before symptoms occur. Children with Beckwith-Wiedemann syndrome or hemihyperplasia are also screened for liver and adrenal tumors that are linked to these genetic syndromes. A test to check the alpha-fetoprotein (AFP) level in the blood and an ultrasound of the abdomen are done until the child is 4 years old. An ultrasound of the kidneys is done after the child is 4 years old. In children with certain gene changes, a different schedule for ultrasound of the abdomen may be used. Children with aniridia and a certain gene change are screened for Wilms tumor every three months until they are 8 years old. An ultrasound test of the abdomen is used for screening. Some children develop Wilms tumor in both kidneys. These often appear when Wilms tumor is first diagnosed, but Wilms tumor may also occur in the second kidney after the child is successfully treated for Wilms tumor in one kidney. Children with an increased risk of a second Wilms tumor in the other kidney should be screened for Wilms tumor every three months for up to eight years. An ultrasound test of the abdomen may be used for screening. Tests that examine the kidney and the blood are used to detect (find) and diagnose Wilms tumor and other childhood kidney tumors. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following: - The number of red blood cells, white blood cells, and platelets. - The amount of hemoglobin (the protein that carries oxygen) in the red blood cells. - The portion of the blood sample made up of red blood cells. - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. This test is done to check how well the liver and kidneys are working. - Renal function test : A procedure in which blood or urine samples are checked to measure the amounts of certain substances released into the blood or urine by the kidneys. A higher or lower than normal amount of a substance can be a sign that the kidneys are not working as they should. - Urinalysis : A test to check the color of urine and its contents, such as sugar, protein, blood, and bacteria. - Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. An ultrasound of the abdomen is done to diagnose a kidney tumor. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the chest, abdomen, and pelvis, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye is injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - MRI (magnetic resonance imaging) with gadolinium: A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body, such as the abdomen. A substance called gadolinium is injected into a vein. The gadolinium collects around the cancer cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI). - X-ray: An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body, such as the chest and abdomen. - PET-CT scan : A procedure that combines the pictures from a positron emission tomography (PET) scan and a computed tomography (CT) scan. The PET and CT scans are done at the same time on the same machine. The pictures from both scans are combined to make a more detailed picture than either test would make by itself. A PET scan is a procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. - Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. The decision of whether to do a biopsy is based on the following: - The size of the tumor. - The stage of the cancer. - Whether cancer is in one or both kidneys. - Whether imaging tests clearly show the cancer. - Whether the tumor can be removed by surgery. - Whether the patient is in a clinical trial. A biopsy may be done before any treatment is given, after chemotherapy to shrink the tumor, or after surgery to remove the tumor.",Wilms Tumor and Other Childhood Kidney Tumors,0000026_3,CancerGov,https://www.cancer.gov/types/kidney/patient/wilms-treatment-pdq,C1333003,T191,Disorders What is the outlook for Wilms Tumor and Other Childhood Kidney Tumors ?,0000026_3-5,outlook,Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options for Wilms tumor depend on the following: - How different the tumor cells are from normal kidney cells when looked at under a microscope. - The stage of the cancer. - The type of tumor. - The age of the child. - Whether the tumor can be completely removed by surgery. - Whether there are certain changes in chromosomes or genes. - Whether the cancer has just been diagnosed or has recurred (come back). The prognosis for renal cell cancer depends on the following: - The stage of the cancer. - Whether the cancer has spread to the lymph nodes. The prognosis for rhabdoid tumor of the kidney depends on the following: - The age of the child at the time of diagnosis. - The stage of the cancer. - Whether the cancer has spread to the brain or spinal cord. The prognosis for clear cell sarcoma of the kidney depends on the following: - The age of the child at the time of diagnosis. - The stage of the cancer.,Wilms Tumor and Other Childhood Kidney Tumors,0000026_3,CancerGov,https://www.cancer.gov/types/kidney/patient/wilms-treatment-pdq,C1333003,T191,Disorders What are the stages of Wilms Tumor and Other Childhood Kidney Tumors ?,0000026_3-6,stages,"Key Points - Wilms tumors are staged during surgery and with imaging tests. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - In addition to the stages, Wilms tumors are described by their histology. - The following stages are used for both favorable histology and anaplastic Wilms tumors: - Stage I - Stage II - Stage III - Stage IV - Stage V - The treatment of other childhood kidney tumors depends on the tumor type. Wilms tumors are staged during surgery and with imaging tests. The process used to find out if cancer has spread outside of the kidney to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The doctor will use results of the diagnostic and staging tests to help find out the stage of the disease. The following tests may be done to see if cancer has spread to other places in the body: - Lymph node biopsy : A surgical procedure in which lymph nodes in the abdomen are removed and a sample of tissue is checked under a microscope for signs of cancer. This procedure is also called lymphadenectomy or lymph node dissection. - Liver function test : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by the liver. A higher than normal amount of a substance can be a sign that the liver is not working as it should. - X-ray of the chest and bones: An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body, such as the chest. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the abdomen, pelvis, chest, and brain, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye is injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - PET-CT scan : A procedure that combines the pictures from a positron emission tomography (PET) scan and a computed tomography (CT) scan. The PET and CT scans are done at the same time on the same machine. The pictures from both scans are combined to make a more detailed picture than either test would make by itself. A PET scan is a procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body, such as the abdomen, pelvis, and brain. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Bone scan : A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones with cancer and is detected by a scanner. - Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. An ultrasound of the major heart vessels is done to stage Wilms tumor. - Cystoscopy : A procedure to look inside the bladder and urethra to check for abnormal areas. A cystoscope is inserted through the urethra into the bladder. A cystoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if Wilms tumor spreads to the lung, the cancer cells in the lung are actually Wilms tumor cells. The disease is metastatic Wilms tumor, not lung cancer. In addition to the stages, Wilms tumors are described by their histology. The histology (how the cells look under a microscope) of the tumor affects the prognosis and the treatment of Wilms tumor. The histology may be favorable or anaplastic (unfavorable). Tumors with a favorable histology have a better prognosis and respond better to chemotherapy than anaplastic tumors. Tumor cells that are anaplastic divide quickly and under a microscope do not look like the type of cells they came from. Anaplastic tumors are harder to treat with chemotherapy than other Wilms tumors at the same stage. The following stages are used for both favorable histology and anaplastic Wilms tumors: Stage I In stage I, the tumor was completely removed by surgery and all of the following are true: - Cancer was found only in the kidney and had not spread to blood vessels in the renal sinus (the part of the kidney where it joins the ureter) or to the lymph nodes. - The outer layer of the kidney did not break open. - The tumor did not break open. - A biopsy was not done before the tumor was removed. - No cancer cells were found at the edges of the area where the tumor was removed. Stage II In stage II, the tumor was completely removed by surgery and no cancer cells were found at the edges of the area where the cancer was removed. Cancer has not spread to lymph nodes. Before the tumor was removed, one of the following was true: - Cancer had spread to the renal sinus (the part of the kidney where it joins the ureter). - Cancer had spread to blood vessels outside the area of the kidney where urine is made, such as the renal sinus. Stage III In stage III, cancer remains in the abdomen after surgery and one of the following may be true: - Cancer has spread to lymph nodes in the abdomen or pelvis (the part of the body between the hips). - Cancer has spread to or through the surface of the peritoneum (the layer of tissue that lines the abdominal cavity and covers most organs in the abdomen). - A biopsy of the tumor was done before it was removed. - The tumor broke open before or during surgery to remove it. - The tumor was removed in more than one piece. - Cancer cells are found at the edges of the area where the tumor was removed. - The entire tumor could not be removed because important organs or tissues in the body would be damaged. Stage IV In stage IV, cancer has spread through the blood to organs such as the lungs, liver, bone, or brain, or to lymph nodes outside of the abdomen and pelvis. Stage V In stage V, cancer cells are found in both kidneys when the cancer is first diagnosed. The treatment of other childhood kidney tumors depends on the tumor type.",Wilms Tumor and Other Childhood Kidney Tumors,0000026_3,CancerGov,https://www.cancer.gov/types/kidney/patient/wilms-treatment-pdq,C1333003,T191,Disorders What are the treatments for Wilms Tumor and Other Childhood Kidney Tumors ?,0000026_3-7,treatment,"Key Points - There are different types of treatment for patients with Wilms tumor and other childhood kidney tumors. - Children with Wilms tumor or other childhood kidney tumors should have their treatment planned by a team of health care providers who are experts in treating cancer in children. - Some cancer treatments cause side effects months or years after treatment has ended. - Five types of standard treatment are used: - Surgery - Radiation therapy - Chemotherapy - Biologic therapy - High-dose chemotherapy with stem cell rescue - New types of treatment are being tested in clinical trials. - Targeted therapy - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with Wilms tumor and other childhood kidney tumors. Different types of treatment are available for children with Wilms and other childhood kidney tumors. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment. Children with Wilms tumor or other childhood kidney tumors should have their treatment planned by a team of health care providers who are experts in treating cancer in children. Your child's treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other pediatric health care providers who are experts in treating children with Wilms tumor or other childhood kidney tumors and who specialize in certain areas of medicine. These may include the following specialists: - Pediatrician. - Pediatric surgeon or urologist. - Radiation oncologist. - Rehabilitation specialist. - Pediatric nurse specialist. - Social worker. Some cancer treatments cause side effects months or years after treatment has ended. Side effects from cancer treatment that begin during or after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include the following: - Physical problems, such as heart problems, kidney problems, or problems during pregnancy. - Changes in mood, feelings, thinking, learning, or memory. - Second cancers (new types of cancer), such as cancer of the gastrointestinal tract or breast cancer. Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. (See the PDQ summary about Late Effects of Treatment for Childhood Cancer for more information). Clinical trials are being done to find out if lower doses of chemotherapy and radiation can be used to lessen the late effects of treatment without changing how well the treatment works. Five types of standard treatment are used: Surgery Two types of surgery are used to treat kidney tumors: - Nephrectomy: Wilms tumor and other childhood kidney tumors are usually treated with nephrectomy (surgery to remove the whole kidney). Nearby lymph nodes may also be removed and checked for signs of cancer. Sometimes a kidney transplant (surgery to remove the kidney and replace it with a kidney from a donor) is done when the cancer is in both kidneys and the kidneys are not working well. - Partial nephrectomy: If cancer is found in both kidneys or is likely to spread to both kidneys, surgery may include a partial nephrectomy (removal of the cancer in the kidney and a small amount of normal tissue around it). Partial nephrectomy is done to keep as much of the kidney working as possible. A partial nephrectomy is also called renal-sparing surgery. Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk of the cancer coming back, is called adjuvant therapy. Sometimes, a second-look surgery is done to see if cancer remains after chemotherapy or radiation therapy. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated and whether a biopsy was done before surgery to remove the tumor. External radiation therapy is used to treat Wilms tumor and other childhood kidney tumors. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is treatment using two or more anticancer drugs. The way the chemotherapy is given depends on the type and stage of the cancer being treated. Systemic chemotherapy is used to treat Wilms tumor and other childhood kidney tumors. Sometimes the tumor cannot be removed by surgery for one of the following reasons: - The tumor is too close to important organs or blood vessels. - The tumor is too large to remove. - The cancer is in both kidneys. - There is a blood clot in the vessels near the liver. - The patient has trouble breathing because cancer has spread to the lungs. In this case, a biopsy is done first. Then chemotherapy is given to reduce the size of the tumor before surgery, in order to save as much healthy tissue as possible and lessen problems after surgery. This is called neoadjuvant chemotherapy. Radiation therapy is given after surgery. See Drugs Approved for Wilms Tumor and Other Childhood Kidney Cancers for more information. Biologic therapy Biologic therapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy. Interferon and interleukin-2 (IL-2) are types of biologic therapy used to treat childhood renal cell cancer. Interferon affects the division of cancer cells and can slow tumor growth. IL-2 boosts the growth and activity of many immune cells, especially lymphocytes (a type of white blood cell). Lymphocytes can attack and kill cancer cells. High-dose chemotherapy with stem cell rescue High-dose chemotherapy with stem cell rescue is a method of giving high doses of chemotherapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These re-infused stem cells grow into (and restore) the body's blood cells. High-dose chemotherapy with stem cell rescue may be used to treat recurrent Wilms tumor. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Wilms Tumor Stage I Wilms Tumor Treatment of stage I Wilms tumor with favorable histology may include: - Nephrectomy with removal of lymph nodes, followed by combination chemotherapy. - A clinical trial of nephrectomy only. Treatment of stage I anaplastic Wilms tumor may include: - Nephrectomy with removal of lymph nodes followed by combination chemotherapy and radiation therapy to the flank area (either side of the body between the ribs and hipbone). Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I Wilms tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Stage II Wilms Tumor Treatment of stage II Wilms tumor with favorable histology may include: - Nephrectomy with removal of lymph nodes, followed by combination chemotherapy. Treatment of stage II anaplastic Wilms tumor may include: - Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage II Wilms tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Stage III Wilms Tumor Treatment of stage III Wilms tumor with favorable histology may include: - Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy. Treatment of stage III anaplastic Wilms tumor may include: - Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy. - Combination chemotherapy followed by nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III Wilms tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Stage IV Wilms Tumor Treatment of stage IV Wilms tumor with favorable histology may include: - Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy. If cancer has spread to other parts of the body, patients will also receive radiation therapy to those areas. Treatment of stage IV anaplastic Wilms tumor may include: - Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy. If cancer has spread to other parts of the body, patients will also receive radiation therapy to those areas. - Combination chemotherapy given before nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen. If cancer has spread to other parts of the body, patients will also receive radiation therapy to those areas. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage IV Wilms tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Stage V Wilms Tumor and patients at high risk of developing bilateral Wilms tumor Treatment of stage V Wilms tumor may be different for each patient and may include: - Combination chemotherapy to shrink the tumor, followed by repeat imaging at 4 to 8 weeks to decide on further therapy (partial nephrectomy, biopsy, continued chemotherapy, and/or radiation therapy). - A biopsy of the kidneys is followed by combination chemotherapy to shrink the tumor. A second surgery is done to remove as much of the cancer as possible. This may be followed by more chemotherapy and/or radiation therapy if cancer remains after surgery. If a kidney transplant is needed because of kidney problems, it is delayed until 1 to 2 years after treatment is completed and there are no signs of cancer. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage V Wilms tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Treatment Options for Other Childhood Kidney Tumors Renal Cell Cancer (RCC) Treatment of renal cell cancer usually includes: - Surgery, which may be: - nephrectomy with removal of lymph nodes; or - partial nephrectomy with removal of lymph nodes. - Biologic therapy (interferon and interleukin-2) for cancer that has spread to other parts of the body. See the PDQ summary about Renal Cell Cancer Treatment for more information. Check the list of NCI-supported cancer clinical trials that are now accepting patients with renal cell carcinoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Rhabdoid Tumor of the Kidney There is no standard treatment for rhabdoid tumor of the kidney. Treatment may include: - A combination of surgery, chemotherapy, and/or radiation therapy. - A clinical trial of targeted therapy (tazemetostat). Check the list of NCI-supported cancer clinical trials that are now accepting patients with rhabdoid tumor of the kidney. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Clear Cell Sarcoma of the Kidney Treatment of clear cell sarcoma of the kidney may include: - Nephrectomy with removal of lymph nodes followed by combination chemotherapy and radiation therapy to the abdomen. - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with clear cell sarcoma of the kidney. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Congenital Mesoblastic Nephroma Treatment for congenital mesoblastic nephroma usually includes: - Surgery that may be followed by chemotherapy. - A clinical trial of targeted therapy (LOXO-101 or entrectinib). Check the list of NCI-supported cancer clinical trials that are now accepting patients with congenital mesoblastic nephroma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Ewing Sarcoma of the Kidney There is no standard treatment for Ewing sarcoma of the kidney. Treatment may include: - A combination of surgery, chemotherapy, and radiation therapy. It may also be treated in the same way that Ewing sarcoma is treated. See the PDQ summary about Ewing Sarcoma Treatment for more information. Check the list of NCI-supported cancer clinical trials that are now accepting patients with Ewing sarcoma/peripheral primitive neuroectodermal tumor (PNET). For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Primary Renal Myoepithelial Carcinoma There is no standard treatment for primary renal myoepithelial carcinoma. Treatment may include: - A combination of surgery, chemotherapy, and radiation therapy. Cystic Partially Differentiated Nephroblastoma Treatment of cystic partially differentiated nephroblastoma may include: - Surgery that may be followed by chemotherapy. Multilocular Cystic Nephroma Treatment of multilocular cystic nephroma usually includes: - Surgery. Primary Renal Synovial Sarcoma Treatment of primary renal synovial sarcoma usually includes: - Chemotherapy. Anaplastic Sarcoma of the Kidney There is no standard treatment for anaplastic sarcoma of the kidney. Treatment is usually the same treatment given for anaplastic Wilms tumor. Nephroblastomatosis (Diffuse Hyperplastic Perilobar Nephroblastomatosis) The treatment of nephroblastomatosis depends on the following: - Whether the child has abnormal groups of cells in one or both kidneys. - Whether the child has Wilms tumor in one kidney and groups of abnormal cells in the other kidney. Treatment of nephroblastomatosis may include: - Chemotherapy followed by nephrectomy. Sometimes a partial nephrectomy may be done to keep as much kidney function as possible.",Wilms Tumor and Other Childhood Kidney Tumors,0000026_3,CancerGov,https://www.cancer.gov/types/kidney/patient/wilms-treatment-pdq,C1333003,T191,Disorders what research (or clinical trials) is being done for Wilms Tumor and Other Childhood Kidney Tumors ?,0000026_3-8,research,"New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Targeted therapy Targeted therapy is a treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Targeted therapy used to treat childhood kidney tumors may include the following: - Monoclonal antibodies: This targeted therapy uses antibodies made in the laboratory, from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Lorvotuzumab is being studied in the treatment of recurrent Wilms tumor. - Kinase inhibitors: This targeted therapy blocks signals that cancer cells need to grow and divide. LOXO-101 and entrectinib are kinase inhibitors being studied to treat congenital mesoblastic nephroma. - Histone methyltransferase inhibitors: This targeted therapy slows down the cancer cell's ability to grow and divide. Tazemetostat is being studied in the treatment of rhabdoid tumor of the kidney. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Wilms Tumor and Other Childhood Kidney Tumors,0000026_3,CancerGov,https://www.cancer.gov/types/kidney/patient/wilms-treatment-pdq,C1333003,T191,Disorders What is (are) Breast Cancer ?,0000027_1-1,information,"Key Points - Breast cancer is a disease in which malignant (cancer) cells form in the tissues of the breast. - A family history of breast cancer and other factors increase the risk of breast cancer. - Breast cancer is sometimes caused by inherited gene mutations (changes). - The use of certain medicines and other factors decrease the risk of breast cancer. - Signs of breast cancer include a lump or change in the breast. - Tests that examine the breasts are used to detect (find) and diagnose breast cancer. - If cancer is found, tests are done to study the cancer cells. - Certain factors affect prognosis (chance of recovery) and treatment options. Breast cancer is a disease in which malignant (cancer) cells form in the tissues of the breast. The breast is made up of lobes and ducts. Each breast has 15 to 20 sections called lobes. Each lobe has many smaller sections called lobules. Lobules end in dozens of tiny bulbs that can make milk. The lobes, lobules, and bulbs are linked by thin tubes called ducts. Each breast also has blood vessels and lymph vessels. The lymph vessels carry an almost colorless fluid called lymph. Lymph vessels carry lymph between lymph nodes. Lymph nodes are small bean-shaped structures that are found throughout the body. They filter substances in lymph and help fight infection and disease. Clusters of lymph nodes are found near the breast in the axilla (under the arm), above the collarbone, and in the chest. The most common type of breast cancer is ductal carcinoma, which begins in the cells of the ducts. Cancer that begins in the lobes or lobules is called lobular carcinoma and is more often found in both breasts than are other types of breast cancer. Inflammatory breast cancer is an uncommon type of breast cancer in which the breast is warm, red, and swollen. See the following PDQ summaries for more information about breast cancer: - Breast Cancer Prevention - Breast Cancer Screening - Breast Cancer Treatment and Pregnancy - Male Breast Cancer Treatment - Unusual Cancers of Childhood Treatment (for information about breast cancer in childhood)",Breast Cancer,0000027_1,CancerGov,https://www.cancer.gov/types/breast/patient/breast-treatment-pdq,C0006142,T191,Disorders Who is at risk for Breast Cancer? ?,0000027_1-2,susceptibility,"A family history of breast cancer and other factors increase the risk of breast cancer. Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk to your doctor if you think you may be at risk for breast cancer. Risk factors for breast cancer include the following: - A personal history of invasive breast cancer, ductal carcinoma in situ (DCIS), or lobular carcinoma in situ (LCIS). - A personal history of benign (noncancer) breast disease. - A family history of breast cancer in a first-degree relative (mother, daughter, or sister). - Inherited changes in the BRCA1 or BRCA2 genes or in other genes that increase the risk of breast cancer. - Breast tissue that is dense on a mammogram. - Exposure of breast tissue to estrogen made by the body. This may be caused by: - Menstruating at an early age. - Older age at first birth or never having given birth. - Starting menopause at a later age. - Taking hormones such as estrogen combined with progestin for symptoms of menopause. - Treatment with radiation therapy to the breast/chest. - Drinking alcohol. - Obesity. Older age is the main risk factor for most cancers. The chance of getting cancer increases as you get older. NCI's Breast Cancer Risk Assessment Tool uses a woman's risk factors to estimate her risk for breast cancer during the next five years and up to age 90. This online tool is meant to be used by a health care provider. For more information on breast cancer risk, call 1-800-4-CANCER.",Breast Cancer,0000027_1,CancerGov,https://www.cancer.gov/types/breast/patient/breast-treatment-pdq,C0006142,T191,Disorders How to prevent Breast Cancer ?,0000027_1-3,prevention,The use of certain medicines and other factors decrease the risk of breast cancer. Anything that decreases your chance of getting a disease is called a protective factor. Protective factors for breast cancer include the following: - Taking any of the following: - Estrogen-only hormone therapy after a hysterectomy. - Selective estrogen receptor modulators (SERMs). - Aromatase inhibitors. - Less exposure of breast tissue to estrogen made by the body. This can be a result of: - Early pregnancy. - Breastfeeding. - Getting enough exercise. - Having any of the following procedures: - Mastectomy to reduce the risk of cancer. - Oophorectomy to reduce the risk of cancer. - Ovarian ablation.,Breast Cancer,0000027_1,CancerGov,https://www.cancer.gov/types/breast/patient/breast-treatment-pdq,C0006142,T191,Disorders Is Breast Cancer inherited ?,0000027_1-4,inheritance,"Breast cancer is sometimes caused by inherited gene mutations (changes). The genes in cells carry the hereditary information that is received from a persons parents. Hereditary breast cancer makes up about 5% to 10% of all breast cancer. Some mutated genes related to breast cancer are more common in certain ethnic groups. Women who have certain gene mutations, such as a BRCA1 or BRCA2 mutation, have an increased risk of breast cancer. These women also have an increased risk of ovarian cancer, and may have an increased risk of other cancers. Men who have a mutated gene related to breast cancer also have an increased risk of breast cancer. For more information, see the PDQ summary on Male Breast Cancer Treatment. There are tests that can detect (find) mutated genes. These genetic tests are sometimes done for members of families with a high risk of cancer. See the PDQ summary on Genetics of Breast and Gynecologic Cancers for more information.",Breast Cancer,0000027_1,CancerGov,https://www.cancer.gov/types/breast/patient/breast-treatment-pdq,C0006142,T191,Disorders What are the symptoms of Breast Cancer ?,0000027_1-5,symptoms,"Signs of breast cancer include a lump or change in the breast. These and other signs may be caused by breast cancer or by other conditions. Check with your doctor if you have any of the following: - A lump or thickening in or near the breast or in the underarm area. - A change in the size or shape of the breast. - A dimple or puckering in the skin of the breast. - A nipple turned inward into the breast. - Fluid, other than breast milk, from the nipple, especially if it's bloody. - Scaly, red, or swollen skin on the breast, nipple, or areola (the dark area of skin around the nipple). - Dimples in the breast that look like the skin of an orange, called peau dorange.",Breast Cancer,0000027_1,CancerGov,https://www.cancer.gov/types/breast/patient/breast-treatment-pdq,C0006142,T191,Disorders How to diagnose Breast Cancer ?,0000027_1-6,exams and tests,"Tests that examine the breasts are used to detect (find) and diagnose breast cancer. Check with your doctor if you notice any changes in your breasts. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Clinical breast exam (CBE): An exam of the breast by a doctor or other health professional. The doctor will carefully feel the breasts and under the arms for lumps or anything else that seems unusual. - Mammogram: An x-ray of the breast. - Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of both breasts. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. - Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. If a lump in the breast is found, a biopsy may be done. There are four types of biopsy used to check for breast cancer: - Excisional biopsy : The removal of an entire lump of tissue. - Incisional biopsy : The removal of part of a lump or a sample of tissue. - Core biopsy : The removal of tissue using a wide needle. - Fine-needle aspiration (FNA) biopsy : The removal of tissue or fluid, using a thin needle. If cancer is found, tests are done to study the cancer cells. Decisions about the best treatment are based on the results of these tests. The tests give information about: - how quickly the cancer may grow. - how likely it is that the cancer will spread through the body. - how well certain treatments might work. - how likely the cancer is to recur (come back). Tests include the following: - Estrogen and progesterone receptor test : A test to measure the amount of estrogen and progesterone (hormones) receptors in cancer tissue. If there are more estrogen and progesterone receptors than normal, the cancer is called estrogen and/or progesterone receptor positive. This type of breast cancer may grow more quickly. The test results show whether treatment to block estrogen and progesterone may stop the cancer from growing. - Human epidermal growth factor type 2 receptor (HER2/neu) test : A laboratory test to measure how many HER2/neu genes there are and how much HER2/neu protein is made in a sample of tissue. If there are more HER2/neu genes or higher levels of HER2/neu protein than normal, the cancer is called HER2/neu positive. This type of breast cancer may grow more quickly and is more likely to spread to other parts of the body. The cancer may be treated with drugs that target the HER2/neu protein, such as trastuzumab and pertuzumab. - Multigene tests: Tests in which samples of tissue are studied to look at the activity of many genes at the same time. These tests may help predict whether cancer will spread to other parts of the body or recur (come back). There are many types of multigene tests. The following multigene tests have been studied in clinical trials: - Oncotype DX : This test helps predict whether stage I or stage II breast cancer that is estrogen receptor positive and node negative will spread to other parts of the body. If the risk that the cancer will spread is high, chemotherapy may be given to lower the risk. - MammaPrint : This test helps predict whether stage I or stage II breast cancer that is node negative will spread to other parts of the body. If the risk that the cancer will spread is high, chemotherapy may be given to lower the risk. Based on these tests, breast cancer is described as one of the following types: - Hormone receptor positive (estrogen and/or progesterone receptor positive) or hormone receptor negative (estrogen and/or progesterone receptor negative). - HER2/neu positive or HER2/neu negative. - Triple negative (estrogen receptor, progesterone receptor, and HER2/neu negative). This information helps the doctor decide which treatments will work best for your cancer.",Breast Cancer,0000027_1,CancerGov,https://www.cancer.gov/types/breast/patient/breast-treatment-pdq,C0006142,T191,Disorders What is the outlook for Breast Cancer ?,0000027_1-7,outlook,"Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - The stage of the cancer (the size of the tumor and whether it is in the breast only or has spread to lymph nodes or other places in the body). - The type of breast cancer. - Estrogen receptor and progesterone receptor levels in the tumor tissue. - Human epidermal growth factor type 2 receptor (HER2/neu) levels in the tumor tissue. - Whether the tumor tissue is triple negative (cells that do not have estrogen receptors, progesterone receptors, or high levels of HER2/neu). - How fast the tumor is growing. - How likely the tumor is to recur (come back). - A womans age, general health, and menopausal status (whether a woman is still having menstrual periods). - Whether the cancer has just been diagnosed or has recurred (come back).",Breast Cancer,0000027_1,CancerGov,https://www.cancer.gov/types/breast/patient/breast-treatment-pdq,C0006142,T191,Disorders What are the stages of Breast Cancer ?,0000027_1-8,stages,"Key Points - After breast cancer has been diagnosed, tests are done to find out if cancer cells have spread within the breast or to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - The following stages are used for breast cancer: - Stage 0 (carcinoma in situ) - Stage I - Stage II - Stage IIIA - Stage IIIB - Stage IIIC - Stage IV - The treatment of breast cancer depends partly on the stage of the disease. After breast cancer has been diagnosed, tests are done to find out if cancer cells have spread within the breast or to other parts of the body. The process used to find out whether the cancer has spread within the breast or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The results of some of the tests used to diagnose breast cancer are also used to stage the disease. (See the General Information section.) The following tests and procedures also may be used in the staging process: - Sentinel lymph node biopsy : The removal of the sentinel lymph node during surgery. The sentinel lymph node is the first lymph node to receive lymphatic drainage from a tumor. It is the first lymph node the cancer is likely to spread to from the tumor. A radioactive substance and/or blue dye is injected near the tumor. The substance or dye flows through the lymph ducts to the lymph nodes. The first lymph node to receive the substance or dye is removed. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are not found, it may not be necessary to remove more lymph nodes. - Chest x-ray : An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - Bone scan : A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones and is detected by a scanner. - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if breast cancer spreads to the bone, the cancer cells in the bone are actually breast cancer cells. The disease is metastatic breast cancer, not bone cancer. The following stages are used for breast cancer: This section describes the stages of breast cancer. The breast cancer stage is based on the results of tests that are done on the tumor and lymph nodes removed during surgery and on other tests. Stage 0 (carcinoma in situ) There are 3 types of breast carcinoma in situ: - Ductal carcinoma in situ (DCIS) is a noninvasive condition in which abnormal cells are found in the lining of a breast duct. The abnormal cells have not spread outside the duct to other tissues in the breast. In some cases, DCIS may become invasive cancer and spread to other tissues. At this time, there is no way to know which lesions could become invasive. - Lobular carcinoma in situ (LCIS) is a condition in which abnormal cells are found in the lobules of the breast. This condition seldom becomes invasive cancer. Information about LCIS is not included in this summary. - Paget disease of the nipple is a condition in which abnormal cells are found in the nipple only. Stage I In stage I, cancer has formed. Stage I is divided into stages IA and IB. - In stage IA, the tumor is 2 centimeters or smaller. Cancer has not spread outside the breast. - In stage IB, small clusters of breast cancer cells (larger than 0.2 millimeter but not larger than 2 millimeters) are found in the lymph nodes and either: - no tumor is found in the breast; or - the tumor is 2 centimeters or smaller. Stage II Stage II is divided into stages IIA and IIB. - In stage IIA: - no tumor is found in the breast or the tumor is 2 centimeters or smaller. Cancer (larger than 2 millimeters) is found in 1 to 3 axillary lymph nodes or in the lymph nodes near the breastbone (found during a sentinel lymph node biopsy); or - the tumor is larger than 2 centimeters but not larger than 5 centimeters. Cancer has not spread to the lymph nodes. - In stage IIB, the tumor is: - larger than 2 centimeters but not larger than 5 centimeters. Small clusters of breast cancer cells (larger than 0.2 millimeter but not larger than 2 millimeters) are found in the lymph nodes; or - larger than 2 centimeters but not larger than 5 centimeters. Cancer has spread to 1 to 3 axillary lymph nodes or to the lymph nodes near the breastbone (found during a sentinel lymph node biopsy); or - larger than 5 centimeters. Cancer has not spread to the lymph nodes. Stage IIIA In stage IIIA: - no tumor is found in the breast or the tumor may be any size. Cancer is found in 4 to 9 axillary lymph nodes or in the lymph nodes near the breastbone (found during imaging tests or a physical exam); or - the tumor is larger than 5 centimeters. Small clusters of breast cancer cells (larger than 0.2 millimeter but not larger than 2 millimeters) are found in the lymph nodes; or - the tumor is larger than 5 centimeters. Cancer has spread to 1 to 3 axillary lymph nodes or to the lymph nodes near the breastbone (found during a sentinel lymph node biopsy). Stage IIIB In stage IIIB, the tumor may be any size and cancer has spread to the chest wall and/or to the skin of the breast and caused swelling or an ulcer. Also, cancer may have spread to: - up to 9 axillary lymph nodes; or - the lymph nodes near the breastbone. Cancer that has spread to the skin of the breast may also be inflammatory breast cancer. See the section on Inflammatory Breast Cancer for more information. Stage IIIC In stage IIIC, no tumor is found in the breast or the tumor may be any size. Cancer may have spread to the skin of the breast and caused swelling or an ulcer and/or has spread to the chest wall. Also, cancer has spread to: - 10 or more axillary lymph nodes; or - lymph nodes above or below the collarbone; or - axillary lymph nodes and lymph nodes near the breastbone. Cancer that has spread to the skin of the breast may also be inflammatory breast cancer. See the section on Inflammatory Breast Cancer for more information. Stage IV In stage IV, cancer has spread to other organs of the body, most often the bones, lungs, liver, or brain. The treatment of breast cancer depends partly on the stage of the disease. For ductal carcinoma in situ (DCIS) treatment options, see Ductal Carcinoma in Situ. For treatment options for stage I, stage II, stage IIIA, and operable stage IIIC breast cancer, see Early, Localized, or Operable Breast Cancer. For treatment options for stage IIIB, inoperable stage IIIC, and inflammatory breast cancer, see Locally Advanced or Inflammatory Breast Cancer. For treatment options for cancer that has recurred near the area where it first formed, see Locoregional Recurrent Breast Cancer. For treatment options for stage IV breast cancer or breast cancer that has recurred in other parts of the body, see Metastatic Breast Cancer.",Breast Cancer,0000027_1,CancerGov,https://www.cancer.gov/types/breast/patient/breast-treatment-pdq,C0006142,T191,Disorders What are the treatments for Breast Cancer ?,0000027_1-9,treatment,"Key Points - There are different types of treatment for patients with breast cancer. - Five types of standard treatment are used: - Surgery - Radiation therapy - Chemotherapy - Hormone therapy - Targeted therapy - New types of treatment are being tested in clinical trials. - High-dose chemotherapy with stem cell transplant - Treatment for breast cancer may cause side effects. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with breast cancer. Different types of treatment are available for patients with breast cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Five types of standard treatment are used: Surgery Most patients with breast cancer have surgery to remove the cancer. Sentinel lymph node biopsy is the removal of the sentinel lymph node during surgery. The sentinel lymph node is the first lymph node to receive lymphatic drainage from a tumor. It is the first lymph node where the cancer is likely to spread. A radioactive substance and/or blue dye is injected near the tumor. The substance or dye flows through the lymph ducts to the lymph nodes. The first lymph node to receive the substance or dye is removed. A pathologist views the tissue under a microscope to look for cancer cells. After the sentinel lymph node biopsy, the surgeon removes the tumor using breast-conserving surgery or mastectomy. If cancer cells were not found in the sentinel lymph node, it may not be necessary to remove more lymph nodes. If cancer cells were found, more lymph nodes will be removed through a separate incision. This is called a lymph node dissection. Types of surgery include the following: - Breast-conserving surgery is an operation to remove the cancer and some normal tissue around it, but not the breast itself. Part of the chest wall lining may also be removed if the cancer is near it. This type of surgery may also be called lumpectomy, partial mastectomy, segmental mastectomy, quadrantectomy, or breast-sparing surgery. - Total mastectomy: Surgery to remove the whole breast that has cancer. This procedure is also called a simple mastectomy. Some of the lymph nodes under the arm may be removed and checked for cancer. This may be done at the same time as the breast surgery or after. This is done through a separate incision. - Modified radical mastectomy: Surgery to remove the whole breast that has cancer, many of the lymph nodes under the arm, the lining over the chest muscles, and sometimes, part of the chest wall muscles. Chemotherapy may be given before surgery to remove the tumor. When given before surgery, chemotherapy will shrink the tumor and reduce the amount of tissue that needs to be removed during surgery. Treatment given before surgery is called preoperative therapy or neoadjuvant therapy. Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given radiation therapy, chemotherapy, or hormone therapy after surgery, to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called postoperative therapy or adjuvant therapy. If a patient is going to have a mastectomy, breast reconstruction (surgery to rebuild a breasts shape after a mastectomy) may be considered. Breast reconstruction may be done at the time of the mastectomy or at some time after. The reconstructed breast may be made with the patients own (nonbreast) tissue or by using implants filled with saline or silicone gel. Before the decision to get an implant is made, patients can call the Food and Drug Administration's (FDA) Center for Devices and Radiologic Health at 1-888-INFO-FDA (1-888-463-6332) or visit the FDA website for more information on breast implants. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External radiation therapy is used to treat breast cancer. Internal radiation therapy with strontium-89 (a radionuclide) is used to relieve bone pain caused by breast cancer that has spread to the bones. Strontium-89 is injected into a vein and travels to the surface of the bones. Radiation is released and kills cancer cells in the bones. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. Systemic chemotherapy is used in the treatment of breast cancer. See Drugs Approved for Breast Cancer for more information. Hormone therapy Hormone therapy is a cancer treatment that removes hormones or blocks their action and stops cancer cells from growing. Hormones are substances made by glands in the body and circulated in the bloodstream. Some hormones can cause certain cancers to grow. If tests show that the cancer cells have places where hormones can attach (receptors), drugs, surgery, or radiation therapy is used to reduce the production of hormones or block them from working. The hormone estrogen, which makes some breast cancers grow, is made mainly by the ovaries. Treatment to stop the ovaries from making estrogen is called ovarian ablation. Hormone therapy with tamoxifen is often given to patients with early localized breast cancer that can be removed by surgery and those with metastatic breast cancer (cancer that has spread to other parts of the body). Hormone therapy with tamoxifen or estrogens can act on cells all over the body and may increase the chance of developing endometrial cancer. Women taking tamoxifen should have a pelvic exam every year to look for any signs of cancer. Any vaginal bleeding, other than menstrual bleeding, should be reported to a doctor as soon as possible. Hormone therapy with a luteinizing hormone-releasing hormone (LHRH) agonist is given to some premenopausal women who have just been diagnosed with hormone receptor positive breast cancer. LHRH agonists decrease the body's estrogen and progesterone. Hormone therapy with an aromatase inhibitor is given to some postmenopausal women who have hormone receptor positive breast cancer. Aromatase inhibitors decrease the body's estrogen by blocking an enzyme called aromatase from turning androgen into estrogen. Anastrozole, letrozole, and exemestane are types of aromatase inhibitors. For the treatment of early localized breast cancer that can be removed by surgery, certain aromatase inhibitors may be used as adjuvant therapy instead of tamoxifen or after 2 to 3 years of tamoxifen use. For the treatment of metastatic breast cancer, aromatase inhibitors are being tested in clinical trials to compare them to hormone therapy with tamoxifen. Other types of hormone therapy include megestrol acetate or anti-estrogen therapy such as fulvestrant. See Drugs Approved for Breast Cancer for more information. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Monoclonal antibodies, tyrosine kinase inhibitors, cyclin-dependent kinase inhibitors, mammalian target of rapamycin (mTOR) inhibitors, and PARP inhibitors are types of targeted therapies used in the treatment of breast cancer. Monoclonal antibody therapy is a cancer treatment that uses antibodies made in the laboratory, from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Monoclonal antibodies may be used in combination with chemotherapy as adjuvant therapy. Types of monoclonal antibody therapy include the following: - Trastuzumab is a monoclonal antibody that blocks the effects of the growth factor protein HER2, which sends growth signals to breast cancer cells. It may be used with other therapies to treat HER2 positive breast cancer. - Pertuzumab is a monoclonal antibody that may be combined with trastuzumab and chemotherapy to treat breast cancer. It may be used to treat certain patients with HER2 positive breast cancer that has metastasized (spread to other parts of the body). It may also be used as neoadjuvant therapy in certain patients with early stage HER2 positive breast cancer. - Ado-trastuzumab emtansine is a monoclonal antibody linked to an anticancer drug. This is called an antibody-drug conjugate. It is used to treat HER2 positive breast cancer that has spread to other parts of the body or recurred (come back). Tyrosine kinase inhibitors are targeted therapy drugs that block signals needed for tumors to grow. Tyrosine kinase inhibitors may be used with other anticancer drugs as adjuvant therapy. Tyrosine kinase inhibitors include the following: - Lapatinib is a tyrosine kinase inhibitor that blocks the effects of the HER2 protein and other proteins inside tumor cells. It may be used with other drugs to treat patients with HER2 positive breast cancer that has progressed after treatment with trastuzumab. Cyclin-dependent kinase inhibitors are targeted therapy drugs that block proteins called cyclin-dependent kinases, which cause the growth of cancer cells. Cyclin-dependent kinase inhibitors include the following: - Palbociclib is a cyclin-dependent kinase inhibitor used with the drug letrozole to treat breast cancer that is estrogen receptor positive and HER2 negative and has spread to other parts of the body. It is used in postmenopausal women whose cancer has not been treated with hormone therapy. Palbociclib may also be used with fulvestrant in women whose disease has gotten worse after treatment with hormone therapy. - Ribociclib is a cyclin-dependent kinase inhibitor used with letrozole to treat breast cancer that is hormone receptor positive and HER2 negative and has come back or spread to other parts of the body. It is used in postmenopausal women whose cancer has not been treated with hormone therapy. Mammalian target of rapamycin (mTOR) inhibitors block a protein called mTOR, which may keep cancer cells from growing and prevent the growth of new blood vessels that tumors need to grow. mTOR inhibitors include the following: - Everolimus is an mTOR inhibitor used in postmenopausal women with advanced hormone receptor positive breast cancer that is also HER2 negative and has not gotten better with other treatment. PARP inhibitors are a type of targeted therapy that block DNA repair and may cause cancer cells to die. PARP inhibitor therapy is being studied for the treatment of patients with triple negative breast cancer or tumors with BRCA1 or BRCA2 mutations. See Drugs Approved for Breast Cancer for more information. New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. High-dose chemotherapy with stem cell transplant High-dose chemotherapy with stem cell transplant is a way of giving high doses of chemotherapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the bodys blood cells. Studies have shown that high-dose chemotherapy followed by stem cell transplant does not work better than standard chemotherapy in the treatment of breast cancer. Doctors have decided that, for now, high-dose chemotherapy should be tested only in clinical trials. Before taking part in such a trial, women should talk with their doctors about the serious side effects, including death, that may be caused by high-dose chemotherapy. Treatment for breast cancer may cause side effects. For information about side effects that begin during treatment for cancer, see our Side Effects page. Some treatments for breast cancer may cause side effects that continue or appear months or years after treatment has ended. These are called late effects. Late effects of radiation therapy are not common, but may include: - Inflammation of the lung after radiation therapy to the breast, especially when chemotherapy is given at the same time. - Arm lymphedema, especially when radiation therapy is given after lymph node dissection. - In women younger than 45 years who receive radiation therapy to the chest wall after mastectomy, there may be a higher risk of developing breast cancer in the other breast. Late effects of chemotherapy depend on the drugs used, but may include: - Heart failure. - Blood clots. - Premature menopause. - Second cancer, such as leukemia. Late effects of targeted therapy with trastuzumab, lapatinib, or pertuzumab may include: - Heart problems such as heart failure. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Breast Cancer Early, Localized, or Operable Breast Cancer Treatment of early, localized, or operable breast cancer may include the following: Surgery - Breast-conserving surgery and sentinel lymph node biopsy. If cancer is found in the lymph nodes, a lymph node dissection may be done. - Modified radical mastectomy. Breast reconstruction surgery may also be done. Postoperative radiation therapy For women who had breast-conserving surgery, radiation therapy is given to the whole breast to lessen the chance the cancer will come back. Radiation therapy may also be given to lymph nodes in the area. For women who had a modified radical mastectomy, radiation therapy may be given to lessen the chance the cancer will come back if any of the following are true: - Cancer was found in 4 or more lymph nodes. - Cancer had spread to tissue around the lymph nodes. - The tumor was large. - There is tumor close to or remaining in the tissue near the edges of where the tumor was removed. Postoperative systemic therapy Systemic therapy is the use of drugs that can enter the bloodstream and reach cancer cells throughout the body. Postoperative systemic therapy is given to lessen the chance the cancer will come back after surgery to remove the tumor. Postoperative systemic therapy is given depending on whether: - The tumor is hormone receptor negative or positive. - The tumor is HER2/neu negative or positive. - The tumor is hormone receptor negative and HER2/neu negative (triple negative). - The size of the tumor. In premenopausal women with hormone receptor positive tumors, no more treatment may be needed or postoperative therapy may include: - Tamoxifen therapy with or without chemotherapy. - Tamoxifen therapy and treatment to stop or lessen how much estrogen is made by the ovaries. Drug therapy, surgery to remove the ovaries, or radiation therapy to the ovaries may be used. - Aromatase inhibitor therapy and treatment to stop or lessen how much estrogen is made by the ovaries. Drug therapy, surgery to remove the ovaries, or radiation therapy to the ovaries may be used. In postmenopausal women with hormone receptor positive tumors, no more treatment may be needed or postoperative therapy may include: - Aromatase inhibitor therapy with or without chemotherapy. - Tamoxifen followed by aromatase inhibitor therapy, with or without chemotherapy. In women with hormone receptor negative tumors, no more treatment may be needed or postoperative therapy may include: - Chemotherapy. In women with HER2/neu negative tumors, postoperative therapy may include: - Chemotherapy. In women with small, HER2/neu positive tumors, and no cancer in the lymph nodes, no more treatment may be needed. If there is cancer in the lymph nodes, or the tumor is large, postoperative therapy may include: - Chemotherapy and targeted therapy (trastuzumab). - Hormone therapy, such as tamoxifen or aromatase inhibitor therapy, for tumors that are also hormone receptor positive. In women with small, hormone receptor negative and HER2/neu negative tumors (triple negative) and no cancer in the lymph nodes, no more treatment may be needed. If there is cancer in the lymph nodes or the tumor is large, postoperative therapy may include: - Chemotherapy. - Radiation therapy. - A clinical trial of a new chemotherapy regimen. - A clinical trial of PARP inhibitor therapy. Preoperative systemic therapy Systemic therapy is the use of drugs that can enter the bloodstream and reach cancer cells throughout the body. Preoperative systemic therapy is given to shrink the tumor before surgery. In postmenopausal women with hormone receptor positive tumors, preoperative therapy may include: - Chemotherapy. - Hormone therapy, such as tamoxifen or aromatase inhibitor therapy, for women who cannot have chemotherapy. In premenopausal women with hormone receptor positive tumors, preoperative therapy may include: - A clinical trial of hormone therapy, such as tamoxifen or aromatase inhibitor therapy. In women with HER2/neu positive tumors, preoperative therapy may include: - Chemotherapy and targeted therapy (trastuzumab). - Targeted therapy (pertuzumab). In women with HER2/neu negative tumors or triple negative tumors, preoperative therapy may include: - Chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I breast cancer, stage II breast cancer, stage IIIA breast cancer and stage IIIC breast cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Locally Advanced or Inflammatory Breast Cancer Treatment of locally advanced or inflammatory breast cancer is a combination of therapies that may include the following: - Surgery (breast-conserving surgery or total mastectomy) with lymph node dissection. - Chemotherapy before and/or after surgery. - Radiation therapy after surgery. - Hormone therapy after surgery for tumors that are estrogen receptor positive or estrogen receptor unknown. - Clinical trials testing new anticancer drugs, new drug combinations, and new ways of giving treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage IIIB breast cancer, stage IIIC breast cancer, stage IV breast cancer and inflammatory breast cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Locoregional Recurrent Breast Cancer Treatment of locoregional recurrent breast cancer (cancer that has come back after treatment in the breast, in the chest wall, or in nearby lymph nodes), may include the following: - Chemotherapy. - Hormone therapy for tumors that are hormone receptor positive. - Radiation therapy. - Surgery. - Targeted therapy (trastuzumab). - A clinical trial of a new treatment. See the Metastatic Breast Cancer section for information about treatment options for breast cancer that has spread to parts of the body outside the breast, chest wall, or nearby lymph nodes. Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent breast cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Metastatic Breast Cancer Treatment options for metastatic breast cancer (cancer that has spread to distant parts of the body) may include the following: Hormone therapy In postmenopausal women who have just been diagnosed with metastatic breast cancer that is hormone receptor positive or if the hormone receptor status is not known, treatment may include: - Tamoxifen therapy. - Aromatase inhibitor therapy (anastrozole, letrozole, or exemestane). Sometimes cyclin-dependent kinase inhibitor therapy (palbociclib) is also given. In premenopausal women who have just been diagnosed with metastatic breast cancer that is hormone receptor positive, treatment may include: - Tamoxifen, an LHRH agonist, or both. In women whose tumors are hormone receptor positive or hormone receptor unknown, with spread to the bone or soft tissue only, and who have been treated with tamoxifen, treatment may include: - Aromatase inhibitor therapy. - Other hormone therapy such as megestrol acetate, estrogen or androgen therapy, or anti-estrogen therapy such as fulvestrant. Targeted therapy In women with metastatic breast cancer that is hormone receptor positive and has not responded to other treatments, options may include targeted therapy such as: - Trastuzumab, lapatinib, pertuzumab, or mTOR inhibitors. - Antibody-drug conjugate therapy with ado-trastuzumab emtansine. - Cyclin-dependent kinase inhibitor therapy (palbociclib) combined with letrozole. In women with metastatic breast cancer that is HER2/neu positive, treatment may include: - Targeted therapy such as trastuzumab, pertuzumab, ado-trastuzumab emtansine, or lapatinib. Chemotherapy In women with metastatic breast cancer that is hormone receptor negative, has not responded to hormone therapy, has spread to other organs or has caused symptoms, treatment may include: - Chemotherapy with one or more drugs. Surgery - Total mastectomy for women with open or painful breast lesions. Radiation therapy may be given after surgery. - Surgery to remove cancer that has spread to the brain or spine. Radiation therapy may be given after surgery. - Surgery to remove cancer that has spread to the lung. - Surgery to repair or help support weak or broken bones. Radiation therapy may be given after surgery. - Surgery to remove fluid that has collected around the lungs or heart. Radiation therapy - Radiation therapy to the bones, brain, spinal cord, breast, or chest wall to relieve symptoms and improve quality of life. - Strontium-89 (a radionuclide) to relieve pain from cancer that has spread to bones throughout the body. Other treatment options Other treatment options for metastatic breast cancer include: - Drug therapy with bisphosphonates or denosumab to reduce bone disease and pain when cancer has spread to the bone. (See the PDQ summary on Cancer Pain for more information about bisphosphonates.) - A clinical trial of high-dose chemotherapy with stem cell transplant. - Clinical trials testing new anticancer drugs, new drug combinations, and new ways of giving treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with metastatic cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Breast Cancer,0000027_1,CancerGov,https://www.cancer.gov/types/breast/patient/breast-treatment-pdq,C0006142,T191,Disorders what research (or clinical trials) is being done for Breast Cancer ?,0000027_1-10,research,"New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. High-dose chemotherapy with stem cell transplant High-dose chemotherapy with stem cell transplant is a way of giving high doses of chemotherapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the bodys blood cells. Studies have shown that high-dose chemotherapy followed by stem cell transplant does not work better than standard chemotherapy in the treatment of breast cancer. Doctors have decided that, for now, high-dose chemotherapy should be tested only in clinical trials. Before taking part in such a trial, women should talk with their doctors about the serious side effects, including death, that may be caused by high-dose chemotherapy. Treatment for breast cancer may cause side effects. For information about side effects that begin during treatment for cancer, see our Side Effects page. Some treatments for breast cancer may cause side effects that continue or appear months or years after treatment has ended. These are called late effects. Late effects of radiation therapy are not common, but may include: - Inflammation of the lung after radiation therapy to the breast, especially when chemotherapy is given at the same time. - Arm lymphedema, especially when radiation therapy is given after lymph node dissection. - In women younger than 45 years who receive radiation therapy to the chest wall after mastectomy, there may be a higher risk of developing breast cancer in the other breast. Late effects of chemotherapy depend on the drugs used, but may include: - Heart failure. - Blood clots. - Premature menopause. - Second cancer, such as leukemia. Late effects of targeted therapy with trastuzumab, lapatinib, or pertuzumab may include: - Heart problems such as heart failure. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Breast Cancer,0000027_1,CancerGov,https://www.cancer.gov/types/breast/patient/breast-treatment-pdq,C0006142,T191,Disorders What is (are) Male Breast Cancer ?,0000027_2-1,information,"Key Points - Male breast cancer is a disease in which malignant (cancer) cells form in the tissues of the breast. - Radiation exposure, high levels of estrogen, and a family history of breast cancer can increase a mans risk of breast cancer. - Male breast cancer is sometimes caused by inherited gene mutations (changes). - Men with breast cancer usually have lumps that can be felt. - Tests that examine the breasts are used to detect (find) and diagnose breast cancer in men. - If cancer is found, tests are done to study the cancer cells. - Survival for men with breast cancer is similar to survival for women with breast cancer. - Certain factors affect prognosis (chance of recovery) and treatment options. Male breast cancer is a disease in which malignant (cancer) cells form in the tissues of the breast. Breast cancer may occur in men. Men at any age may develop breast cancer, but it is usually detected (found) in men between 60 and 70 years of age. Male breast cancer makes up less than 1% of all cases of breast cancer. The following types of breast cancer are found in men: - Infiltrating ductal carcinoma: Cancer that has spread beyond the cells lining ducts in the breast. Most men with breast cancer have this type of cancer. - Ductal carcinoma in situ: Abnormal cells that are found in the lining of a duct; also called intraductal carcinoma. - Inflammatory breast cancer: A type of cancer in which the breast looks red and swollen and feels warm. - Paget disease of the nipple: A tumor that has grown from ducts beneath the nipple onto the surface of the nipple. Lobular carcinoma in situ (abnormal cells found in one of the lobes or sections of the breast), which sometimes occurs in women, has not been seen in men. Men with breast cancer usually have lumps that can be felt. Lumps and other signs may be caused by male breast cancer or by other conditions. Check with your doctor if you notice a change in your breasts.",Male Breast Cancer,0000027_2,CancerGov,https://www.cancer.gov/types/breast/patient/male-breast-treatment-pdq,C0242787,T191,Disorders Who is at risk for Male Breast Cancer? ?,0000027_2-2,susceptibility,"Radiation exposure, high levels of estrogen, and a family history of breast cancer can increase a mans risk of breast cancer. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesnt mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for breast cancer in men may include the following: - Being exposed to radiation. - Having a disease linked to high levels of estrogen in the body, such as cirrhosis (liver disease) or Klinefelter syndrome (a genetic disorder.) - Having several female relatives who have had breast cancer, especially relatives who have an alteration of the BRCA2 gene.",Male Breast Cancer,0000027_2,CancerGov,https://www.cancer.gov/types/breast/patient/male-breast-treatment-pdq,C0242787,T191,Disorders Is Male Breast Cancer inherited ?,0000027_2-3,inheritance,Male breast cancer is sometimes caused by inherited gene mutations (changes).The genes in cells carry the hereditary information that is received from a persons parents. Hereditary breast cancer makes up about 5% to 10% of all breast cancer. Some mutated genes related to breast cancer are more common in certain ethnic groups. Men who have a mutated gene related to breast cancer have an increased risk of this disease. There are tests that can detect (find) mutated genes. These genetic tests are sometimes done for members of families with a high risk of cancer. See the following PDQ summaries for more information: - Genetics of Breast and Gynecologic Cancers - Breast Cancer Prevention - Breast Cancer Screening,Male Breast Cancer,0000027_2,CancerGov,https://www.cancer.gov/types/breast/patient/male-breast-treatment-pdq,C0242787,T191,Disorders What are the symptoms of Male Breast Cancer ?,0000027_2-4,symptoms,Men with breast cancer usually have lumps that can be felt.Lumps and other signs may be caused by male breast cancer or by other conditions. Check with your doctor if you notice a change in your breasts.,Male Breast Cancer,0000027_2,CancerGov,https://www.cancer.gov/types/breast/patient/male-breast-treatment-pdq,C0242787,T191,Disorders How to diagnose Male Breast Cancer ?,0000027_2-5,exams and tests,"Tests that examine the breasts are used to detect (find) and diagnose breast cancer in men. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Clinical breast exam (CBE): An exam of the breast by a doctor or other health professional. The doctor will carefully feel the breasts and under the arms for lumps or anything else that seems unusual. - Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. - Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. The following are different types of biopsies: - Fine-needle aspiration (FNA) biopsy : The removal of tissue or fluid using a thin needle. - Core biopsy : The removal of tissue using a wide needle. - Excisional biopsy : The removal of an entire lump of tissue. If cancer is found, tests are done to study the cancer cells. Decisions about the best treatment are based on the results of these tests. The tests give information about: - How quickly the cancer may grow. - How likely it is that the cancer will spread through the body. - How well certain treatments might work. - How likely the cancer is to recur (come back). Tests include the following: - Estrogen and progesterone receptor test : A test to measure the amount of estrogen and progesterone (hormones) receptors in cancer tissue. If cancer is found in the breast, tissue from the tumor is checked in the laboratory to find out whether estrogen and progesterone could affect the way cancer grows. The test results show whether hormone therapy may stop the cancer from growing. - HER2 test: A test to measure the amount of HER2 in cancer tissue. HER2 is a growth factor protein that sends growth signals to cells. When cancer forms, the cells may make too much of the protein, causing more cancer cells to grow. If cancer is found in the breast, tissue from the tumor is checked in the laboratory to find out if there is too much HER2 in the cells. The test results show whether monoclonal antibody therapy may stop the cancer from growing.",Male Breast Cancer,0000027_2,CancerGov,https://www.cancer.gov/types/breast/patient/male-breast-treatment-pdq,C0242787,T191,Disorders What is the outlook for Male Breast Cancer ?,0000027_2-6,outlook,"Survival for men with breast cancer is similar to survival for women with breast cancer. Survival for men with breast cancer is similar to that for women with breast cancer when their stage at diagnosis is the same. Breast cancer in men, however, is often diagnosed at a later stage. Cancer found at a later stage may be less likely to be cured. Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - The stage of the cancer (whether it is in the breast only or has spread to other places in the body). - The type of breast cancer. - Estrogen-receptor and progesterone-receptor levels in the tumor tissue. - Whether the cancer is also found in the other breast. - The patients age and general health.",Male Breast Cancer,0000027_2,CancerGov,https://www.cancer.gov/types/breast/patient/male-breast-treatment-pdq,C0242787,T191,Disorders What are the stages of Male Breast Cancer ?,0000027_2-7,stages,"Key Points - After breast cancer has been diagnosed, tests are done to find out if cancer cells have spread within the breast or to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - The following stages are used for male breast cancer: - Stage 0 (carcinoma in situ) - Stage I - Stage II - Stage IIIA - Stage IIIB - Stage IIIC - Stage IV After breast cancer has been diagnosed, tests are done to find out if cancer cells have spread within the breast or to other parts of the body. After breast cancer has been diagnosed, tests are done to find out if cancer cells have spread within the breast or to other parts of the body. This process is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. Breast cancer in men is staged the same as it is in women. The spread of cancer from the breast to lymph nodes and other parts of the body appears to be similar in men and women. The following tests and procedures may be used in the staging process: - Sentinel lymph node biopsy : The removal of the sentinel lymph node during surgery. The sentinel lymph node is the first lymph node to receive lymphatic drainage from a tumor. It is the first lymph node the cancer is likely to spread to from the tumor. A radioactive substance and/or blue dye is injected near the tumor. The substance or dye flows through the lymph ducts to the lymph nodes. The first lymph node to receive the substance or dye is removed. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are not found, it may not be necessary to remove more lymph nodes. - Chest x-ray : An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - Bone scan : A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones and is detected by a scanner. - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if breast cancer spreads to the bone, the cancer cells in the bone are actually breast cancer cells. The disease is metastatic breast cancer, not bone cancer. The following stages are used for male breast cancer: This section describes the stages of breast cancer. The breast cancer stage is based on the results of testing that is done on the tumor and lymph nodes removed during surgery and other tests. Stage 0 (carcinoma in situ) There are 3 types of breast carcinoma in situ: - Ductal carcinoma in situ (DCIS) is a noninvasive condition in which abnormal cells are found in the lining of a breast duct. The abnormal cells have not spread outside the duct to other tissues in the breast. In some cases, DCIS may become invasive cancer and spread to other tissues. At this time, there is no way to know which lesions could become invasive. - Paget disease of the nipple is a condition in which abnormal cells are found in the nipple only. - Lobular carcinoma in situ (LCIS) is a condition in which abnormal cells are found in the lobules of the breast. This condition has not been seen in men. Stage I In stage I, cancer has formed. Stage I is divided into stages IA and IB. - In stage IA, the tumor is 2 centimeters or smaller. Cancer has not spread outside the breast. - In stage IB, small clusters of breast cancer cells (larger than 0.2 millimeter but not larger than 2 millimeters) are found in the lymph nodes and either: - no tumor is found in the breast; or - the tumor is 2 centimeters or smaller. Stage II Stage II is divided into stages IIA and IIB. - In stage IIA - no tumor is found in the breast or the tumor is 2 centimeters or smaller. Cancer (larger than 2 millimeters) is found in 1 to 3 axillary lymph nodes or in the lymph nodes near the breastbone (found during a sentinel lymph node biopsy); or - the tumor is larger than 2 centimeters but not larger than 5 centimeters. Cancer has not spread to the lymph nodes. - In stage IIB, the tumor is: - larger than 2 centimeters but not larger than 5 centimeters. Small clusters of breast cancer cells (larger than 0.2 millimeter but not larger than 2 millimeters) are found in the lymph nodes; or - larger than 2 centimeters but not larger than 5 centimeters. Cancer has spread to 1 to 3 axillary lymph nodes or to the lymph nodes near the breastbone (found during a sentinel lymph node biopsy); or - larger than 5 centimeters. Cancer has not spread to the lymph nodes. Stage IIIA In stage IIIA: - no tumor is found in the breast or the tumor may be any size. Cancer is found in 4 to 9 axillary lymph nodes or in the lymph nodes near the breastbone (found during imaging tests or a physical exam); or - the tumor is larger than 5 centimeters. Small clusters of breast cancer cells (larger than 0.2 millimeter but not larger than 2 millimeters) are found in the lymph nodes; or - the tumor is larger than 5 centimeters. Cancer has spread to 1 to 3 axillary lymph nodes or to the lymph nodes near the breastbone (found during a sentinel lymph node biopsy). Stage IIIB In stage IIIB, the tumor may be any size and cancer has spread to the chest wall and/or to the skin of the breast and caused swelling or an ulcer. Also, cancer may have spread to : - up to 9 axillary lymph nodes; or - the lymph nodes near the breastbone. Cancer that has spread to the skin of the breast may also be inflammatory breast cancer. See the section on Inflammatory Male Breast Cancer for more information. Stage IIIC In stage IIIC, no tumor is found in the breast or the tumor may be any size. Cancer may have spread to the skin of the breast and caused swelling or an ulcer and/or has spread to the chest wall. Also, cancer has spread to: - 10 or more axillary lymph nodes; or - lymph nodes above or below the collarbone; or - axillary lymph nodes and lymph nodes near the breastbone. Cancer that has spread to the skin of the breast may also be inflammatory breast cancer. See the section on Inflammatory Male Breast Cancer for more information. For treatment, stage IIIC breast cancer is divided into operable and inoperable stage IIIC. Stage IV In stage IV, cancer has spread to other organs of the body, most often the bones, lungs, liver, or brain.",Male Breast Cancer,0000027_2,CancerGov,https://www.cancer.gov/types/breast/patient/male-breast-treatment-pdq,C0242787,T191,Disorders What are the treatments for Male Breast Cancer ?,0000027_2-8,treatment,"Key Points - There are different types of treatment for men with breast cancer. - Five types of standard treatment are used to treat men with breast cancer: - Surgery - Chemotherapy - Hormone therapy - Radiation therapy - Targeted therapy - Treatment for male breast cancer may cause side effects. There are different types of treatment for men with breast cancer. Different types of treatment are available for men with breast cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. For some patients, taking part in a clinical trial may be the best treatment choice. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. Information about clinical trials is available from the NCI website. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team. Five types of standard treatment are used to treat men with breast cancer: Surgery Surgery for men with breast cancer is usually a modified radical mastectomy (removal of the breast, many of the lymph nodes under the arm, the lining over the chest muscles, and sometimes part of the chest wall muscles). Breast-conserving surgery, an operation to remove the cancer but not the breast itself, is also used for some men with breast cancer. A lumpectomy is done to remove the tumor (lump) and a small amount of normal tissue around it. Radiation therapy is given after surgery to kill any cancer cells that are left. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. See Drugs Approved for Breast Cancer for more information. Hormone therapy Hormone therapy is a cancer treatment that removes hormones or blocks their action and stops cancer cells from growing. Hormones are substances made by glands in the body and circulated in the bloodstream. Some hormones can cause certain cancers to grow. If tests show that the cancer cells have places where hormones can attach (receptors), drugs, surgery, or radiation therapy is used to reduce the production of hormones or block them from working. See Drugs Approved for Breast Cancer for more information. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External radiation therapy is used to treat male breast cancer. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Monoclonal antibody therapy is a type of targeted therapy used to treat men with breast cancer. Monoclonal antibody therapy uses antibodies made in the laboratory from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Monoclonal antibodies are also used with chemotherapy as adjuvant therapy (treatment given after surgery to lower the risk that the cancer will come back). Trastuzumab is a monoclonal antibody that blocks the effects of the growth factor protein HER2. See Drugs Approved for Breast Cancer for more information. Treatment for male breast cancer may cause side effects. For information about side effects caused by treatment for cancer, see our Side Effects page. Treatment Options for Male Breast Cancer Initial Surgery Treatment for men diagnosed with breast cancer is usually modified radical mastectomy. Breast-conserving surgery with lumpectomy may be used for some men. Adjuvant Therapy Therapy given after an operation when cancer cells can no longer be seen is called adjuvant therapy. Even if the doctor removes all the cancer that can be seen at the time of the operation, the patient may be given radiation therapy, chemotherapy, hormone therapy, and/or targeted therapy after surgery, to try to kill any cancer cells that may be left. - Node-negative: For men whose cancer is node-negative (cancer has not spread to the lymph nodes), adjuvant therapy should be considered on the same basis as for a woman with breast cancer because there is no evidence that response to therapy is different for men and women. - Node-positive: For men whose cancer is node-positive (cancer has spread to the lymph nodes), adjuvant therapy may include the following: - Chemotherapy plus tamoxifen (to block the effect of estrogen). - Other hormone therapy. - Targeted therapy with a monoclonal antibody (trastuzumab). These treatments appear to increase survival in men as they do in women. The patients response to hormone therapy depends on whether there are hormone receptors (proteins) in the tumor. Most breast cancers in men have these receptors. Hormone therapy is usually recommended for male breast cancer patients, but it can have many side effects, including hot flashes and impotence (the inability to have an erection adequate for sexual intercourse). Distant Metastases Treatment for men with distant metastases (cancer that has spread to other parts of the body) may be hormone therapy, chemotherapy, or both. Hormone therapy may include the following: - Orchiectomy (the removal of the testicles to decrease the amount of hormone made). - Luteinizing hormone-releasing hormone agonist with or without total androgen blockade (to decrease the the amount of sex hormones made). - Tamoxifen for cancer that is estrogen-receptor positive. - Progestin (a female hormone made in a laboratory). - Aromatase inhibitors (to decrease the amount of estrogen made). Hormone therapies may be used in sequence (one after the other). Standard chemotherapy regimens may be used if hormone therapy does not work. Men usually respond to therapy in the same way as women who have breast cancer.",Male Breast Cancer,0000027_2,CancerGov,https://www.cancer.gov/types/breast/patient/male-breast-treatment-pdq,C0242787,T191,Disorders What is (are) Breast Cancer ?,0000027_3-1,information,"Key Points - Breast cancer is a disease in which malignant (cancer) cells form in the tissues of the breast. - Sometimes breast cancer occurs in women who are pregnant or have just given birth. - Signs of breast cancer include a lump or change in the breast. - It may be difficult to detect (find) breast cancer early in pregnant or nursing women. - Breast exams should be part of prenatal and postnatal care. - Tests that examine the breasts are used to detect (find) and diagnose breast cancer. - If cancer is found, tests are done to study the cancer cells. - Certain factors affect prognosis (chance of recovery) and treatment options. Breast cancer is a disease in which malignant (cancer) cells form in the tissues of the breast. The breast is made up of lobes and ducts. Each breast has 15 to 20 sections called lobes. Each lobe has many smaller sections called lobules. Lobules end in dozens of tiny bulbs that can make milk. The lobes, lobules, and bulbs are linked by thin tubes called ducts. Each breast also has blood vessels and lymph vessels. The lymph vessels carry an almost colorless fluid called lymph. Lymph vessels carry lymph between lymph nodes. Lymph nodes are small bean-shaped structures that are found throughout the body. They filter substances in lymph and help fight infection and disease. Clusters of lymph nodes are found near the breast in the axilla (under the arm), above the collarbone, and in the chest. It may be difficult to detect (find) breast cancer early in pregnant or nursing women. The breasts usually get larger, tender, or lumpy in women who are pregnant, nursing, or have just given birth. This occurs because of normal hormone changes that take place during pregnancy. These changes can make small lumps difficult to detect. The breasts may also become denser. It is more difficult to detect breast cancer in women with dense breasts using mammography. Because these breast changes can delay diagnosis, breast cancer is often found at a later stage in these women. Other Information About Pregnancy and Breast Cancer Key Points - Lactation (breast milk production) and breast-feeding should be stopped if surgery or chemotherapy is planned. - Breast cancer does not appear to harm the unborn baby. - Pregnancy does not seem to affect the survival of women who have had breast cancer in the past. Lactation (breast milk production) and breast-feeding should be stopped if surgery or chemotherapy is planned. If surgery is planned, breast-feeding should be stopped to reduce blood flow in the breasts and make them smaller. Breast-feeding should also be stopped if chemotherapy is planned. Many anticancer drugs, especially cyclophosphamide and methotrexate, may occur in high levels in breast milk and may harm the nursing baby. Women receiving chemotherapy should not breast-feed. Stopping lactation does not improve the mother's prognosis. Breast cancer does not appear to harm the unborn baby. Breast cancer cells do not seem to pass from the mother to the unborn baby. Pregnancy does not seem to affect the survival of women who have had breast cancer in the past. For women who have had breast cancer, pregnancy does not seem to affect their survival. However, some doctors recommend that a woman wait 2 years after treatment for breast cancer before trying to have a baby, so that any early return of the cancer would be detected. This may affect a womans decision to become pregnant. The unborn baby does not seem to be affected if the mother has had breast cancer.",Breast Cancer,0000027_3,CancerGov,https://www.cancer.gov/types/breast/patient/pregnancy-breast-treatment-pdq,C0006142,T191,Disorders Who is at risk for Breast Cancer? ?,0000027_3-2,susceptibility,"Sometimes breast cancer occurs in women who are pregnant or have just given birth. Breast cancer occurs about once in every 3,000 pregnancies. It occurs most often between the ages of 32 and 38.",Breast Cancer,0000027_3,CancerGov,https://www.cancer.gov/types/breast/patient/pregnancy-breast-treatment-pdq,C0006142,T191,Disorders What are the symptoms of Breast Cancer ?,0000027_3-3,symptoms,"Signs of breast cancer include a lump or change in the breast. These and other signs may be caused by breast cancer or by other conditions. Check with your doctor if you have any of the following: - A lump or thickening in or near the breast or in the underarm area. - A change in the size or shape of the breast. - A dimple or puckering in the skin of the breast. - A nipple turned inward into the breast. - Fluid, other than breast milk, from the nipple, especially if it's bloody. - Scaly, red, or swollen skin on the breast, nipple, or areola (the dark area of skin around the nipple). - Dimples in the breast that look like the skin of an orange, called peau dorange. It may be difficult to detect (find) breast cancer early in pregnant or nursing women. The breasts usually get larger, tender, or lumpy in women who are pregnant, nursing, or have just given birth. This occurs because of normal hormone changes that take place during pregnancy. These changes can make small lumps difficult to detect. The breasts may also become denser. It is more difficult to detect breast cancer in women with dense breasts using mammography. Because these breast changes can delay diagnosis, breast cancer is often found at a later stage in these women.",Breast Cancer,0000027_3,CancerGov,https://www.cancer.gov/types/breast/patient/pregnancy-breast-treatment-pdq,C0006142,T191,Disorders How to diagnose Breast Cancer ?,0000027_3-4,exams and tests,"Breast exams should be part of prenatal and postnatal care. To detect breast cancer, pregnant and nursing women should examine their breasts themselves. Women should also receive clinical breast exams during their regular prenatal and postnatal check-ups. Talk to your doctor if you notice any changes in your breasts that you do not expect or that worry you. Tests that examine the breasts are used to detect (find) and diagnose breast cancer. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Clinical breast exam (CBE): An exam of the breast by a doctor or other health professional. The doctor will carefully feel the breasts and under the arms for lumps or anything else that seems unusual. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of both breasts. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to look at later. - Mammogram : An x-ray of the breast. A mammogram can be done with little risk to the unborn baby. Mammograms in pregnant women may appear negative even though cancer is present. - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. - Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. If a lump in the breast is found, a biopsy may be done. There are four types of breast biopsies: - Excisional biopsy : The removal of an entire lump of tissue. - Incisional biopsy : The removal of part of a lump or a sample of tissue. - Core biopsy : The removal of tissue using a wide needle. - Fine-needle aspiration (FNA) biopsy : The removal of tissue or fluid, using a thin needle. If cancer is found, tests are done to study the cancer cells. Decisions about the best treatment are based on the results of these tests and the age of the unborn baby. The tests give information about: - How quickly the cancer may grow. - How likely it is that the cancer will spread to other parts of the body. - How well certain treatments might work. - How likely the cancer is to recur (come back). Tests may include the following: - Estrogen and progesterone receptor test : A test to measure the amount of estrogen and progesterone (hormones) receptors in cancer tissue. If there are more estrogen and progesterone receptors than normal, the cancer is called estrogen and/or progesterone receptor positive. This type of breast cancer may grow more quickly. The test results show whether treatment to block estrogen and progesterone given after the baby is born may stop the cancer from growing. - Human epidermal growth factor type 2 receptor (HER2/neu) test : A laboratory test to measure how many HER2/neu genes there are and how much HER2/neu protein is made in a sample of tissue. If there are more HER2/neu genes or higher levels of HER2/neu protein than normal, the cancer is called HER2/neu positive. This type of breast cancer may grow more quickly and is more likely to spread to other parts of the body. The cancer may be treated with drugs that target the HER2/neu protein, such as trastuzumab and pertuzumab, after the baby is born. - Multigene tests: Tests in which samples of tissue are studied to look at the activity of many genes at the same time. These tests may help predict whether cancer will spread to other parts of the body or recur (come back). - Oncotype DX : This test helps predict whether stage I or stage II breast cancer that is estrogen receptor positive and node-negative will spread to other parts of the body. If the risk of the cancer spreading is high, chemotherapy may be given to lower the risk. - MammaPrint : This test helps predict whether stage I or stage II breast cancer that is node-negative will spread to other parts of the body. If the risk of the cancer spreading is high, chemotherapy may be given to lower the risk.",Breast Cancer,0000027_3,CancerGov,https://www.cancer.gov/types/breast/patient/pregnancy-breast-treatment-pdq,C0006142,T191,Disorders What is the outlook for Breast Cancer ?,0000027_3-5,outlook,Certain factors affect prognosis (chance of recovery) and treatment options.The prognosis (chance of recovery) and treatment options depend on the following: - The stage of the cancer (the size of the tumor and whether it is in the breast only or has spread to other parts of the body). - The type of breast cancer. - The age of the unborn baby. - Whether there are signs or symptoms. - The patients general health.,Breast Cancer,0000027_3,CancerGov,https://www.cancer.gov/types/breast/patient/pregnancy-breast-treatment-pdq,C0006142,T191,Disorders What are the stages of Breast Cancer ?,0000027_3-6,stages,"Key Points - After breast cancer has been diagnosed, tests are done to find out if cancer cells have spread within the breast or to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - The following stages are used for breast cancer: - Stage 0 (carcinoma in situ) - Stage I - Stage II - Stage IIIA - Stage IIIB - Stage IIIC - Stage IV After breast cancer has been diagnosed, tests are done to find out if cancer cells have spread within the breast or to other parts of the body. The process used to find out if the cancer has spread within the breast or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. Some procedures may expose the unborn baby to harmful radiation or dyes. These procedures are done only if absolutely necessary. Certain actions can be taken to expose the unborn baby to as little radiation as possible, such as the use of a lead-lined shield to cover the abdomen. The following tests and procedures may be used to stage breast cancer during pregnancy: - Sentinel lymph node biopsy : The removal of the sentinel lymph node during surgery. The sentinel lymph node is the first lymph node to receive lymphatic drainage from a tumor. It is the first lymph node the cancer is likely to spread to from the tumor. A radioactive substance and/or blue dye is injected near the tumor. The substance or dye flows through the lymph ducts to the lymph nodes. The first lymph node to receive the substance or dye is removed. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are not found, it may not be necessary to remove more lymph nodes. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - Bone scan : A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in bones with cancer and is detected by a scanner. - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body, such as the brain. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs, such as the liver, and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later. - Chest x-ray : An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if breast cancer spreads to the bone, the cancer cells in the bone are actually breast cancer cells. The disease is metastatic breast cancer, not bone cancer. The following stages are used for breast cancer: This section describes the stages of breast cancer. The breast cancer stage is based on the results of testing that is done on the tumor and lymph nodes removed during surgery and other tests. Stage 0 (carcinoma in situ) There are 3 types of breast carcinoma in situ: - Ductal carcinoma in situ (DCIS) is a noninvasive condition in which abnormal cells are found in the lining of a breast duct. The abnormal cells have not spread outside the duct to other tissues in the breast. In some cases, DCIS may become invasive cancer and spread to other tissues. At this time, there is no way to know which lesions could become invasive. - Lobular carcinoma in situ (LCIS) is a condition in which abnormal cells are found in the lobules of the breast. This condition seldom becomes invasive cancer. However, having LCIS in one breast increases the risk of developing breast cancer in either breast. - Paget disease of the nipple is a condition in which abnormal cells are found in the nipple only. Stage I In stage I, cancer has formed. Stage I is divided into stages IA and IB. - In stage IA, the tumor is 2 centimeters or smaller. Cancer has not spread outside the breast. - In stage IB, small clusters of breast cancer cells (larger than 0.2 millimeter but not larger than 2 millimeters) are found in the lymph nodes and either: - no tumor is found in the breast; or - the tumor is 2 centimeters or smaller. Stage II Stage II is divided into stages IIA and IIB. - In stage IIA: - no tumor is found in the breast or the tumor is 2 centimeters or smaller. Cancer (larger than 2 millimeters) is found in 1 to 3 axillary lymph nodes or in the lymph nodes near the breastbone (found during a sentinel lymph node biopsy); or - the tumor is larger than 2 centimeters but not larger than 5 centimeters. Cancer has not spread to the lymph nodes. - In stage IIB, the tumor is: - larger than 2 centimeters but not larger than 5 centimeters. Small clusters of breast cancer cells (larger than 0.2 millimeter but not larger than 2 millimeters) are found in the lymph nodes; or - larger than 2 centimeters but not larger than 5 centimeters. Cancer has spread to 1 to 3 axillary lymph nodes or to the lymph nodes near the breastbone (found during a sentinel lymph node biopsy); or - larger than 5 centimeters. Cancer has not spread to the lymph nodes. Stage IIIA In stage IIIA: - no tumor is found in the breast or the tumor may be any size. Cancer is found in 4 to 9 axillary lymph nodes or in the lymph nodes near the breastbone (found during imaging tests or a physical exam); or - the tumor is larger than 5 centimeters. Small clusters of breast cancer cells (larger than 0.2 millimeter but not larger than 2 millimeters) are found in the lymph nodes; or - the tumor is larger than 5 centimeters. Cancer has spread to 1 to 3 axillary lymph nodes or to the lymph nodes near the breastbone (found during a sentinel lymph node biopsy). Stage IIIB In stage IIIB, the tumor may be any size and cancer has spread to the chest wall and/or to the skin of the breast and caused swelling or an ulcer. Also, cancer may have spread to: - up to 9 axillary lymph nodes; or - the lymph nodes near the breastbone. Cancer that has spread to the skin of the breast may also be inflammatory breast cancer. See the section on Inflammatory Breast Cancer for more information. Stage IIIC In stage IIIC, no tumor is found in the breast or the tumor may be any size. Cancer may have spread to the skin of the breast and caused swelling or an ulcer and/or has spread to the chest wall. Also, cancer has spread to: - 10 or more axillary lymph nodes; or - lymph nodes above or below the collarbone; or - axillary lymph nodes and lymph nodes near the breastbone. Cancer that has spread to the skin of the breast may also be inflammatory breast cancer. See the section on Inflammatory Breast Cancer for more information. For treatment, stage IIIC breast cancer is divided into operable and inoperable stage IIIC. Stage IV In stage IV, cancer has spread to other organs of the body, most often the bones, lungs, liver, or brain.",Breast Cancer,0000027_3,CancerGov,https://www.cancer.gov/types/breast/patient/pregnancy-breast-treatment-pdq,C0006142,T191,Disorders What are the treatments for Breast Cancer ?,0000027_3-7,treatment,"Key Points - Treatment options for pregnant women depend on the stage of the disease and the age of the unborn baby. - Three types of standard treatment are used: - Surgery - Radiation therapy - Chemotherapy - Ending the pregnancy does not seem to improve the mothers chance of survival. - Treatment for breast cancer may cause side effects. Treatment options for pregnant women depend on the stage of the disease and the age of the unborn baby. Three types of standard treatment are used: Surgery Most pregnant women with breast cancer have surgery to remove the breast. Some of the lymph nodes under the arm may be removed and checked under a microscope for signs of cancer. Types of surgery to remove the cancer include: - Modified radical mastectomy: Surgery to remove the whole breast that has cancer, many of the lymph nodes under the arm, the lining over the chest muscles, and sometimes, part of the chest wall muscles. This type of surgery is most common in pregnant women. - Breast-conserving surgery: Surgery to remove the cancer and some normal tissue around it, but not the breast itself. Part of the chest wall lining may also be removed if the cancer is near it. This type of surgery may also be called lumpectomy, partial mastectomy, segmental mastectomy, quadrantectomy, or breast-sparing surgery. Even if the doctor removes all of the cancer that can be seen at the time of surgery, the patient may be given radiation therapy or chemotherapy after surgery to try to kill any cancer cells that may be left. For pregnant women with early-stage breast cancer, radiation therapy and hormone therapy are given after the baby is born. Treatment given after surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External radiation therapy is not given to pregnant women with early stage (stage I or II) breast cancer because it can harm the unborn baby. For women with late stage (stage III or IV) breast cancer, radiation therapy is not given during the first 3 months of pregnancy and is delayed until after the baby is born, if possible. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. Chemotherapy is usually not given during the first 3 months of pregnancy. Chemotherapy given after this time does not usually harm the unborn baby but may cause early labor and low birth weight. See Drugs Approved for Breast Cancer for more information. Ending the pregnancy does not seem to improve the mothers chance of survival. Because ending the pregnancy is not likely to improve the mothers chance of survival, it is not usually a treatment option. Treatment for breast cancer may cause side effects. For information about side effects caused by treatment for cancer, see our Side Effects page. Treatment Options by Stage Early Stage Breast Cancer (Stage I and Stage II) Treatment of early-stage breast cancer (stage I and stage II) may include the following: - Modified radical mastectomy. - Breast-conserving surgery followed by radiation therapy. In pregnant women, radiation therapy is delayed until after the baby is born. - Modified radical mastectomy or breast-conserving surgery during pregnancy followed by chemotherapy after the first 3 months of pregnancy. Late Stage Breast Cancer (Stage III and Stage IV) Treatment of late-stage breast cancer (stage III and stage IV) may include the following: - Radiation therapy. - Chemotherapy. Radiation therapy and chemotherapy should not be given during the first 3 months of pregnancy.",Breast Cancer,0000027_3,CancerGov,https://www.cancer.gov/types/breast/patient/pregnancy-breast-treatment-pdq,C0006142,T191,Disorders What is (are) Breast Cancer ?,0000027_4-1,information,"Key Points - Breast cancer is a disease in which malignant (cancer) cells form in the tissues of the breast. - Breast cancer is the second most common type of cancer in American women. Breast cancer is a disease in which malignant (cancer) cells form in the tissues of the breast. The breast is made up of lobes and ducts. Each breast has 15 to 20 sections called lobes, which have many smaller sections called lobules. Lobules end in dozens of tiny bulbs that can make milk. The lobes, lobules, and bulbs are linked by thin tubes called ducts. Each breast also has blood vessels and lymph vessels. The lymph vessels carry an almost colorless fluid called lymph. Lymph vessels lead to organs called lymph nodes. Lymph nodes are small bean-shaped structures that are found throughout the body. They filter lymph and store white blood cells that help fight infection and disease. Clusters of lymph nodes are found near the breast in the axilla (under the arm), above the collarbone, and in the chest. See the following PDQ summaries for more information about breast cancer: - Breast Cancer Screening - Breast Cancer Treatment - Breast Cancer Treatment and Pregnancy - Male Breast Cancer Treatment - Genetics of Breast and Ovarian Cancer Breast cancer is the second most common type of cancer in American women. Women in the United States get breast cancer more than any other type of cancer except skin cancer. Breast cancer is second to lung cancer as a cause of cancer death in American women. However, deaths from breast cancer have decreased a little bit every year between 2003 and 2012. Breast cancer also occurs in men, but the number of new cases is small.",Breast Cancer,0000027_4,CancerGov,https://www.cancer.gov/types/breast/patient/breast-prevention-pdq,C0006142,T191,Disorders How to prevent Breast Cancer ?,0000027_4-2,prevention,"Key Points - Avoiding risk factors and increasing protective factors may help prevent cancer. - The following are risk factors for breast cancer: - Older age - A personal history of breast cancer or benign (noncancer) breast disease - Inherited risk of breast cancer - Dense breasts - Exposure of breast tissue to estrogen made in the body - Taking hormone therapy for symptoms of menopause - Radiation therapy to the breast or chest - Obesity - Drinking alcohol - The following are protective factors for breast cancer: - Less exposure of breast tissue to estrogen made by the body - Taking estrogen-only hormone therapy after hysterectomy, selective estrogen receptor modulators, or aromatase inhibitors and inactivators - Estrogen-only hormone therapy after hysterectomy - Selective estrogen receptor modulators - Aromatase inhibitors and inactivators - Risk-reducing mastectomy - Ovarian ablation - Getting enough exercise - It is not clear whether the following affect the risk of breast cancer: - Oral contraceptives - Environment - Studies have shown that some factors do not affect the risk of breast cancer. - Cancer prevention clinical trials are used to study ways to prevent cancer. - New ways to prevent breast cancer are being studied in clinical trials. Avoiding risk factors and increasing protective factors may help prevent cancer. Avoiding cancer risk factors may help prevent certain cancers. Risk factors include smoking, being overweight, and not getting enough exercise. Increasing protective factors such as quitting smoking and exercising may also help prevent some cancers. Talk to your doctor or other health care professional about how you might lower your risk of cancer. NCI's Breast Cancer Risk Assessment Tool uses a woman's risk factors to estimate her risk for breast cancer during the next five years and up to age 90. This online tool is meant to be used by a health care provider. For more information on breast cancer risk, call 1-800-4-CANCER. The following are risk factors for breast cancer: Older age Older age is the main risk factor for most cancers. The chance of getting cancer increases as you get older. A personal history of breast cancer or benign (noncancer) breast disease Women with any of the following have an increased risk of breast cancer: - A personal history of invasive breast cancer, ductal carcinoma in situ (DCIS), or lobular carcinoma in situ (LCIS). - A personal history of benign (noncancer) breast disease. Inherited risk of breast cancer Women with a family history of breast cancer in a first-degree relative (mother, sister, or daughter) have an increased risk of breast cancer. Women who have inherited changes in the BRCA1 and BRCA2 genes or in certain other genes have a higher risk of breast cancer. The risk of breast cancer caused by inherited gene changes depends on the type of gene mutation, family history of cancer, and other factors. Dense breasts Having breast tissue that is dense on a mammogram is a factor in breast cancer risk. The level of risk depends on how dense the breast tissue is. Women with very dense breasts have a higher risk of breast cancer than women with low breast density. Increased breast density is often an inherited trait, but it may also occur in women who have not had children, have a first pregnancy late in life, take postmenopausal hormones, or drink alcohol. Exposure of breast tissue to estrogen made in the body Estrogen is a hormone made by the body. It helps the body develop and maintain female sex characteristics. Being exposed to estrogen over a long time may increase the risk of breast cancer. Estrogen levels are highest during the years a woman is menstruating. A woman's exposure to estrogen is increased in the following ways: - Early menstruation: Beginning to have menstrual periods at age 11 or younger increases the number of years the breast tissue is exposed to estrogen. - Starting menopause at a later age: The more years a woman menstruates, the longer her breast tissue is exposed to estrogen. - Older age at first birth or never having given birth: Because estrogen levels are lower during pregnancy, breast tissue is exposed to more estrogen in women who become pregnant for the first time after age 35 or who never become pregnant. Taking hormone therapy for symptoms of menopause Hormones, such as estrogen and progesterone, can be made into a pill form in a laboratory. Estrogen, progestin, or both may be given to replace the estrogen no longer made by the ovaries in postmenopausal women or women who have had their ovaries removed. This is called hormone replacement therapy (HRT) or hormone therapy (HT). Combination HRT/HT is estrogen combined with progestin. This type of HRT/HT increases the risk of breast cancer. Studies show that when women stop taking estrogen combined with progestin, the risk of breast cancer decreases. Radiation therapy to the breast or chest Radiation therapy to the chest for the treatment of cancer increases the risk of breast cancer, starting 10 years after treatment. The risk of breast cancer depends on the dose of radiation and the age at which it is given. The risk is highest if radiation treatment was used during puberty, when breasts are forming. Radiation therapy to treat cancer in one breast does not appear to increase the risk of cancer in the other breast. For women who have inherited changes in the BRCA1 and BRCA2 genes, exposure to radiation, such as that from chest x-rays, may further increase the risk of breast cancer, especially in women who were x-rayed before 20 years of age. Obesity Obesity increases the risk of breast cancer, especially in postmenopausal women who have not used hormone replacement therapy. Drinking alcohol Drinking alcohol increases the risk of breast cancer. The level of risk rises as the amount of alcohol consumed rises. The following are protective factors for breast cancer: Less exposure of breast tissue to estrogen made by the body Decreasing the length of time a woman's breast tissue is exposed to estrogen may help prevent breast cancer. Exposure to estrogen is reduced in the following ways: - Early pregnancy: Estrogen levels are lower during pregnancy. Women who have a full-term pregnancy before age 20 have a lower risk of breast cancer than women who have not had children or who give birth to their first child after age 35. - Breast-feeding: Estrogen levels may remain lower while a woman is breast-feeding. Women who breastfed have a lower risk of breast cancer than women who have had children but did not breastfeed. Taking estrogen-only hormone therapy after hysterectomy, selective estrogen receptor modulators, or aromatase inhibitors and inactivators Estrogen-only hormone therapy after hysterectomy Hormone therapy with estrogen only may be given to women who have had a hysterectomy. In these women, estrogen-only therapy after menopause may decrease the risk of breast cancer. There is an increased risk of stroke and heart and blood vessel disease in postmenopausal women who take estrogen after a hysterectomy. Selective estrogen receptor modulators Tamoxifen and raloxifene belong to the family of drugs called selective estrogen receptor modulators (SERMs). SERMs act like estrogen on some tissues in the body, but block the effect of estrogen on other tissues. Treatment with tamoxifen lowers the risk of estrogen receptor-positive (ER-positive) breast cancer and ductal carcinoma in situ in premenopausal and postmenopausal women at high risk. Treatment with raloxifene also lowers the risk of breast cancer in postmenopausal women. With either drug, the reduced risk lasts for several years or longer after treatment is stopped. Lower rates of broken bones have been noted in patients taking raloxifene. Taking tamoxifen increases the risk of hot flashes, endometrial cancer, stroke, cataracts, and blood clots (especially in the lungs and legs). The risk of having these problems increases markedly in women older than 50 years compared with younger women. Women younger than 50 years who have a high risk of breast cancer may benefit the most from taking tamoxifen. The risk of having these problems decreases after tamoxifen is stopped. Talk with your doctor about the risks and benefits of taking this drug. Taking raloxifene increases the risk of blood clots in the lungs and legs, but does not appear to increase the risk of endometrial cancer. In postmenopausal women with osteoporosis (decreased bone density), raloxifene lowers the risk of breast cancer for women who have a high or low risk of breast cancer. It is not known if raloxifene would have the same effect in women who do not have osteoporosis. Talk with your doctor about the risks and benefits of taking this drug. Other SERMs are being studied in clinical trials. Aromatase inhibitors and inactivators Aromatase inhibitors (anastrozole, letrozole) and inactivators (exemestane) lower the risk of recurrence and of new breast cancers in women who have a history of breast cancer. Aromatase inhibitors also decrease the risk of breast cancer in women with the following conditions: - Postmenopausal women with a personal history of breast cancer. - Women with no personal history of breast cancer who are 60 years and older, have a history of ductal carcinoma in situ with mastectomy, or have a high risk of breast cancer based on the Gail model tool (a tool used to estimate the risk of breast cancer). In women with an increased risk of breast cancer, taking aromatase inhibitors decreases the amount of estrogen made by the body. Before menopause, estrogen is made by the ovaries and other tissues in a woman's body, including the brain, fat tissue, and skin. After menopause, the ovaries stop making estrogen, but the other tissues do not. Aromatase inhibitors block the action of an enzyme called aromatase, which is used to make all of the body's estrogen. Aromatase inactivators stop the enzyme from working. Possible harms from taking aromatase inhibitors include muscle and joint pain, osteoporosis, hot flashes, and feeling very tired. Risk-reducing mastectomy Some women who have a high risk of breast cancer may choose to have a risk-reducing mastectomy (the removal of both breasts when there are no signs of cancer). The risk of breast cancer is much lower in these women and most feel less anxious about their risk of breast cancer. However, it is very important to have a cancer risk assessment and counseling about the different ways to prevent breast cancer before making this decision. Ovarian ablation The ovaries make most of the estrogen that is made by the body. Treatments that stop or lower the amount of estrogen made by the ovaries include surgery to remove the ovaries, radiation therapy, or taking certain drugs. This is called ovarian ablation. Premenopausal women who have a high risk of breast cancer due to certain changes in the BRCA1 and BRCA2 genes may choose to have a risk-reducing oophorectomy (the removal of both ovaries when there are no signs of cancer). This decreases the amount of estrogen made by the body and lowers the risk of breast cancer. Risk-reducing oophorectomy also lowers the risk of breast cancer in normal premenopausal women and in women with an increased risk of breast cancer due to radiation to the chest. However, it is very important to have a cancer risk assessment and counseling before making this decision. The sudden drop in estrogen levels may cause the symptoms of menopause to begin. These include hot flashes, trouble sleeping, anxiety, and depression. Long-term effects include decreased sex drive, vaginal dryness, and decreased bone density. Getting enough exercise Women who exercise four or more hours a week have a lower risk of breast cancer. The effect of exercise on breast cancer risk may be greatest in premenopausal women who have normal or low body weight. It is not clear whether the following affect the risk of breast cancer: Oral contraceptives Certain oral contraceptives contain estrogen. Some studies have shown that taking oral contraceptives (""the pill"") may slightly increase the risk of breast cancer in current users. This risk decreases over time. Other studies have not shown an increased risk of breast cancer in women who take oral contraceptives. Progestin -only contraceptives that are injected or implanted do not appear to increase the risk of breast cancer. More studies are needed to know whether progestin-only oral contraceptives increase the risk of breast cancer. Environment Studies have not proven that being exposed to certain substances in the environment, such as chemicals, increases the risk of breast cancer. Studies have shown that some factors do not affect the risk of breast cancer. The following do not affect the risk of breast cancer: - Having an abortion. - Making diet changes such as eating less fat or more fruits and vegetables. - Taking vitamins, including fenretinide (a type of vitamin A). - Cigarette smoking, both active and passive (inhaling secondhand smoke). - Using underarm deodorant or antiperspirant. - Taking statins (cholesterol -lowering drugs). - Taking bisphosphonates (drugs used to treat osteoporosis and hypercalcemia) by mouth or by intravenous infusion. Cancer prevention clinical trials are used to study ways to prevent cancer. Cancer prevention clinical trials are used to study ways to lower the risk of developing certain types of cancer. Some cancer prevention trials are conducted with healthy people who have not had cancer but who have an increased risk for cancer. Other prevention trials are conducted with people who have had cancer and are trying to prevent another cancer of the same type or to lower their chance of developing a new type of cancer. Other trials are done with healthy volunteers who are not known to have any risk factors for cancer. The purpose of some cancer prevention clinical trials is to find out whether actions people take can prevent cancer. These may include exercising more or quitting smoking or taking certain medicines, vitamins, minerals, or food supplements. New ways to prevent breast cancer are being studied in clinical trials.",Breast Cancer,0000027_4,CancerGov,https://www.cancer.gov/types/breast/patient/breast-prevention-pdq,C0006142,T191,Disorders Who is at risk for Breast Cancer? ?,0000027_4-3,susceptibility,"Key Points - Avoiding risk factors and increasing protective factors may help prevent cancer. - The following are risk factors for breast cancer: - Older age - A personal history of breast cancer or benign (noncancer) breast disease - Inherited risk of breast cancer - Dense breasts - Exposure of breast tissue to estrogen made in the body - Taking hormone therapy for symptoms of menopause - Radiation therapy to the breast or chest - Obesity - Drinking alcohol - The following are protective factors for breast cancer: - Less exposure of breast tissue to estrogen made by the body - Taking estrogen-only hormone therapy after hysterectomy, selective estrogen receptor modulators, or aromatase inhibitors and inactivators - Estrogen-only hormone therapy after hysterectomy - Selective estrogen receptor modulators - Aromatase inhibitors and inactivators - Risk-reducing mastectomy - Ovarian ablation - Getting enough exercise - It is not clear whether the following affect the risk of breast cancer: - Oral contraceptives - Environment - Studies have shown that some factors do not affect the risk of breast cancer. - Cancer prevention clinical trials are used to study ways to prevent cancer. - New ways to prevent breast cancer are being studied in clinical trials. Avoiding risk factors and increasing protective factors may help prevent cancer. Avoiding cancer risk factors may help prevent certain cancers. Risk factors include smoking, being overweight, and not getting enough exercise. Increasing protective factors such as quitting smoking and exercising may also help prevent some cancers. Talk to your doctor or other health care professional about how you might lower your risk of cancer. NCI's Breast Cancer Risk Assessment Tool uses a woman's risk factors to estimate her risk for breast cancer during the next five years and up to age 90. This online tool is meant to be used by a health care provider. For more information on breast cancer risk, call 1-800-4-CANCER. The following are risk factors for breast cancer: Older age Older age is the main risk factor for most cancers. The chance of getting cancer increases as you get older. A personal history of breast cancer or benign (noncancer) breast disease Women with any of the following have an increased risk of breast cancer: - A personal history of invasive breast cancer, ductal carcinoma in situ (DCIS), or lobular carcinoma in situ (LCIS). - A personal history of benign (noncancer) breast disease. Inherited risk of breast cancer Women with a family history of breast cancer in a first-degree relative (mother, sister, or daughter) have an increased risk of breast cancer. Women who have inherited changes in the BRCA1 and BRCA2 genes or in certain other genes have a higher risk of breast cancer. The risk of breast cancer caused by inherited gene changes depends on the type of gene mutation, family history of cancer, and other factors. Dense breasts Having breast tissue that is dense on a mammogram is a factor in breast cancer risk. The level of risk depends on how dense the breast tissue is. Women with very dense breasts have a higher risk of breast cancer than women with low breast density. Increased breast density is often an inherited trait, but it may also occur in women who have not had children, have a first pregnancy late in life, take postmenopausal hormones, or drink alcohol. Exposure of breast tissue to estrogen made in the body Estrogen is a hormone made by the body. It helps the body develop and maintain female sex characteristics. Being exposed to estrogen over a long time may increase the risk of breast cancer. Estrogen levels are highest during the years a woman is menstruating. A woman's exposure to estrogen is increased in the following ways: - Early menstruation: Beginning to have menstrual periods at age 11 or younger increases the number of years the breast tissue is exposed to estrogen. - Starting menopause at a later age: The more years a woman menstruates, the longer her breast tissue is exposed to estrogen. - Older age at first birth or never having given birth: Because estrogen levels are lower during pregnancy, breast tissue is exposed to more estrogen in women who become pregnant for the first time after age 35 or who never become pregnant. Taking hormone therapy for symptoms of menopause Hormones, such as estrogen and progesterone, can be made into a pill form in a laboratory. Estrogen, progestin, or both may be given to replace the estrogen no longer made by the ovaries in postmenopausal women or women who have had their ovaries removed. This is called hormone replacement therapy (HRT) or hormone therapy (HT). Combination HRT/HT is estrogen combined with progestin. This type of HRT/HT increases the risk of breast cancer. Studies show that when women stop taking estrogen combined with progestin, the risk of breast cancer decreases. Radiation therapy to the breast or chest Radiation therapy to the chest for the treatment of cancer increases the risk of breast cancer, starting 10 years after treatment. The risk of breast cancer depends on the dose of radiation and the age at which it is given. The risk is highest if radiation treatment was used during puberty, when breasts are forming. Radiation therapy to treat cancer in one breast does not appear to increase the risk of cancer in the other breast. For women who have inherited changes in the BRCA1 and BRCA2 genes, exposure to radiation, such as that from chest x-rays, may further increase the risk of breast cancer, especially in women who were x-rayed before 20 years of age. Obesity Obesity increases the risk of breast cancer, especially in postmenopausal women who have not used hormone replacement therapy. Drinking alcohol Drinking alcohol increases the risk of breast cancer. The level of risk rises as the amount of alcohol consumed rises. The following are protective factors for breast cancer: Less exposure of breast tissue to estrogen made by the body Decreasing the length of time a woman's breast tissue is exposed to estrogen may help prevent breast cancer. Exposure to estrogen is reduced in the following ways: - Early pregnancy: Estrogen levels are lower during pregnancy. Women who have a full-term pregnancy before age 20 have a lower risk of breast cancer than women who have not had children or who give birth to their first child after age 35. - Breast-feeding: Estrogen levels may remain lower while a woman is breast-feeding. Women who breastfed have a lower risk of breast cancer than women who have had children but did not breastfeed. Taking estrogen-only hormone therapy after hysterectomy, selective estrogen receptor modulators, or aromatase inhibitors and inactivators Estrogen-only hormone therapy after hysterectomy Hormone therapy with estrogen only may be given to women who have had a hysterectomy. In these women, estrogen-only therapy after menopause may decrease the risk of breast cancer. There is an increased risk of stroke and heart and blood vessel disease in postmenopausal women who take estrogen after a hysterectomy. Selective estrogen receptor modulators Tamoxifen and raloxifene belong to the family of drugs called selective estrogen receptor modulators (SERMs). SERMs act like estrogen on some tissues in the body, but block the effect of estrogen on other tissues. Treatment with tamoxifen lowers the risk of estrogen receptor-positive (ER-positive) breast cancer and ductal carcinoma in situ in premenopausal and postmenopausal women at high risk. Treatment with raloxifene also lowers the risk of breast cancer in postmenopausal women. With either drug, the reduced risk lasts for several years or longer after treatment is stopped. Lower rates of broken bones have been noted in patients taking raloxifene. Taking tamoxifen increases the risk of hot flashes, endometrial cancer, stroke, cataracts, and blood clots (especially in the lungs and legs). The risk of having these problems increases markedly in women older than 50 years compared with younger women. Women younger than 50 years who have a high risk of breast cancer may benefit the most from taking tamoxifen. The risk of having these problems decreases after tamoxifen is stopped. Talk with your doctor about the risks and benefits of taking this drug. Taking raloxifene increases the risk of blood clots in the lungs and legs, but does not appear to increase the risk of endometrial cancer. In postmenopausal women with osteoporosis (decreased bone density), raloxifene lowers the risk of breast cancer for women who have a high or low risk of breast cancer. It is not known if raloxifene would have the same effect in women who do not have osteoporosis. Talk with your doctor about the risks and benefits of taking this drug. Other SERMs are being studied in clinical trials. Aromatase inhibitors and inactivators Aromatase inhibitors (anastrozole, letrozole) and inactivators (exemestane) lower the risk of recurrence and of new breast cancers in women who have a history of breast cancer. Aromatase inhibitors also decrease the risk of breast cancer in women with the following conditions: - Postmenopausal women with a personal history of breast cancer. - Women with no personal history of breast cancer who are 60 years and older, have a history of ductal carcinoma in situ with mastectomy, or have a high risk of breast cancer based on the Gail model tool (a tool used to estimate the risk of breast cancer). In women with an increased risk of breast cancer, taking aromatase inhibitors decreases the amount of estrogen made by the body. Before menopause, estrogen is made by the ovaries and other tissues in a woman's body, including the brain, fat tissue, and skin. After menopause, the ovaries stop making estrogen, but the other tissues do not. Aromatase inhibitors block the action of an enzyme called aromatase, which is used to make all of the body's estrogen. Aromatase inactivators stop the enzyme from working. Possible harms from taking aromatase inhibitors include muscle and joint pain, osteoporosis, hot flashes, and feeling very tired. Risk-reducing mastectomy Some women who have a high risk of breast cancer may choose to have a risk-reducing mastectomy (the removal of both breasts when there are no signs of cancer). The risk of breast cancer is much lower in these women and most feel less anxious about their risk of breast cancer. However, it is very important to have a cancer risk assessment and counseling about the different ways to prevent breast cancer before making this decision. Ovarian ablation The ovaries make most of the estrogen that is made by the body. Treatments that stop or lower the amount of estrogen made by the ovaries include surgery to remove the ovaries, radiation therapy, or taking certain drugs. This is called ovarian ablation. Premenopausal women who have a high risk of breast cancer due to certain changes in the BRCA1 and BRCA2 genes may choose to have a risk-reducing oophorectomy (the removal of both ovaries when there are no signs of cancer). This decreases the amount of estrogen made by the body and lowers the risk of breast cancer. Risk-reducing oophorectomy also lowers the risk of breast cancer in normal premenopausal women and in women with an increased risk of breast cancer due to radiation to the chest. However, it is very important to have a cancer risk assessment and counseling before making this decision. The sudden drop in estrogen levels may cause the symptoms of menopause to begin. These include hot flashes, trouble sleeping, anxiety, and depression. Long-term effects include decreased sex drive, vaginal dryness, and decreased bone density. Getting enough exercise Women who exercise four or more hours a week have a lower risk of breast cancer. The effect of exercise on breast cancer risk may be greatest in premenopausal women who have normal or low body weight. It is not clear whether the following affect the risk of breast cancer: Oral contraceptives Certain oral contraceptives contain estrogen. Some studies have shown that taking oral contraceptives (""the pill"") may slightly increase the risk of breast cancer in current users. This risk decreases over time. Other studies have not shown an increased risk of breast cancer in women who take oral contraceptives. Progestin -only contraceptives that are injected or implanted do not appear to increase the risk of breast cancer. More studies are needed to know whether progestin-only oral contraceptives increase the risk of breast cancer. Environment Studies have not proven that being exposed to certain substances in the environment, such as chemicals, increases the risk of breast cancer. Studies have shown that some factors do not affect the risk of breast cancer. The following do not affect the risk of breast cancer: - Having an abortion. - Making diet changes such as eating less fat or more fruits and vegetables. - Taking vitamins, including fenretinide (a type of vitamin A). - Cigarette smoking, both active and passive (inhaling secondhand smoke). - Using underarm deodorant or antiperspirant. - Taking statins (cholesterol -lowering drugs). - Taking bisphosphonates (drugs used to treat osteoporosis and hypercalcemia) by mouth or by intravenous infusion.",Breast Cancer,0000027_4,CancerGov,https://www.cancer.gov/types/breast/patient/breast-prevention-pdq,C0006142,T191,Disorders what research (or clinical trials) is being done for Breast Cancer ?,0000027_4-4,research,"Cancer prevention clinical trials are used to study ways to prevent cancer. Cancer prevention clinical trials are used to study ways to lower the risk of developing certain types of cancer. Some cancer prevention trials are conducted with healthy people who have not had cancer but who have an increased risk for cancer. Other prevention trials are conducted with people who have had cancer and are trying to prevent another cancer of the same type or to lower their chance of developing a new type of cancer. Other trials are done with healthy volunteers who are not known to have any risk factors for cancer. The purpose of some cancer prevention clinical trials is to find out whether actions people take can prevent cancer. These may include exercising more or quitting smoking or taking certain medicines, vitamins, minerals, or food supplements. New ways to prevent breast cancer are being studied in clinical trials.",Breast Cancer,0000027_4,CancerGov,https://www.cancer.gov/types/breast/patient/breast-prevention-pdq,C0006142,T191,Disorders What is (are) Breast Cancer ?,0000027_5-1,information,"Key Points - Breast cancer is a disease in which malignant (cancer) cells form in the tissues of the breast. - Breast cancer is the second leading cause of death from cancer in American women. - Different factors increase or decrease the risk of breast cancer. Breast cancer is a disease in which malignant (cancer) cells form in the tissues of the breast. The breast is made up of lobes and ducts. Each breast has 15 to 20 sections called lobes, which have many smaller sections called lobules. Lobules end in dozens of tiny bulbs that can produce milk. The lobes, lobules, and bulbs are linked by thin tubes called ducts. Each breast also contains blood vessels and lymph vessels. The lymph vessels carry an almost colorless fluid called lymph. Lymph vessels lead to organs called lymph nodes. Lymph nodes are small bean-shaped structures that are found throughout the body. They filter substances in lymph and help fight infection and disease. Clusters of lymph nodes are found near the breast in the axilla (under the arm), above the collarbone, and in the chest. See the following PDQ summaries for more information about breast cancer: - Breast Cancer Prevention - Breast Cancer Treatment - Genetics of Breast and Gynecologic Cancers Breast cancer is the second leading cause of death from cancer in American women. Women in the United States get breast cancer more than any other type of cancer except for skin cancer. Breast cancer is second only to lung cancer as a cause of cancer death in women. Breast cancer occurs more often in white women than in black women. However, black women are more likely than white women to die from the disease. Breast cancer occurs in men also, but the number of cases is small.",Breast Cancer,0000027_5,CancerGov,https://www.cancer.gov/types/breast/patient/breast-screening-pdq,C0006142,T191,Disorders Who is at risk for Breast Cancer? ?,0000027_5-2,susceptibility,"Different factors increase or decrease the risk of breast cancer. Anything that increases your chance of getting a disease is called a risk factor. Anything that decreases your chance of getting a disease is called a protective factor. For information about risk factors and protective factors for breast cancer, see the PDQ summary on Breast Cancer Prevention.",Breast Cancer,0000027_5,CancerGov,https://www.cancer.gov/types/breast/patient/breast-screening-pdq,C0006142,T191,Disorders what research (or clinical trials) is being done for Breast Cancer ?,0000027_5-4,research,Other screening tests are being studied in clinical trials. Thermography Thermography is a procedure in which a special camera that senses heat is used to record the temperature of the skin that covers the breasts. A computer makes a map of the breast showing the changes in temperature. Tumors can cause temperature changes that may show up on the thermogram. There have been no clinical trials of thermography to find out how well it detects breast cancer or if having the procedure decreases the risk of dying from breast cancer. Tissue sampling Breast tissue sampling is taking cells from breast tissue to check under a microscope. Abnormal cells in breast fluid have been linked to an increased risk of breast cancer in some studies. Scientists are studying whether breast tissue sampling can be used to find breast cancer at an early stage or predict the risk of developing breast cancer. Three ways of taking tissue samples are being studied: - Fine-needle aspiration: A thin needle is inserted into the breast tissue around the areola (darkened area around the nipple) to take out a sample of cells and fluid. - Nipple aspiration: The use of gentle suction to collect fluid through the nipple. This is done with a device similar to the breast pumps used by women who are breast-feeding. - Ductal lavage: A hair-size catheter (tube) is inserted into the nipple and a small amount of salt water is released into the duct. The water picks up breast cells and is removed. Screening clinical trials are taking place in many parts of the country. Information about ongoing clinical trials is available from the NCI website.,Breast Cancer,0000027_5,CancerGov,https://www.cancer.gov/types/breast/patient/breast-screening-pdq,C0006142,T191,Disorders What is (are) Skin Cancer ?,0000028_1-1,information,"Key Points - Skin cancer is a disease in which malignant (cancer) cells form in the tissues of the skin. - There are different types of cancer that start in the skin. - Skin color and being exposed to sunlight can increase the risk of nonmelanoma skin cancer and actinic keratosis. - Nonmelanoma skin cancer and actinic keratosis often appear as a change in the skin. - Tests or procedures that examine the skin are used to detect (find) and diagnose nonmelanoma skin cancer and actinic keratosis. - Certain factors affect prognosis (chance of recovery) and treatment options. Skin cancer is a disease in which malignant (cancer) cells form in the tissues of the skin. The skin is the bodys largest organ. It protects against heat, sunlight, injury, and infection. Skin also helps control body temperature and stores water, fat, and vitamin D. The skin has several layers, but the two main layers are the epidermis (upper or outer layer) and the dermis (lower or inner layer). Skin cancer begins in the epidermis, which is made up of three kinds of cells: - Squamous cells: Thin, flat cells that form the top layer of the epidermis. - Basal cells: Round cells under the squamous cells. - Melanocytes: Cells that make melanin and are found in the lower part of the epidermis. Melanin is the pigment that gives skin its natural color. When skin is exposed to the sun, melanocytes make more pigment and cause the skin to darken. Skin cancer can occur anywhere on the body, but it is most common in skin that is often exposed to sunlight, such as the face, neck, hands, and arms. There are different types of cancer that start in the skin. The most common types are basal cell carcinoma and squamous cell carcinoma, which are nonmelanoma skin cancers. Nonmelanoma skin cancers rarely spread to other parts of the body. Melanoma is a much rarer type of skin cancer. It is more likely to invade nearby tissues and spread to other parts of the body. Actinic keratosis is a skin condition that sometimes becomes squamous cell carcinoma. This summary is about nonmelanoma skin cancer and actinic keratosis. See the following PDQ summaries for information on melanoma and other kinds of cancer that affect the skin: - Melanoma Treatment - Mycosis Fungoides and the Szary Syndrome Treatment - Kaposi Sarcoma Treatment - Merkel Cell Carcinoma Treatment - Unusual Cancers of Childhood Treatment - Genetics of Skin Cancer Nonmelanoma skin cancer and actinic keratosis often appear as a change in the skin. Not all changes in the skin are a sign of nonmelanoma skin cancer or actinic keratosis. Check with your doctor if you notice any changes in your skin. Signs of nonmelanoma skin cancer include the following: - A sore that does not heal. - Areas of the skin that are: - Raised, smooth, shiny, and look pearly. - Firm and look like a scar, and may be white, yellow, or waxy. - Raised, and red or reddish-brown. - Scaly, bleeding or crusty.",Skin Cancer,0000028_1,CancerGov,https://www.cancer.gov/types/skin/patient/skin-treatment-pdq,C0007114,T191,Disorders Who is at risk for Skin Cancer? ?,0000028_1-2,susceptibility,"Skin color and being exposed to sunlight can increase the risk of nonmelanoma skin cancer and actinic keratosis. Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesnt mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for basal cell carcinoma and squamous cell carcinoma include the following: - Being exposed to natural sunlight or artificial sunlight (such as from tanning beds) over long periods of time. - Having a fair complexion, which includes the following: - Fair skin that freckles and burns easily, does not tan, or tans poorly. - Blue or green or other light-colored eyes. - Red or blond hair. - Having actinic keratosis. - Past treatment with radiation. - Having a weakened immune system. - Having certain changes in the genes that are linked to skin cancer. - Being exposed to arsenic.",Skin Cancer,0000028_1,CancerGov,https://www.cancer.gov/types/skin/patient/skin-treatment-pdq,C0007114,T191,Disorders How to diagnose Skin Cancer ?,0000028_1-3,exams and tests,"Tests or procedures that examine the skin are used to detect (find) and diagnose nonmelanoma skin cancer and actinic keratosis. The following procedures may be used: - Skin exam: A doctor or nurse checks the skin for bumps or spots that look abnormal in color, size, shape, or texture. - Skin biopsy : All or part of the abnormal-looking growth is cut from the skin and viewed under a microscope by a pathologist to check for signs of cancer. There are four main types of skin biopsies: - Shave biopsy : A sterile razor blade is used to shave-off the abnormal-looking growth. - Punch biopsy : A special instrument called a punch or a trephine is used to remove a circle of tissue from the abnormal-looking growth. - Incisional biopsy : A scalpel is used to remove part of a growth. - Excisional biopsy : A scalpel is used to remove the entire growth.",Skin Cancer,0000028_1,CancerGov,https://www.cancer.gov/types/skin/patient/skin-treatment-pdq,C0007114,T191,Disorders What is the outlook for Skin Cancer ?,0000028_1-4,outlook,Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) depends mostly on the stage of the cancer and the type of treatment used to remove the cancer. Treatment options depend on the following: - The stage of the cancer (whether it has spread deeper into the skin or to other places in the body). - The type of cancer. - The size of the tumor and what part of the body it affects. - The patients general health.,Skin Cancer,0000028_1,CancerGov,https://www.cancer.gov/types/skin/patient/skin-treatment-pdq,C0007114,T191,Disorders What are the stages of Skin Cancer ?,0000028_1-5,stages,"Key Points - After nonmelanoma skin cancer has been diagnosed, tests are done to find out if cancer cells have spread within the skin or to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - Staging of nonmelanoma skin cancer depends on whether the tumor has certain ""high-risk"" features and if the tumor is on the eyelid. - The following stages are used for nonmelanoma skin cancer that is not on the eyelid: - Stage 0 (Carcinoma in Situ) - Stage I Stage I nonmelanoma skin cancer. The tumor is no more than 2 centimeters. - Stage II Stage II nonmelanoma skin cancer. The tumor is more than 2 centimeters wide. - Stage III - Stage IV - The following stages are used for nonmelanoma skin cancer on the eyelid: - Stage 0 (Carcinoma in Situ) - Stage I - Stage II - Stage III - Stage IV - Treatment is based on the type of nonmelanoma skin cancer or other skin condition diagnosed: - Basal cell carcinoma - Squamous cell carcinoma - Actinic keratosis After nonmelanoma skin cancer has been diagnosed, tests are done to find out if cancer cells have spread within the skin or to other parts of the body. The process used to find out if cancer has spread within the skin or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following tests and procedures may be used in the staging process: - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Lymph node biopsy : For squamous cell carcinoma, the lymph nodes may be removed and checked to see if cancer has spread to them. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if skin cancer spreads to the lung, the cancer cells in the lung are actually skin cancer cells. The disease is metastatic skin cancer, not lung cancer. Staging of nonmelanoma skin cancer depends on whether the tumor has certain ""high-risk"" features and if the tumor is on the eyelid. Staging for nonmelanoma skin cancer that is on the eyelid is different from staging for nonmelanoma skin cancer that affects other parts of the body. The following are high-risk features for nonmelanoma skin cancer that is not on the eyelid: - The tumor is thicker than 2 millimeters. - The tumor is described as Clark level IV (has spread into the lower layer of the dermis) or Clark level V (has spread into the layer of fat below the skin). - The tumor has grown and spread along nerve pathways. - The tumor began on an ear or on a lip that has hair on it. - The tumor has cells that look very different from normal cells under a microscope. The following stages are used for nonmelanoma skin cancer that is not on the eyelid: Stage 0 (Carcinoma in Situ) In stage 0, abnormal cells are found in the squamous cell or basal cell layer of the epidermis (topmost layer of the skin). These abnormal cells may become cancer and spread into nearby normal tissue. Stage 0 is also called carcinoma in situ. Stage I In stage I, cancer has formed. The tumor is not larger than 2 centimeters at its widest point and may have one high-risk feature. Stage II In stage II, the tumor is either: - larger than 2 centimeters at its widest point; or - any size and has two or more high-risk features. Stage III In stage III: - The tumor has spread to the jaw, eye socket, or side of the skull. Cancer may have spread to one lymph node on the same side of the body as the tumor. The lymph node is not larger than 3 centimeters. or - Cancer has spread to one lymph node on the same side of the body as the tumor. The lymph node is not larger than 3 centimeters and one of the following is true: - the tumor is not larger than 2 centimeters at its widest point and may have one high-risk feature; or - the tumor is larger than 2 centimeters at its widest point; or - the tumor is any size and has two or more high-risk features. Stage IV In stage IV, one of the following is true: - The tumor is any size and may have spread to the jaw, eye socket, or side of the skull. Cancer has spread to one lymph node on the same side of the body as the tumor and the affected node is larger than 3 centimeters but not larger than 6 centimeters, or cancer has spread to more than one lymph node on one or both sides of the body and the affected nodes are not larger than 6 centimeters; or - The tumor is any size and may have spread to the jaw, eye socket, skull, spine, or ribs. Cancer has spread to one lymph node that is larger than 6 centimeters; or - The tumor is any size and has spread to the base of the skull, spine, or ribs. Cancer may have spread to the lymph nodes; or - Cancer has spread to other parts of the body, such as the lung. The following stages are used for nonmelanoma skin cancer on the eyelid: Stage 0 (Carcinoma in Situ) In stage 0, abnormal cells are found in the epidermis (topmost layer of the skin). These abnormal cells may become cancer and spread into nearby normal tissue. Stage 0 is also called carcinoma in situ. Stage I Stage I is divided into stages IA, IB, and IC. - Stage IA: The tumor is 5 millimeters or smaller and has not spread to the connective tissue of the eyelid or to the edge of the eyelid where the lashes are. - Stage IB: The tumor is larger than 5 millimeters but not larger than 10 millimeters or has spread to the connective tissue of the eyelid, or to the edge of the eyelid where the lashes are. - Stage IC: The tumor is larger than 10 millimeters but not larger than 20 millimeters or has spread through the full thickness of the eyelid. Stage II In stage II, one of the following is true: - The tumor is larger than 20 millimeters. - The tumor has spread to nearby parts of the eye or eye socket. - The tumor has spread to spaces around the nerves in the eyelid. Stage III Stage III is divided into stages IIIA, IIIB, and IIIC. - Stage IIIA: To remove all of the tumor, the whole eye and part of the optic nerve must be removed. The bone, muscles, fat, and connective tissue around the eye may also be removed. - Stage IIIB: The tumor may be anywhere in or near the eye and has spread to nearby lymph nodes. - Stage IIIC: The tumor has spread to structures around the eye or in the face, or to the brain, and cannot be removed in surgery. Stage IV The tumor has spread to distant parts of the body. Treatment is based on the type of nonmelanoma skin cancer or other skin condition diagnosed: Basal cell carcinoma Basal cell carcinoma is the most common type of skin cancer. It usually occurs on areas of the skin that have been in the sun, most often the nose. Often this cancer appears as a raised bump that looks smooth and pearly. Another type looks like a scar and is flat and firm and may be white, yellow, or waxy. Basal cell carcinoma may spread to tissues around the cancer, but it usually does not spread to other parts of the body. Squamous cell carcinoma Squamous cell carcinoma occurs on areas of the skin that have been in the sun, such as the ears, lower lip, and the back of the hands. Squamous cell carcinoma may also appear on areas of the skin that have been burned or exposed to chemicals or radiation. Often this cancer appears as a firm red bump. The tumor may feel scaly, bleed, or form a crust. Squamous cell tumors may spread to nearby lymph nodes. Squamous cell carcinoma that has not spread can usually be cured. Actinic keratosis Actinic keratosis is a skin condition that is not cancer, but sometimes changes into squamous cell carcinoma. It usually occurs in areas that have been exposed to the sun, such as the face, the back of the hands, and the lower lip. It looks like rough, red, pink, or brown scaly patches on the skin that may be flat or raised, or the lower lip cracks and peels and is not helped by lip balm or petroleum jelly.",Skin Cancer,0000028_1,CancerGov,https://www.cancer.gov/types/skin/patient/skin-treatment-pdq,C0007114,T191,Disorders What are the treatments for Skin Cancer ?,0000028_1-6,treatment,"Key Points - There are different types of treatment for patients with nonmelanoma skin cancer and actinic keratosis. - Six types of standard treatment are used: - Surgery - Radiation therapy - Chemotherapy - Photodynamic therapy - Biologic therapy - Targeted therapy - New types of treatment are being tested in clinical trials. - Treatment for skin cancer may cause side effects. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with nonmelanoma skin cancer and actinic keratosis. Different types of treatment are available for patients with nonmelanoma skin cancer and actinic keratosis. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Six types of standard treatment are used: Surgery One or more of the following surgical procedures may be used to treat nonmelanoma skin cancer or actinic keratosis: - Mohs micrographic surgery: The tumor is cut from the skin in thin layers. During surgery, the edges of the tumor and each layer of tumor removed are viewed through a microscope to check for cancer cells. Layers continue to be removed until no more cancer cells are seen. This type of surgery removes as little normal tissue as possible and is often used to remove skin cancer on the face. - Simple excision: The tumor is cut from the skin along with some of the normal skin around it. - Shave excision: The abnormal area is shaved off the surface of the skin with a small blade. - Electrodesiccation and curettage: The tumor is cut from the skin with a curette (a sharp, spoon-shaped tool). A needle-shaped electrode is then used to treat the area with an electric current that stops the bleeding and destroys cancer cells that remain around the edge of the wound. The process may be repeated one to three times during the surgery to remove all of the cancer. - Cryosurgery: A treatment that uses an instrument to freeze and destroy abnormal tissue, such as carcinoma in situ. This type of treatment is also called cryotherapy. - Laser surgery: A surgical procedure that uses a laser beam (a narrow beam of intense light) as a knife to make bloodless cuts in tissue or to remove a surface lesion such as a tumor. - Dermabrasion: Removal of the top layer of skin using a rotating wheel or small particles to rub away skin cells. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type of cancer being treated. External radiation therapy is used to treat skin cancer. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Chemotherapy for nonmelanoma skin cancer and actinic keratosis is usually topical (applied to the skin in a cream or lotion). The way the chemotherapy is given depends on the condition being treated. Retinoids (drugs related to vitamin A) are sometimes used to treat squamous cell carcinoma of the skin. See Drugs Approved for Basal Cell Carcinoma for more information. Photodynamic therapy Photodynamic therapy (PDT) is a cancer treatment that uses a drug and a certain type of laser light to kill cancer cells. A drug that is not active until it is exposed to light is injected into a vein. The drug collects more in cancer cells than in normal cells. For skin cancer, laser light is shined onto the skin and the drug becomes active and kills the cancer cells. Photodynamic therapy causes little damage to healthy tissue. Biologic therapy Biologic therapy is a treatment that uses the patients immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the bodys natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy. Interferon and imiquimod are biologic agents used to treat skin cancer. Interferon (by injection) may be used to treat squamous cell carcinoma of the skin. Topical imiquimod therapy (a cream applied to the skin) may be used to treat some small basal cell carcinomas. See Drugs Approved for Basal Cell Carcinoma for more information. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to attack cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. Targeted therapy with a signal transduction inhibitor is used to treat basal cell carcinoma. Signal transduction inhibitors block signals that are passed from one molecule to another inside a cell. Blocking these signals may kill cancer cells. Vismodegib and sonidegib are signal transduction inhibitors used to treat basal cell carcinoma. See Drugs Approved for Basal Cell Carcinoma for more information. New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Treatment for skin cancer may cause side effects. For information about side effects caused by treatment for cancer, see our Side Effects page. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Basal cell carcinoma and squamous cell carcinoma are likely to recur (come back), usually within 5 years, or new tumors may form. Talk to your doctor about how often you should have your skin checked for signs of cancer. Treatment Options for Nonmelanoma Skin Cancer Basal Cell Carcinoma Treatment of basal cell carcinoma may include the following: - Simple excision. - Mohs micrographic surgery. - Radiation therapy. - Electrodesiccation and curettage. - Cryosurgery. - Photodynamic therapy. - Topical chemotherapy. - Topical biologic therapy with imiquimod. - Laser surgery. Treatment of recurrent basal cell carcinoma is usually Mohs micrographic surgery. Treatment of basal cell carcinoma that is metastatic or cannot be treated with local therapy may include the following: - Targeted therapy with a signal transduction inhibitor. - Chemotherapy. - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with basal cell carcinoma of the skin. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Squamous Cell Carcinoma Treatment of squamous cell carcinoma may include the following: - Simple excision. - Mohs micrographic surgery. - Radiation therapy. - Electrodesiccation and curettage. - Cryosurgery. Treatment of recurrent squamous cell carcinoma may include the following: - Simple excision. - Mohs micrographic surgery. - Radiation therapy. Treatment of squamous cell carcinoma that is metastatic or cannot be treated with local therapy may include the following: - Chemotherapy. - Retinoid therapy and biologic therapy with interferon. - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with squamous cell carcinoma of the skin. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Skin Cancer,0000028_1,CancerGov,https://www.cancer.gov/types/skin/patient/skin-treatment-pdq,C0007114,T191,Disorders what research (or clinical trials) is being done for Skin Cancer ?,0000028_1-7,research,"New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Treatment for skin cancer may cause side effects. For information about side effects caused by treatment for cancer, see our Side Effects page. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Skin Cancer,0000028_1,CancerGov,https://www.cancer.gov/types/skin/patient/skin-treatment-pdq,C0007114,T191,Disorders What is (are) Melanoma ?,0000028_2-1,information,"Key Points - Melanoma is a disease in which malignant (cancer) cells form in melanocytes (cells that color the skin). - There are different types of cancer that start in the skin. - Melanoma can occur anywhere on the skin. - Unusual moles, exposure to sunlight, and health history can affect the risk of melanoma. - Signs of melanoma include a change in the way a mole or pigmented area looks. - Tests that examine the skin are used to detect (find) and diagnose melanoma. - Certain factors affect prognosis (chance of recovery) and treatment options. Melanoma is a disease in which malignant (cancer) cells form in melanocytes (cells that color the skin). The skin is the bodys largest organ. It protects against heat, sunlight, injury, and infection. Skin also helps control body temperature and stores water, fat, and vitamin D. The skin has several layers, but the two main layers are the epidermis (upper or outer layer) and the dermis (lower or inner layer). Skin cancer begins in the epidermis, which is made up of three kinds of cells: - Squamous cells: Thin, flat cells that form the top layer of the epidermis. - Basal cells: Round cells under the squamous cells. - Melanocytes: Cells that make melanin and are found in the lower part of the epidermis. Melanin is the pigment that gives skin its natural color. When skin is exposed to the sun or artificial light, melanocytes make more pigment and cause the skin to darken. The number of new cases of melanoma has been increasing over the last 40 years. Melanoma is most common in adults, but it is sometimes found in children and adolescents. (See the PDQ summary on Unusual Cancers of Childhood Treatment for more information on melanoma in children and adolescents.) There are different types of cancer that start in the skin. There are two forms of skin cancer: melanoma and nonmelanoma. Melanoma is a rare form of skin cancer. It is more likely to invade nearby tissues and spread to other parts of the body than other types of skin cancer. When melanoma starts in the skin, it is called cutaneous melanoma. Melanoma may also occur in mucous membranes (thin, moist layers of tissue that cover surfaces such as the lips). This PDQ summary is about cutaneous (skin) melanoma and melanoma that affects the mucous membranes. The most common types of skin cancer are basal cell carcinoma and squamous cell carcinoma. They are nonmelanoma skin cancers. Nonmelanoma skin cancers rarely spread to other parts of the body. (See the PDQ summary on Skin Cancer Treatment for more information on basal cell and squamous cell skin cancer.) Melanoma can occur anywhere on the skin. In men, melanoma is often found on the trunk (the area from the shoulders to the hips) or the head and neck. In women, melanoma forms most often on the arms and legs. When melanoma occurs in the eye, it is called intraocular or ocular melanoma. (See the PDQ summary on Intraocular (Uveal) Melanoma Treatment for more information.)",Melanoma,0000028_2,CancerGov,https://www.cancer.gov/types/skin/patient/melanoma-treatment-pdq,C0025202,T191,Disorders Who is at risk for Melanoma? ?,0000028_2-2,susceptibility,"Unusual moles, exposure to sunlight, and health history can affect the risk of melanoma. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for melanoma include the following: - Having a fair complexion, which includes the following: - Fair skin that freckles and burns easily, does not tan, or tans poorly. - Blue or green or other light-colored eyes. - Red or blond hair. - Being exposed to natural sunlight or artificial sunlight (such as from tanning beds) over long periods of time. - Being exposed to certain factors in the environment (in the air, your home or workplace, and your food and water). Some of the environmental risk factors for melanoma are radiation, solvents, vinyl chloride, and PCBs. - Having a history of many blistering sunburns, especially as a child or teenager. - Having several large or many small moles. - Having a family history of unusual moles (atypical nevus syndrome). - Having a family or personal history of melanoma. - Being white. - Having a weakened immune system. - Having certain changes in the genes that are linked to melanoma. Being white or having a fair complexion increases the risk of melanoma, but anyone can have melanoma, including people with dark skin. See the following PDQ summaries for more information on risk factors for melanoma: - Genetics of Skin Cancer - Skin Cancer Prevention",Melanoma,0000028_2,CancerGov,https://www.cancer.gov/types/skin/patient/melanoma-treatment-pdq,C0025202,T191,Disorders What are the symptoms of Melanoma ?,0000028_2-3,symptoms,"Signs of melanoma include a change in the way a mole or pigmented area looks. These and other signs and symptoms may be caused by melanoma or by other conditions. Check with your doctor if you have any of the following: - A mole that: - changes in size, shape, or color. - has irregular edges or borders. - is more than one color. - is asymmetrical (if the mole is divided in half, the 2 halves are different in size or shape). - itches. - oozes, bleeds, or is ulcerated (a hole forms in the skin when the top layer of cells breaks down and the tissue below shows through). - A change in pigmented (colored) skin. - Satellite moles (new moles that grow near an existing mole). For pictures and descriptions of common moles and melanoma, see Common Moles, Dysplastic Nevi, and Risk of Melanoma.",Melanoma,0000028_2,CancerGov,https://www.cancer.gov/types/skin/patient/melanoma-treatment-pdq,C0025202,T191,Disorders How to diagnose Melanoma ?,0000028_2-4,exams and tests,"Tests that examine the skin are used to detect (find) and diagnose melanoma. If a mole or pigmented area of the skin changes or looks abnormal, the following tests and procedures can help find and diagnose melanoma: - Skin exam: A doctor or nurse checks the skin for moles, birthmarks, or other pigmented areas that look abnormal in color, size, shape, or texture. - Biopsy : A procedure to remove the abnormal tissue and a small amount of normal tissue around it. A pathologist looks at the tissue under a microscope to check for cancer cells. It can be hard to tell the difference between a colored mole and an early melanoma lesion. Patients may want to have the sample of tissue checked by a second pathologist. If the abnormal mole or lesion is cancer, the sample of tissue may also be tested for certain gene changes. It is important that abnormal areas of the skin not be shaved off or cauterized (destroyed with a hot instrument, an electric current, or a caustic substance) because cancer cells that remain may grow and spread. See the PDQ summary on Skin Cancer Screening for more information.",Melanoma,0000028_2,CancerGov,https://www.cancer.gov/types/skin/patient/melanoma-treatment-pdq,C0025202,T191,Disorders What is the outlook for Melanoma ?,0000028_2-5,outlook,Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - The thickness of the tumor and where it is in the body. - How quickly the cancer cells are dividing. - Whether there was bleeding or ulceration of the tumor. - How much cancer is in the lymph nodes. - The number of places cancer has spread to in the body. - The level of lactate dehydrogenase (LDH) in the blood. - Whether the cancer has certain mutations (changes) in a gene called BRAF. - The patients age and general health.,Melanoma,0000028_2,CancerGov,https://www.cancer.gov/types/skin/patient/melanoma-treatment-pdq,C0025202,T191,Disorders What are the stages of Melanoma ?,0000028_2-6,stages,"Key Points - After melanoma has been diagnosed, tests are done to find out if cancer cells have spread within the skin or to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - The method used to stage melanoma is based mainly on the thickness of the tumor and whether cancer has spread to lymph nodes or other parts of the body. - The following stages are used for melanoma: - Stage 0 (Melanoma in Situ) - Stage I - Stage II - Stage III - Stage IV After melanoma has been diagnosed, tests are done to find out if cancer cells have spread within the skin or to other parts of the body. The process used to find out whether cancer has spread within the skin or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following tests and procedures may be used in the staging process: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Lymph node mapping and sentinel lymph node biopsy : Procedures in which a radioactive substance and/or blue dye is injected near the tumor. The substance or dye flows through lymph ducts to the sentinel node or nodes (the first lymph node or nodes where cancer cells are likely to spread). The surgeon removes only the nodes with the radioactive substance or dye. A pathologist views a sample of tissue under a microscope to check for cancer cells. If no cancer cells are found, it may not be necessary to remove more nodes. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. For melanoma, pictures may be taken of the chest, abdomen, and pelvis. - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. - MRI (magnetic resonance imaging) with gadolinium : A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body, such as the brain. A substance called gadolinium is injected into a vein. The gadolinium collects around the cancer cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. For melanoma, the blood is checked for an enzyme called lactate dehydrogenase (LDH). LDH levels that are higher than normal may be a sign of melanoma. The results of these tests are viewed together with the results of the tumor biopsy to find out the stage of the melanoma. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if melanoma spreads to the lung, the cancer cells in the lung are actually melanoma cells. The disease is metastatic melanoma, not lung cancer. The method used to stage melanoma is based mainly on the thickness of the tumor and whether cancer has spread to lymph nodes or other parts of the body. The staging of melanoma depends on the following: - The thickness of the tumor. The thickness is described using the Breslow scale. - Whether the tumor is ulcerated (has broken through the skin). - Whether the tumor has spread to the lymph nodes and if the lymph nodes are joined together (matted). - Whether the tumor has spread to other parts of the body. The following stages are used for melanoma: Stage 0 (Melanoma in Situ) In stage 0, abnormal melanocytes are found in the epidermis. These abnormal melanocytes may become cancer and spread into nearby normal tissue. Stage 0 is also called melanoma in situ. Stage I In stage I, cancer has formed. Stage I is divided into stages IA and IB. - Stage IA: In stage IA, the tumor is not more than 1 millimeter thick, with no ulceration. - Stage IB: In stage IB, the tumor is either: - not more than 1 millimeter thick and it has ulceration; or - more than 1 but not more than 2 millimeters thick, with no ulceration. Stage II Stage II is divided into stages IIA, IIB, and IIC. - Stage IIA: In stage IIA, the tumor is either: - more than 1 but not more than 2 millimeters thick, with ulceration; or - more than 2 but not more than 4 millimeters thick, with no ulceration. - Stage IIB: In stage IIB, the tumor is either: - more than 2 but not more than 4 millimeters thick, with ulceration; or - more than 4 millimeters thick, with no ulceration. - Stage IIC: In stage IIC, the tumor is more than 4 millimeters thick, with ulceration. Stage III In stage III, the tumor may be any thickness, with or without ulceration. One or more of the following is true: - Cancer has spread to one or more lymph nodes. - Lymph nodes are joined together (matted). - Cancer is in a lymph vessel between the primary tumor and nearby lymph nodes. The cancer is more than 2 centimeters away from the primary tumor. - Very small tumors are found on or under the skin, not more than 2 centimeters away from the primary tumor. Stage IV In stage IV, the cancer has spread to other places in the body, such as the lung, liver, brain, bone, soft tissue, or gastrointestinal (GI) tract. Cancer may have spread to places in the skin far away from where it first started.",Melanoma,0000028_2,CancerGov,https://www.cancer.gov/types/skin/patient/melanoma-treatment-pdq,C0025202,T191,Disorders What are the treatments for Melanoma ?,0000028_2-7,treatment,"Key Points - There are different types of treatment for patients with melanoma. - Five types of standard treatment are used: - Surgery - Chemotherapy - Radiation therapy - Immunotherapy - Targeted therapy - New types of treatment are being tested in clinical trials. - Treatment for melanoma may cause side effects. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with melanoma. Different types of treatment are available for patients with melanoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Five types of standard treatment are used: Surgery Surgery to remove the tumor is the primary treatment of all stages of melanoma. A wide local excision is used to remove the melanoma and some of the normal tissue around it. Skin grafting (taking skin from another part of the body to replace the skin that is removed) may be done to cover the wound caused by surgery. It is important to know whether cancer has spread to the lymph nodes. Lymph node mapping and sentinel lymph node biopsy are done to check for cancer in the sentinel lymph node (the first lymph node the cancer is likely to spread to from the tumor) during surgery. A radioactive substance and/or blue dye is injected near the tumor. The substance or dye flows through the lymph ducts to the lymph nodes. The first lymph node to receive the substance or dye is removed. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are found, more lymph nodes will be removed and tissue samples will be checked for signs of cancer. This is called a lymphadenectomy. Even if the doctor removes all the melanoma that can be seen at the time of surgery, some patients may be given chemotherapy after surgery to kill any cancer cells that are left. Chemotherapy given after surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Surgery to remove cancer that has spread to the lymph nodes, lung, gastrointestinal (GI) tract, bone, or brain may be done to improve the patients quality of life by controlling symptoms. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). One type of regional chemotherapy is hyperthermic isolated limb perfusion. With this method, anticancer drugs go directly to the arm or leg the cancer is in. The flow of blood to and from the limb is temporarily stopped with a tourniquet. A warm solution with the anticancer drug is put directly into the blood of the limb. This gives a high dose of drugs to the area where the cancer is. The way the chemotherapy is given depends on the type and stage of the cancer being treated. See Drugs Approved for Melanoma for more information. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External radiation therapy is used to treat melanoma, and may also be used as palliative therapy to relieve symptoms and improve quality of life. Immunotherapy Immunotherapy is a treatment that uses the patients immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the bodys natural defenses against cancer. This type of cancer treatment is also called biotherapy or biologic therapy. The following types of immunotherapy are being used in the treatment of melanoma: - Immune checkpoint inhibitor therapy: Some types of immune cells, such as T cells, and some cancer cells have certain proteins, called checkpoint proteins, on their surface that keep immune responses in check. When cancer cells have large amounts of these proteins, they will not be attacked and killed by T cells. Immune checkpoint inhibitors block these proteins and the ability of T cells to kill cancer cells is increased. They are used to treat some patients with advanced melanoma or tumors that cannot be removed by surgery. There are two types of immune checkpoint inhibitor therapy: - CTLA-4 inhibitor: CTL4-A is a protein on the surface of T cells that helps keep the bodys immune responses in check. When CTLA-4 attaches to another protein called B7 on a cancer cell, it stops the T cell from killing the cancer cell. CTLA-4 inhibitors attach to CTLA-4 and allow the T cells to kill cancer cells. Ipilimumab is a type of CTLA-4 inhibitor. - PD-1 inhibitor: PD-1 is a protein on the surface of T cells that helps keep the bodys immune responses in check. When PD-1 attaches to another protein called PDL-1 on a cancer cell, it stops the T cell from killing the cancer cell. PD-1 inhibitors attach to PDL-1 and allow the T cells to kill cancer cells. Pembrolizumab and nivolumab are types of PD-1 inhibitors. - Interferon: Interferon affects the division of cancer cells and can slow tumor growth. - Interleukin-2 (IL-2): IL-2 boosts the growth and activity of many immune cells, especially lymphocytes (a type of white blood cell). Lymphocytes can attack and kill cancer cells. - Tumor necrosis factor (TNF) therapy: TNF is a protein made by white blood cells in response to an antigen or infection. TNF is made in the laboratory and used as a treatment to kill cancer cells. It is being studied in the treatment of melanoma. See Drugs Approved for Melanoma for more information. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to attack cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. The following types of targeted therapy are used or being studied in the treatment of melanoma: - Signal transduction inhibitor therapy: Signal transduction inhibitors block signals that are passed from one molecule to another inside a cell. Blocking these signals may kill cancer cells. - Vemurafenib, dabrafenib, trametinib, and cobimetinib are signal transduction inhibitors used to treat some patients with advanced melanoma or tumors that cannot be removed by surgery. Vemurafenib and dabrafenib block the activity of proteins made by mutant BRAF genes. Trametinib and cobimetinib affect the growth and survival of cancer cells. - Oncolytic virus therapy: A type of targeted therapy that is used in the treatment of melanoma. Oncolytic virus therapy uses a virus that infects and breaks down cancer cells but not normal cells. Radiation therapy or chemotherapy may be given after oncolytic virus therapy to kill more cancer cells. - Angiogenesis inhibitors: A type of targeted therapy that is being studied in the treatment of melanoma. Angiogenesis inhibitors block the growth of new blood vessels. In cancer treatment, they may be given to prevent the growth of new blood vessels that tumors need to grow. New targeted therapies and combinations of therapies are being studied in the treatment of melanoma. See Drugs Approved for Melanoma for more information. New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website Treatment for melanoma may cause side effects. For information about side effects caused by treatment for cancer, see our Side Effects page. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options by Stage Stage 0 (Melanoma in Situ) Treatment of stage 0 is usually surgery to remove the area of abnormal cells and a small amount of normal tissue around it. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage 0 melanoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage I Melanoma Treatment of stage I melanoma may include the following: - Surgery to remove the tumor and some of the normal tissue around it. Sometimes lymph node mapping and removal of lymph nodes is also done. - A clinical trial of new ways to find cancer cells in the lymph nodes. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I melanoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage II Melanoma Treatment of stage II melanoma may include the following: - Surgery to remove the tumor and some of the normal tissue around it. Sometimes lymph node mapping and sentinel lymph node biopsy are done to check for cancer in the lymph nodes at the same time as the surgery to remove the tumor. If cancer is found in the sentinel lymph node, more lymph nodes may be removed. - Surgery followed by immunotherapy with interferon if there is a high risk that the cancer will come back. - A clinical trial of new types of treatment to be used after surgery. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage II melanoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage III Melanoma That Can Be Removed By Surgery Treatment of stage III melanoma that can be removed by surgery may include the following: - Surgery to remove the tumor and some of the normal tissue around it. Skin grafting may be done to cover the wound caused by surgery. Sometimes lymph node mapping and sentinel lymph node biopsy are done to check for cancer in the lymph nodes at the same time as the surgery to remove the tumor. If cancer is found in the sentinel lymph node, more lymph nodes may be removed. - Surgery followed by immunotherapy with ipilimumab or interferon if there is a high risk that the cancer will come back. - A clinical trial of immunotherapy or targeted therapy to be used after surgery. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III melanoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage III Melanoma That Cannot Be Removed By Surgery, Stage IV Melanoma, and Recurrent Melanoma Treatment of stage III melanoma that cannot be removed by surgery, stage IV melanoma, and recurrent melanoma may include the following: - Immunotherapy with ipilimumab, pembrolizumab, nivolumab, or interleukin-2 (IL-2). Sometimes ipilimumab and nivolumab are given together. - Targeted therapy with vemurafenib, dabrafenib, trametinib, or cobimetinib. Sometimes vemurafenib and cobimetinib or dabrafenib and trametinib are given together. - Injections into the tumor, such as oncolytic virus therapy. - Chemotherapy. - Palliative therapy to relieve symptoms and improve the quality of life. This may include: - Surgery to remove lymph nodes or tumors in the lung, gastrointestinal (GI) tract, bone, or brain. - Radiation therapy to the brain, spinal cord, or bone. Treatments that are being studied in clinical trials for stage III melanoma that cannot be removed by surgery, stage IV melanoma, and recurrent melanoma include the following: - Immunotherapy alone or in combination with other therapies such as targeted therapy. - Targeted therapy, such as signal transduction inhibitors, angiogenesis inhibitors, oncolytic virus therapy, or drugs that target certain gene mutations. These may be given alone or in combination. - Surgery to remove all known cancer. - Regional chemotherapy (hyperthermic isolated limb perfusion). Some patients may also have immunotherapy with tumor necrosis factor. - Systemic chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage IV melanoma and recurrent melanoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Melanoma,0000028_2,CancerGov,https://www.cancer.gov/types/skin/patient/melanoma-treatment-pdq,C0025202,T191,Disorders what research (or clinical trials) is being done for Melanoma ?,0000028_2-8,research,"New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website Treatment for melanoma may cause side effects. For information about side effects caused by treatment for cancer, see our Side Effects page. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.",Melanoma,0000028_2,CancerGov,https://www.cancer.gov/types/skin/patient/melanoma-treatment-pdq,C0025202,T191,Disorders What is (are) Merkel Cell Carcinoma ?,0000028_3-1,information,"Key Points - Merkel cell carcinoma is a very rare disease in which malignant (cancer) cells form in the skin. - Sun exposure and having a weak immune system can affect the risk of Merkel cell carcinoma. - Merkel cell carcinoma usually appears as a single painless lump on sun-exposed skin. - Tests and procedures that examine the skin are used to detect (find) and diagnose Merkel cell carcinoma. - Certain factors affect prognosis (chance of recovery) and treatment options. Merkel cell carcinoma is a very rare disease in which malignant (cancer) cells form in the skin. Merkel cells are found in the top layer of the skin. These cells are very close to the nerve endings that receive the sensation of touch. Merkel cell carcinoma, also called neuroendocrine carcinoma of the skin or trabecular cancer, is a very rare type of skin cancer that forms when Merkel cells grow out of control. Merkel cell carcinoma starts most often in areas of skin exposed to the sun, especially the head and neck, as well as the arms, legs, and trunk. Merkel cell carcinoma tends to grow quickly and to metastasize (spread) at an early stage. It usually spreads first to nearby lymph nodes and then may spread to lymph nodes or skin in distant parts of the body, lungs, brain, bones, or other organs.",Merkel Cell Carcinoma,0000028_3,CancerGov,https://www.cancer.gov/types/skin/patient/merkel-cell-treatment-pdq,C0007129,T191,Disorders Who is at risk for Merkel Cell Carcinoma? ?,0000028_3-2,susceptibility,"un exposure and having a weak immune system can affect the risk of Merkel cell carcinoma. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for Merkel cell carcinoma include the following: - Being exposed to a lot of natural sunlight. - Being exposed to artificial sunlight, such as from tanning beds or psoralen and ultraviolet A (PUVA) therapy for psoriasis. - Having an immune system weakened by disease, such as chronic lymphocytic leukemia or HIV infection. - Taking drugs that make the immune system less active, such as after an organ transplant. - Having a history of other types of cancer. - Being older than 50 years, male, or white.",Merkel Cell Carcinoma,0000028_3,CancerGov,https://www.cancer.gov/types/skin/patient/merkel-cell-treatment-pdq,C0007129,T191,Disorders What are the symptoms of Merkel Cell Carcinoma ?,0000028_3-3,symptoms,Merkel cell carcinoma usually appears as a single painless lump on sun-exposed skin. This and other changes in the skin may be caused by Merkel cell carcinoma or by other conditions. Check with your doctor if you see changes in your skin. Merkel cell carcinoma usually appears on sun-exposed skin as a single lump that is: - Fast-growing. - Painless. - Firm and dome-shaped or raised. - Red or violet in color.,Merkel Cell Carcinoma,0000028_3,CancerGov,https://www.cancer.gov/types/skin/patient/merkel-cell-treatment-pdq,C0007129,T191,Disorders How to diagnose Merkel Cell Carcinoma ?,0000028_3-4,exams and tests,"Tests and procedures that examine the skin are used to detect (find) and diagnose Merkel cell carcinoma. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Full-body skin exam: A doctor or nurse checks the skin for bumps or spots that look abnormal in color, size, shape, or texture. The size, shape, and texture of the lymph nodes will also be checked. - Skin biopsy : The removal of skin cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer.",Merkel Cell Carcinoma,0000028_3,CancerGov,https://www.cancer.gov/types/skin/patient/merkel-cell-treatment-pdq,C0007129,T191,Disorders What is the outlook for Merkel Cell Carcinoma ?,0000028_3-5,outlook,Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - The stage of the cancer (the size of the tumor and whether it has spread to the lymph nodes or other parts of the body). - Where the cancer is in the body. - Whether the cancer has just been diagnosed or has recurred (come back). - The patient's age and general health. Prognosis also depends on how deeply the tumor has grown into the skin.,Merkel Cell Carcinoma,0000028_3,CancerGov,https://www.cancer.gov/types/skin/patient/merkel-cell-treatment-pdq,C0007129,T191,Disorders What are the stages of Merkel Cell Carcinoma ?,0000028_3-6,stages,"Key Points - After Merkel cell carcinoma has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - The following stages are used for Merkel cell carcinoma: - Stage 0 (carcinoma in situ) - Stage IA - Stage IB - Stage IIA - Stage IIB - Stage IIC - Stage IIIA - Stage IIIB - Stage IV After Merkel cell carcinoma has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body. The process used to find out if cancer has spread to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following tests and procedures may be used in the staging process: - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. A CT scan of the chest and abdomen may be used to check for primary small cell lung cancer, or to find Merkel cell carcinoma that has spread. A CT scan of the head and neck may also be used to find Merkel cell carcinoma that has spread to the lymph nodes. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. - Lymph node biopsy : There are two main types of lymph node biopsy used to stage Merkel cell carcinoma. - Sentinel lymph node biopsy : The removal of the sentinel lymph node during surgery. The sentinel lymph node is the first lymph node to receive lymphatic drainage from a tumor. It is the first lymph node the cancer is likely to spread to from the tumor. A radioactive substance and/or blue dye is injected near the tumor. The substance or dye flows through the lymph ducts to the lymph nodes. The first lymph node to receive the substance or dye is removed. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are not found, it may not be necessary to remove more lymph nodes. - Lymph node dissection : A surgical procedure in which the lymph nodes are removed and a sample of tissue is checked under a microscope for signs of cancer. For a regional lymph node dissection, some of the lymph nodes in the tumor area are removed. For a radical lymph node dissection, most or all of the lymph nodes in the tumor area are removed. This procedure is also called lymphadenectomy. - Immunohistochemistry : A test that uses antibodies to check for certain antigens in a sample of tissue. The antibody is usually linked to a radioactive substance or a dye that causes the tissue to light up under a microscope. This type of test may be used to tell the difference between different types of cancer. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if Merkel cell carcinoma spreads to the liver, the cancer cells in the liver are actually cancerous Merkel cells. The disease is metastatic Merkel cell carcinoma, not liver cancer. The following stages are used for Merkel cell carcinoma: Stage 0 (carcinoma in situ) In stage 0, the tumor is a group of abnormal cells that remain in the place where they first formed and have not spread. These abnormal cells may become cancer and spread to lymph nodes or distant parts of the body. Stage IA In stage IA, the tumor is 2 centimeters or smaller at its widest point and no cancer is found when the lymph nodes are checked under a microscope. Stage IB In stage IB, the tumor is 2 centimeters or smaller at its widest point and no swollen lymph nodes are found by a physical exam or imaging tests. Stage IIA In stage IIA, the tumor is larger than 2 centimeters and no cancer is found when the lymph nodes are checked under a microscope. Stage IIB In stage IIB, the tumor is larger than 2 centimeters and no swollen lymph nodes are found by a physical exam or imaging tests. Stage IIC In stage IIC, the tumor may be any size and has spread to nearby bone, muscle, connective tissue, or cartilage. It has not spread to lymph nodes or distant parts of the body. Stage IIIA In stage IIIA, the tumor may be any size and may have spread to nearby bone, muscle, connective tissue, or cartilage. Cancer is found in the lymph nodes when they are checked under a microscope. Stage IIIB In stage IIIB, the tumor may be any size and may have spread to nearby bone, muscle, connective tissue, or cartilage. Cancer has spread to the lymph nodes near the tumor and is found by a physical exam or imaging test. The lymph nodes are removed and cancer is found in the lymph nodes when they are checked under a microscope. There may also be a second tumor, which is either: - Between the primary tumor and nearby lymph nodes; or - Farther away from the center of the body than the primary tumor is. Stage IV In stage IV, the tumor may be any size and has spread to distant parts of the body, such as the liver, lung, bone, or brain.",Merkel Cell Carcinoma,0000028_3,CancerGov,https://www.cancer.gov/types/skin/patient/merkel-cell-treatment-pdq,C0007129,T191,Disorders What are the treatments for Merkel Cell Carcinoma ?,0000028_3-7,treatment,"Key Points - There are different types of treatment for patients with Merkel cell carcinoma. - Three types of standard treatment are used: - Surgery - Radiation therapy - Chemotherapy - New types of treatment are being tested in clinical trials. - Treatment for Merkel cell carcinoma may cause side effects. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with Merkel cell carcinoma. Different types of treatments are available for patients with Merkel cell carcinoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Three types of standard treatment are used: Surgery One or more of the following surgical procedures may be used to treat Merkel cell carcinoma: - Wide local excision: The cancer is cut from the skin along with some of the tissue around it. A sentinel lymph node biopsy may be done during the wide local excision procedure. If there is cancer in the lymph nodes, a lymph node dissection also may be done. - Lymph node dissection: A surgical procedure in which the lymph nodes are removed and a sample of tissue is checked under a microscope for signs of cancer. For a regional lymph node dissection, some of the lymph nodes in the tumor area are removed; for a radical lymph node dissection, most or all of the lymph nodes in the tumor area are removed. This procedure is also called lymphadenectomy. Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External radiation therapy is used to treat Merkel cell carcinoma, and may also be used as palliative therapy to relieve symptoms and improve quality of life. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. New types of treatment are being tested in clinical trials. Treatment for Merkel cell carcinoma may cause side effects. For information about side effects caused by treatment for cancer, see our Side Effects page. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options by Stage Stage I and Stage II Merkel Cell Carcinoma Treatment of stage I and stage II Merkel cell carcinoma may include the following: - Surgery to remove the tumor, such as wide local excision with or without lymph node dissection. - Radiation therapy after surgery. - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I neuroendocrine carcinoma of the skin and stage II neuroendocrine carcinoma of the skin. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage III Merkel Cell Carcinoma Treatment of stage III Merkel cell carcinoma may include the following: - Wide local excision with or without lymph node dissection. - Radiation therapy. - A clinical trial of chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III neuroendocrine carcinoma of the skin. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage IV Merkel Cell Carcinoma Treatment of stage IV Merkel cell carcinoma may include the following as palliative treatment to relieve symptoms and improve quality of life: - Chemotherapy. - Surgery. - Radiation therapy. - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage IV neuroendocrine carcinoma of the skin. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Merkel Cell Carcinoma,0000028_3,CancerGov,https://www.cancer.gov/types/skin/patient/merkel-cell-treatment-pdq,C0007129,T191,Disorders what research (or clinical trials) is being done for Merkel Cell Carcinoma ?,0000028_3-8,research,"New types of treatment are being tested in clinical trials. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Merkel Cell Carcinoma,0000028_3,CancerGov,https://www.cancer.gov/types/skin/patient/merkel-cell-treatment-pdq,C0007129,T191,Disorders What is (are) Skin Cancer ?,0000028_4-1,information,"Key Points - Skin cancer is a disease in which malignant (cancer) cells form in the tissues of the skin. - There are several types of skin cancer. - Skin cancer is the most common cancer in the United States. Skin cancer is a disease in which malignant (cancer) cells form in the tissues of the skin. The skin is the bodys largest organ. It protects against heat, sunlight, injury, and infection. Skin also helps control body temperature and stores water, fat, and vitamin D. The skin has several layers, but the two main layers are the epidermis (upper or outer layer) and the dermis (lower or inner layer). The epidermis is made up of 3 kinds of cells: - Squamous cells are the thin, flat cells that make up most of the epidermis. - Basal cells are the round cells under the squamous cells. - Melanocytes are found throughout the lower part of the epidermis. They make melanin, the pigment that gives skin its natural color. When skin is exposed to the sun, melanocytes make more pigment, causing the skin to tan, or darken. The dermis contains blood and lymph vessels, hair follicles, and glands. See the following PDQ summaries for more information about skin cancer: - Skin Cancer Screening - Skin Cancer Treatment - Melanoma Treatment - Genetics of Skin Cancer There are several types of skin cancer. The most common types of skin cancer are squamous cell carcinoma, which forms in the squamous cells and basal cell carcinoma, which forms in the basal cells. Squamous cell carcinoma and basal cell carcinoma are also called nonmelanoma skin cancers. Melanoma, which forms in the melanocytes, is a less common type of skin cancer that grows and spreads quickly. Skin cancer can occur anywhere on the body, but it is most common in areas exposed to sunlight, such as the face, neck, hands, and arms. Skin cancer is the most common cancer in the United States. Basal cell carcinoma and squamous cell carcinoma are the most common types of skin cancer in the United States. The number of new cases of nonmelanoma skin cancer appears to be increasing every year. These nonmelanoma skin cancers can usually be cured. The number of new cases of melanoma has been increasing for at least 30 years. Melanoma is more likely to spread to nearby tissues and other parts of the body and can be harder to cure. Finding and treating melanoma skin cancer early may help prevent death from melanoma.",Skin Cancer,0000028_4,CancerGov,https://www.cancer.gov/types/skin/patient/skin-prevention-pdq,C0007114,T191,Disorders How to prevent Skin Cancer ?,0000028_4-2,prevention,"Key Points - Avoiding risk factors and increasing protective factors may help prevent cancer. - Being exposed to ultraviolet radiation is a risk factor for skin cancer. - It is not known if the following lower the risk of nonmelanoma skin cancer: - Sunscreen use and avoiding sun exposure - Chemopreventive agents - It is not known if the following lower the risk of melanoma: - Sunscreen - Counseling and protecting the skin from the sun - Cancer prevention clinical trials are used to study ways to prevent cancer. - New ways to prevent skin cancer are being studied in clinical trials. Avoiding risk factors and increasing protective factors may help prevent cancer. Avoiding cancer risk factors may help prevent certain cancers. Risk factors include smoking, being overweight, and not getting enough exercise. Increasing protective factors such as quitting smoking and exercising may also help prevent some cancers. Talk to your doctor or other health care professional about how you might lower your risk of cancer. Being exposed to ultraviolet radiation is a risk factor for skin cancer. Some studies suggest that being exposed to ultraviolet (UV) radiation and the sensitivity of a persons skin to UV radiation are risk factors for skin cancer. UV radiation is the name for the invisible rays that are part of the energy that comes from the sun. Sunlamps and tanning beds also give off UV radiation. Risk factors for nonmelanoma and melanoma cancers are not the same. - Risk factors for nonmelanoma skin cancer: - Being exposed to natural sunlight or artificial sunlight (such as from tanning beds) over long periods of time. - Having a fair complexion, which includes the following: - Fair skin that freckles and burns easily, does not tan, or tans poorly. - Blue or green or other light-colored eyes. - Red or blond hair. - Having actinic keratosis. - Past treatment with radiation. - Having a weakened immune system. - Being exposed to arsenic. - Risk factors for melanoma skin cancer: - Having a fair complexion, which includes the following: - Fair skin that freckles and burns easily, does not tan, or tans poorly. - Blue or green or other light-colored eyes. - Red or blond hair. - Being exposed to natural sunlight or artificial sunlight (such as from tanning beds) over long periods of time. - Having a history of many blistering sunburns, especially as a child or teenager. - Having several large or many small moles. - Having a family history of unusual moles (atypical nevus syndrome). - Having a family or personal history of melanoma. - Being white. It is not known if the following lower the risk of nonmelanoma skin cancer: Sunscreen use and avoiding sun exposure It is not known if nonmelanoma skin cancer risk is decreased by staying out of the sun, using sunscreens, or wearing protective clothing when outdoors. This is because not enough studies have been done to prove this. Sunscreen may help decrease the amount of UV radiation to the skin. One study found that wearing sunscreen can help prevent actinic keratoses, scaly patches of skin that sometimes become squamous cell carcinoma. The harms of using sunscreen are likely to be small and include allergic reactions to skin creams and lower levels of vitamin D made in the skin because of less sun exposure. It is also possible that when a person uses sunscreen to avoid sunburn they may spend too much time in the sun and be exposed to harmful UV radiation. Although protecting the skin and eyes from the sun has not been proven to lower the chance of getting skin cancer, skin experts suggest the following: - Use sunscreen that protects against UV radiation. - Do not stay out in the sun for long periods of time, especially when the sun is at its strongest. - Wear long sleeve shirts, long pants, sun hats, and sunglasses, when outdoors. Chemopreventive agents Chemoprevention is the use of drugs, vitamins, or other agents to try to reduce the risk of cancer. The following chemopreventive agents have been studied to find whether they lower the risk of nonmelanoma skin cancer: Beta carotene Studies of beta carotene (taken as a supplement in pills) have not shown that it prevents nonmelanoma skin cancer from forming or coming back. Isotretinoin High doses of isotretinoin have been shown to prevent new skin cancers in patients with xeroderma pigmentosum. However, isotretinoin has not been shown to prevent nonmelanoma skin cancers from coming back in patients previously treated for nonmelanoma skin cancers. Treatment with isotretinoin can cause serious side effects. Selenium Studies have shown that selenium (taken in brewer's yeast tablets) does not lower the risk of basal cell carcinoma, and may increase the risk of squamous cell carcinoma. Celecoxib A study of celecoxib in patients with actinic keratosis and a history of nonmelanoma skin cancer found those who took celecoxib had slightly lower rates of recurrent nonmelanoma skin cancers. Celecoxib may have serious side effects on the heart and blood vessels. Alpha-difluoromethylornithine (DFMO) A study of alpha-difluoromethylornithine (DFMO) in patients with a history of nonmelanoma skin cancer showed that those who took DFMO had lower rates of nonmelanoma skin cancers coming back than those who took a placebo. DFMO may cause hearing loss which is usually temporary. Nicotinamide (vitamin B3) Studies have shown that nicotinamide (vitamin B3) helps prevent new actinic keratoses lesions from forming in people who had four or fewer actinic lesions before taking nicotinamide. More studies are needed to find out if nicotinamide prevents nonmelanoma skin cancer from forming or coming back. It is not known if the following lower the risk of melanoma: Sunscreen It has not been proven that using sunscreen to prevent sunburn can protect against melanoma caused by UV radiation. Other risk factors such as having skin that burns easily, having a large number of benign moles, or having atypical nevi may also play a role in whether melanoma forms. Counseling and protecting the skin from the sun It is not known if people who receive counseling or information about avoiding sun exposure make changes in their behavior to protect their skin from the sun. Cancer prevention clinical trials are used to study ways to prevent cancer. Cancer prevention clinical trials are used to study ways to lower the risk of developing certain types of cancer. Some cancer prevention trials are conducted with healthy people who have not had cancer but who have an increased risk for cancer. Other prevention trials are conducted with people who have had cancer and are trying to prevent another cancer of the same type or to lower their chance of developing a new type of cancer. Other trials are done with healthy volunteers who are not known to have any risk factors for cancer. The purpose of some cancer prevention clinical trials is to find out whether actions people take can prevent cancer. These may include eating fruits and vegetables, exercising, quitting smoking, or taking certain medicines, vitamins, minerals, or food supplements. New ways to prevent skin cancer are being studied in clinical trials. Clinical trials are taking place in many parts of the country. Information about clinical trials can be found in the Clinical Trials section of the NCI Web site. Check NCI's list of cancer clinical trials for nonmelanoma skin cancer prevention trials and melanoma prevention trials that are now accepting patients.",Skin Cancer,0000028_4,CancerGov,https://www.cancer.gov/types/skin/patient/skin-prevention-pdq,C0007114,T191,Disorders Who is at risk for Skin Cancer? ?,0000028_4-3,susceptibility,"Key Points - Avoiding risk factors and increasing protective factors may help prevent cancer. - Being exposed to ultraviolet radiation is a risk factor for skin cancer. - It is not known if the following lower the risk of nonmelanoma skin cancer: - Sunscreen use and avoiding sun exposure - Chemopreventive agents - It is not known if the following lower the risk of melanoma: - Sunscreen - Counseling and protecting the skin from the sun - Cancer prevention clinical trials are used to study ways to prevent cancer. - New ways to prevent skin cancer are being studied in clinical trials. Avoiding risk factors and increasing protective factors may help prevent cancer. Avoiding cancer risk factors may help prevent certain cancers. Risk factors include smoking, being overweight, and not getting enough exercise. Increasing protective factors such as quitting smoking and exercising may also help prevent some cancers. Talk to your doctor or other health care professional about how you might lower your risk of cancer. Being exposed to ultraviolet radiation is a risk factor for skin cancer. Some studies suggest that being exposed to ultraviolet (UV) radiation and the sensitivity of a persons skin to UV radiation are risk factors for skin cancer. UV radiation is the name for the invisible rays that are part of the energy that comes from the sun. Sunlamps and tanning beds also give off UV radiation. Risk factors for nonmelanoma and melanoma cancers are not the same. - Risk factors for nonmelanoma skin cancer: - Being exposed to natural sunlight or artificial sunlight (such as from tanning beds) over long periods of time. - Having a fair complexion, which includes the following: - Fair skin that freckles and burns easily, does not tan, or tans poorly. - Blue or green or other light-colored eyes. - Red or blond hair. - Having actinic keratosis. - Past treatment with radiation. - Having a weakened immune system. - Being exposed to arsenic. - Risk factors for melanoma skin cancer: - Having a fair complexion, which includes the following: - Fair skin that freckles and burns easily, does not tan, or tans poorly. - Blue or green or other light-colored eyes. - Red or blond hair. - Being exposed to natural sunlight or artificial sunlight (such as from tanning beds) over long periods of time. - Having a history of many blistering sunburns, especially as a child or teenager. - Having several large or many small moles. - Having a family history of unusual moles (atypical nevus syndrome). - Having a family or personal history of melanoma. - Being white. It is not known if the following lower the risk of nonmelanoma skin cancer: Sunscreen use and avoiding sun exposure It is not known if nonmelanoma skin cancer risk is decreased by staying out of the sun, using sunscreens, or wearing protective clothing when outdoors. This is because not enough studies have been done to prove this. Sunscreen may help decrease the amount of UV radiation to the skin. One study found that wearing sunscreen can help prevent actinic keratoses, scaly patches of skin that sometimes become squamous cell carcinoma. The harms of using sunscreen are likely to be small and include allergic reactions to skin creams and lower levels of vitamin D made in the skin because of less sun exposure. It is also possible that when a person uses sunscreen to avoid sunburn they may spend too much time in the sun and be exposed to harmful UV radiation. Although protecting the skin and eyes from the sun has not been proven to lower the chance of getting skin cancer, skin experts suggest the following: - Use sunscreen that protects against UV radiation. - Do not stay out in the sun for long periods of time, especially when the sun is at its strongest. - Wear long sleeve shirts, long pants, sun hats, and sunglasses, when outdoors. Chemopreventive agents Chemoprevention is the use of drugs, vitamins, or other agents to try to reduce the risk of cancer. The following chemopreventive agents have been studied to find whether they lower the risk of nonmelanoma skin cancer: Beta carotene Studies of beta carotene (taken as a supplement in pills) have not shown that it prevents nonmelanoma skin cancer from forming or coming back. Isotretinoin High doses of isotretinoin have been shown to prevent new skin cancers in patients with xeroderma pigmentosum. However, isotretinoin has not been shown to prevent nonmelanoma skin cancers from coming back in patients previously treated for nonmelanoma skin cancers. Treatment with isotretinoin can cause serious side effects. Selenium Studies have shown that selenium (taken in brewer's yeast tablets) does not lower the risk of basal cell carcinoma, and may increase the risk of squamous cell carcinoma. Celecoxib A study of celecoxib in patients with actinic keratosis and a history of nonmelanoma skin cancer found those who took celecoxib had slightly lower rates of recurrent nonmelanoma skin cancers. Celecoxib may have serious side effects on the heart and blood vessels. Alpha-difluoromethylornithine (DFMO) A study of alpha-difluoromethylornithine (DFMO) in patients with a history of nonmelanoma skin cancer showed that those who took DFMO had lower rates of nonmelanoma skin cancers coming back than those who took a placebo. DFMO may cause hearing loss which is usually temporary. Nicotinamide (vitamin B3) Studies have shown that nicotinamide (vitamin B3) helps prevent new actinic keratoses lesions from forming in people who had four or fewer actinic lesions before taking nicotinamide. More studies are needed to find out if nicotinamide prevents nonmelanoma skin cancer from forming or coming back. It is not known if the following lower the risk of melanoma: Sunscreen It has not been proven that using sunscreen to prevent sunburn can protect against melanoma caused by UV radiation. Other risk factors such as having skin that burns easily, having a large number of benign moles, or having atypical nevi may also play a role in whether melanoma forms. Counseling and protecting the skin from the sun It is not known if people who receive counseling or information about avoiding sun exposure make changes in their behavior to protect their skin from the sun.",Skin Cancer,0000028_4,CancerGov,https://www.cancer.gov/types/skin/patient/skin-prevention-pdq,C0007114,T191,Disorders what research (or clinical trials) is being done for Skin Cancer ?,0000028_4-4,research,"Cancer prevention clinical trials are used to study ways to prevent cancer. Cancer prevention clinical trials are used to study ways to lower the risk of developing certain types of cancer. Some cancer prevention trials are conducted with healthy people who have not had cancer but who have an increased risk for cancer. Other prevention trials are conducted with people who have had cancer and are trying to prevent another cancer of the same type or to lower their chance of developing a new type of cancer. Other trials are done with healthy volunteers who are not known to have any risk factors for cancer. The purpose of some cancer prevention clinical trials is to find out whether actions people take can prevent cancer. These may include eating fruits and vegetables, exercising, quitting smoking, or taking certain medicines, vitamins, minerals, or food supplements. New ways to prevent skin cancer are being studied in clinical trials. Clinical trials are taking place in many parts of the country. Information about clinical trials can be found in the Clinical Trials section of the NCI Web site. Check NCI's list of cancer clinical trials for nonmelanoma skin cancer prevention trials and melanoma prevention trials that are now accepting patients.",Skin Cancer,0000028_4,CancerGov,https://www.cancer.gov/types/skin/patient/skin-prevention-pdq,C0007114,T191,Disorders What is (are) Skin Cancer ?,0000028_5-1,information,"Key Points - Skin cancer is a disease in which malignant (cancer) cells form in the tissues of the skin. - Nonmelanoma skin cancer is the most common cancer in the United States. - Being exposed to ultraviolet radiation may increase the risk of skin cancer. Skin cancer is a disease in which malignant (cancer) cells form in the tissues of the skin. The skin is the body's largest organ. It protects against heat, sunlight, injury, and infection. Skin also helps control body temperature and stores water, fat, and vitamin D. The skin has several layers, but the two main layers are the epidermis (top or outer layer) and the dermis (lower or inner layer). Skin cancer begins in the epidermis, which is made up of three kinds of cells: - Squamous cells: Thin, flat cells that form the top layer of the epidermis. Cancer that forms in squamous cells is called squamous cell carcinoma. - Basal cells: Round cells under the squamous cells. Cancer that forms in basal cells is called basal cell carcinoma. - Melanocytes: Found in the lower part of the epidermis, these cells make melanin, the pigment that gives skin its natural color. When skin is exposed to the sun, melanocytes make more pigment and cause the skin to tan, or darken. Cancer that forms in melanocytes is called melanoma. Nonmelanoma skin cancer is the most common cancer in the United States. Basal cell carcinoma and squamous cell carcinoma are also called nonmelanoma skin cancer and are the most common forms of skin cancer. Most basal cell and squamous cell skin cancers can be cured. Melanoma is more likely to spread to nearby tissues and other parts of the body and can be harder to cure. Melanoma is easier to cure if the tumor is found before it spreads to the dermis (inner layer of skin). Melanoma is less likely to cause death when it is found and treated early. In the United States, the number of cases of nonmelanoma skin cancer seems to have increased in recent years. The number of cases of melanoma has increased over the last 30 years. Part of the reason for these increases may be that people are more aware of skin cancer. They are more likely to have skin exams and biopsies and to be diagnosed with skin cancer. Over the past 20 years, the number of deaths from melanoma has decreased slightly among white men and women younger than 50 years. During that time, the number of deaths from melanoma has increased slightly among white men older than 50 years and stayed about the same among white women older than 50 years. The number of cases of childhood melanoma diagnosed in the United States is low, but increasing over time. The number of childhood deaths from melanoma has stayed about the same. See the following PDQ summaries for more information about skin cancer: - Skin Cancer Prevention - Skin Cancer Treatment - Melanoma Treatment - Genetics of Skin Cancer",Skin Cancer,0000028_5,CancerGov,https://www.cancer.gov/types/skin/patient/skin-screening-pdq,C0007114,T191,Disorders Who is at risk for Skin Cancer? ?,0000028_5-2,susceptibility,"Being exposed to ultraviolet radiation may increase the risk of skin cancer. Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. People who think they may be at risk should discuss this with their doctor. Being exposed to ultraviolet (UV) radiation and having skin that is sensitive to UV radiation are risk factors for skin cancer. UV radiation is the name for the invisible rays that are part of the energy that comes from the sun. Sunlamps and tanning beds also give off UV radiation. Risk factors for nonmelanoma and melanoma cancers are not the same. - Nonmelanoma skin cancer risk factors include: - Being exposed to natural sunlight or artificial sunlight (such as from tanning beds) over long periods of time. - Having a fair complexion, which includes the following: - Fair skin that freckles and burns easily, does not tan, or tans poorly. - Blue or green or other light-colored eyes. - Red or blond hair. - Having actinic keratosis. - Past treatment with radiation. - Having a weakened immune system. - Being exposed to arsenic. - Melanoma skin cancer risk factors include: - Having a fair complexion, which includes the following: - Fair skin that freckles and burns easily, does not tan, or tans poorly. - Blue or green or other light-colored eyes. - Red or blond hair. - Being exposed to natural sunlight or artificial sunlight (such as from tanning beds) over long periods of time. - Having a history of many blistering sunburns, especially as a child or teenager. - Having several large or many small moles. - Having a family history of unusual moles (atypical nevus syndrome). - Having a family or personal history of melanoma. - Being white.",Skin Cancer,0000028_5,CancerGov,https://www.cancer.gov/types/skin/patient/skin-screening-pdq,C0007114,T191,Disorders What is (are) Plasma Cell Neoplasms (Including Multiple Myeloma) ?,0000030_1-1,information,"Key Points - Plasma cell neoplasms are diseases in which the body makes too many plasma cells. - Plasma cell neoplasms can be benign (not cancer) or malignant (cancer). - There are several types of plasma cell neoplasms. - Monoclonal gammopathy of undetermined significance (MGUS) - Plasmacytoma - Multiple myeloma - Multiple myeloma and other plasma cell neoplasms may cause a condition called amyloidosis. - Age can affect the risk of plasma cell neoplasms. - Tests that examine the blood, bone marrow, and urine are used to detect (find) and diagnose multiple myeloma and other plasma cell neoplasms. - Certain factors affect prognosis (chance of recovery) and treatment options. Plasma cell neoplasms are diseases in which the body makes too many plasma cells. Plasma cells develop from B lymphocytes (B cells), a type of white blood cell that is made in the bone marrow. Normally, when bacteria or viruses enter the body, some of the B cells will change into plasma cells. The plasma cells make antibodies to fight bacteria and viruses, to stop infection and disease. Plasma cell neoplasms are diseases in which abnormal plasma cells or myeloma cells form tumors in the bones or soft tissues of the body. The plasma cells also make an antibody protein, called M protein, that is not needed by the body and does not help fight infection. These antibody proteins build up in the bone marrow and can cause the blood to thicken or can damage the kidneys. Plasma cell neoplasms can be benign (not cancer) or malignant (cancer). Monoclonal gammopathy of undetermined significance (MGUS) is not cancer but can become cancer. The following types of plasma cell neoplasms are cancer: - Lymphoplasmacytic lymphoma. (See Adult Non-Hodgkin Lymphoma Treatment for more information.) - Plasmacytoma. - Multiple myeloma. There are several types of plasma cell neoplasms. Plasma cell neoplasms include the following: Monoclonal gammopathy of undetermined significance (MGUS) In this type of plasma cell neoplasm, less than 10% of the bone marrow is made up of abnormal plasma cells and there is no cancer. The abnormal plasma cells make M protein, which is sometimes found during a routine blood or urine test. In most patients, the amount of M protein stays the same and there are no signs, symptoms, or health problems. In some patients, MGUS may later become a more serious condition, such as amyloidosis, or cause problems with the kidneys, heart, or nerves. MGUS can also become cancer, such as multiple myeloma, lymphoplasmacytic lymphoma, or chronic lymphocytic leukemia. Plasmacytoma In this type of plasma cell neoplasm, the abnormal plasma cells (myeloma cells) are in one place and form one tumor, called a plasmacytoma. Sometimes plasmacytoma can be cured. There are two types of plasmacytoma. - In isolated plasmacytoma of bone, one plasma cell tumor is found in the bone, less than 10% of the bone marrow is made up of plasma cells, and there are no other signs of cancer. Plasmacytoma of the bone often becomes multiple myeloma. - In extramedullary plasmacytoma, one plasma cell tumor is found in soft tissue but not in the bone or the bone marrow. Extramedullary plasmacytomas commonly form in tissues of the throat, tonsil, and paranasal sinuses. Signs and symptoms depend on where the tumor is. - In bone, the plasmacytoma may cause pain or broken bones. - In soft tissue, the tumor may press on nearby areas and cause pain or other problems. For example, a plasmacytoma in the throat can make it hard to swallow. Multiple myeloma In multiple myeloma, abnormal plasma cells (myeloma cells) build up in the bone marrow and form tumors in many bones of the body. These tumors may keep the bone marrow from making enough healthy blood cells. Normally, the bone marrow makes stem cells (immature cells) that become three types of mature blood cells: - Red blood cells that carry oxygen and other substances to all tissues of the body. - White blood cells that fight infection and disease. - Platelets that form blood clots to help prevent bleeding. As the number of myeloma cells increases, fewer red blood cells, white blood cells, and platelets are made. The myeloma cells also damage and weaken the bone. Sometimes multiple myeloma does not cause any signs or symptoms. This is called smoldering multiple myeloma. It may be found when a blood or urine test is done for another condition. Signs and symptoms may be caused by multiple myeloma or other conditions. Check with your doctor if you have any of the following: - Bone pain, especially in the back or ribs. - Bones that break easily. - Fever for no known reason or frequent infections. - Easy bruising or bleeding. - Trouble breathing. - Weakness of the arms or legs. - Feeling very tired. A tumor can damage the bone and cause hypercalcemia (too much calcium in the blood). This can affect many organs in the body, including the kidneys, nerves, heart, muscles, and digestive tract, and cause serious health problems. Hypercalcemia may cause the following signs and symptoms: - Loss of appetite. - Nausea or vomiting. - Feeling thirsty. - Frequent urination. - Constipation. - Feeling very tired. - Muscle weakness. - Restlessness. - Confusion or trouble thinking. Multiple myeloma and other plasma cell neoplasms may cause a condition called amyloidosis. In rare cases, multiple myeloma can cause peripheral nerves (nerves that are not in the brain or spinal cord) and organs to fail. This may be caused by a condition called amyloidosis. Antibody proteins build up and stick together in peripheral nerves and organs, such as the kidney and heart. This can cause the nerves and organs to become stiff and unable to work the way they should. Amyloidosis may cause the following signs and symptoms: - Feeling very tired. - Purple spots on the skin. - Enlarged tongue. - Diarrhea. - Swelling caused by fluid in your body's tissues. - Tingling or numbness in your legs and feet.",Plasma Cell Neoplasms (Including Multiple Myeloma),0000030_1,CancerGov,https://www.cancer.gov/types/myeloma/patient/myeloma-treatment-pdq,C0026764,T191,Disorders Who is at risk for Plasma Cell Neoplasms (Including Multiple Myeloma)? ?,0000030_1-2,susceptibility,"Age can affect the risk of plasma cell neoplasms. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Plasma cell neoplasms are most common in people who are middle aged or older. For multiple myeloma and plasmacytoma, other risk factors include the following: - Being black. - Being male. - Having a personal history of MGUS or plasmacytoma. - Being exposed to radiation or certain chemicals.",Plasma Cell Neoplasms (Including Multiple Myeloma),0000030_1,CancerGov,https://www.cancer.gov/types/myeloma/patient/myeloma-treatment-pdq,C0026764,T191,Disorders How to diagnose Plasma Cell Neoplasms (Including Multiple Myeloma) ?,0000030_1-3,exams and tests,"Tests that examine the blood, bone marrow, and urine are used to detect (find) and diagnose multiple myeloma and other plasma cell neoplasms. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Blood and urine immunoglobulin studies: A procedure in which a blood or urine sample is checked to measure the amounts of certain antibodies (immunoglobulins). For multiple myeloma, beta-2-microglobulin, M protein, free light chains, and other proteins made by the myeloma cells are measured. A higher-than-normal amount of these substances can be a sign of disease. - Bone marrow aspiration and biopsy : The removal of bone marrow, blood, and a small piece of bone by inserting a hollow needle into the hipbone or breastbone. A pathologist views the bone marrow, blood, and bone under a microscope to look for abnormal cells. The following test may be done on the sample of tissue removed during the bone marrow aspiration and biopsy: - Cytogenetic analysis : A test in which cells in a sample of bone marrow are viewed under a microscope to look for certain changes in the chromosomes. Other tests, such as fluorescence in situ hybridization (FISH) and flow cytometry, may also be done to look for certain changes in the chromosomes. - Skeletal bone survey: In a skeletal bone survey, x-rays of all the bones in the body are taken. The x-rays are used to find areas where the bone is damaged. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. - Complete blood count (CBC) with differential : A procedure in which a sample of blood is drawn and checked for the following: - The number of red blood cells and platelets. - The number and type of white blood cells. - The amount of hemoglobin (the protein that carries oxygen) in the red blood cells. - The portion of the blood sample made up of red blood cells. - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances, such as calcium or albumin, released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. - Twenty-four-hour urine test: A test in which urine is collected for 24 hours to measure the amounts of certain substances. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. A higher than normal amount of protein may be a sign of multiple myeloma. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). An MRI of the spine and pelvis may be used to find areas where the bone is damaged. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the spine, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - PET-CT scan : A procedure that combines the pictures from a positron emission tomography (PET) scan and a computed tomography (CT) scan. The PET and CT scans are done at the same time with the same machine. The combined scans give more detailed pictures of areas inside the body, such as the spine, than either scan gives by itself.",Plasma Cell Neoplasms (Including Multiple Myeloma),0000030_1,CancerGov,https://www.cancer.gov/types/myeloma/patient/myeloma-treatment-pdq,C0026764,T191,Disorders What is the outlook for Plasma Cell Neoplasms (Including Multiple Myeloma) ?,0000030_1-4,outlook,"Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) depends on the following: - The type of plasma cell neoplasm. - The stage of the disease. - Whether a certain immunoglobulin (antibody) is present. - Whether there are certain genetic changes. - Whether the kidney is damaged. - Whether the cancer responds to initial treatment or recurs (comes back). Treatment options depend on the following: - The type of plasma cell neoplasm. - The age and general health of the patient. - Whether there are signs, symptoms, or health problems, such as kidney failure or infection, related to the disease. - Whether the cancer responds to initial treatment or recurs (comes back).",Plasma Cell Neoplasms (Including Multiple Myeloma),0000030_1,CancerGov,https://www.cancer.gov/types/myeloma/patient/myeloma-treatment-pdq,C0026764,T191,Disorders What are the stages of Plasma Cell Neoplasms (Including Multiple Myeloma) ?,0000030_1-5,stages,"Key Points - There are no standard staging systems for monoclonal gammopathy of undetermined significance (MGUS), macroglobulinemia, and plasmacytoma. - After multiple myeloma has been diagnosed, tests are done to find out the amount of cancer in the body. - The stage of multiple myeloma is based on the levels of beta-2-microglobulin and albumin in the blood. - The following stages are used for multiple myeloma: - Stage I multiple myeloma - Stage II multiple myeloma - Stage III multiple myeloma There are no standard staging systems for monoclonal gammopathy of undetermined significance (MGUS), macroglobulinemia, and plasmacytoma. After multiple myeloma has been diagnosed, tests are done to find out the amount of cancer in the body. The process used to find out the amount of cancer in the body is called staging. It is important to know the stage in order to plan treatment. The following tests and procedures may be used in the staging process: - Skeletal bone survey: In a skeletal bone survey, x-rays of all the bones in the body are taken. The x-rays are used to find areas where the bone is damaged. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body, such as the bone marrow. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Bone densitometry: A procedure that uses a special type of x-ray to measure bone density. The stage of multiple myeloma is based on the levels of beta-2-microglobulin and albumin in the blood. Beta-2-microglobulin and albumin are found in the blood. Beta-2-microglobulin is a protein found on plasma cells. Albumin makes up the biggest part of the blood plasma. It keeps fluid from leaking out of blood vessels. It also brings nutrients to tissues, and carries hormones, vitamins, drugs, and other substances, such as calcium, all through the body. In the blood of patients with multiple myeloma, the amount of beta-2-microglobulin is increased and the amount of albumin is decreased. The following stages are used for multiple myeloma: Stage I multiple myeloma In stage I multiple myeloma, the blood levels are as follows: - beta-2-microglobulin level is lower than 3.5 mg/L; and - albumin level is 3.5 g/dL or higher. Stage II multiple myeloma In stage II multiple myeloma, the blood levels are in between the levels for stage I and stage III. Stage III multiple myeloma In stage III multiple myeloma, the blood level of beta-2-microglobulin is 5.5 mg/L or higher and the patient also has one of the following: - high levels of lactate dehydrogenase (LDH); or - certain changes in the chromosomes.",Plasma Cell Neoplasms (Including Multiple Myeloma),0000030_1,CancerGov,https://www.cancer.gov/types/myeloma/patient/myeloma-treatment-pdq,C0026764,T191,Disorders What are the treatments for Plasma Cell Neoplasms (Including Multiple Myeloma) ?,0000030_1-6,treatment,"Key Points - There are different types of treatment for patients with plasma cell neoplasms. - Eight types of treatment are used: - Chemotherapy - Other drug therapy - Targeted therapy - High-dose chemotherapy with stem cell transplant - Biologic therapy - Radiation therapy - Surgery - Watchful waiting - New types of treatment are being tested in clinical trials. - New combinations of therapies - Supportive care is given to lessen the problems caused by the disease or its treatment. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with plasma cell neoplasms. Different types of treatments are available for patients with plasma cell neoplasms. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Eight types of treatment are used: Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. See Drugs Approved for Multiple Myeloma and Other Plasma Cell Neoplasms for more information. Other drug therapy Corticosteroids are steroids that have antitumor effects in multiple myeloma. Targeted therapy Targeted therapy is a treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Several types of targeted therapy may be used to treat multiple myeloma and other plasma cell neoplasms. Proteasome inhibitor therapy is a type of targeted therapy that blocks the action of proteasomes in cancer cells and may prevent the growth of tumors. Bortezomib, carfilzomib, ixazomib, daratumumab, and elotuzumab are proteasome inhibitors used in the treatment of multiple myeloma and other plasma cell neoplasms. Histone deacetylase (HDAC) inhibitor therapy is a type of targeted therapy that blocks enzymes needed for cell division and may stop the growth of cancer cells. Panobinostat is an HDAC inhibitor used in the treatment of multiple myeloma and other plasma cell neoplasms. See Drugs Approved for Multiple Myeloma and Other Plasma Cell Neoplasms for more information. High-dose chemotherapy with stem cell transplant This treatment is a way of giving high doses of chemotherapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient (autologous transplant) or a donor (allogeneic transplant) and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. Biologic therapy Biologic therapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy. Immunomodulators are a type of biologic therapy. Thalidomide, lenalidomide, and pomalidomide are immunomodulators used to treat multiple myeloma and other plasma cell neoplasms. Interferon is a type of biologic therapy. It affects the division of cancer cells and can slow tumor growth. See Drugs Approved for Multiple Myeloma and Other Plasma Cell Neoplasms for more information. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External radiation therapy is used to treat plasma cell neoplasms. Surgery Surgery to remove the tumor may be done and is usually followed by radiation therapy. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Watchful waiting Watchful waiting is closely monitoring a patients condition without giving any treatment until signs or symptoms appear or change. New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. New combinations of therapies Clinical trials are studying different combinations of biologic therapy, chemotherapy, steroid therapy, and drugs. New treatment regimens using thalidomide or lenalidomide are also being studied. Supportive care is given to lessen the problems caused by the disease or its treatment. This therapy controls problems or side effects caused by the disease or its treatment, and improves quality of life. Supportive care is given to treat problems caused by multiple myeloma and other plasma cell neoplasms. Supportive care may include the following: - Plasmapheresis: If the blood becomes thick with extra antibody proteins and interferes with circulation, plasmapheresis is done to remove extra plasma and antibody proteins from the blood. In this procedure blood is removed from the patient and sent through a machine that separates the plasma (the liquid part of the blood) from the blood cells. The patient's plasma contains the unneeded antibodies and is not returned to the patient. The normal blood cells are returned to the bloodstream along with donated plasma or a plasma replacement. Plasmapheresis does not keep new antibodies from forming. - High-dose chemotherapy with stem cell transplant: If amyloidosis occurs, treatment may include high-dose chemotherapy followed by stem cell transplant using the patient's own stem cells. - Biologic therapy: Biologic therapy with thalidomide, lenalidomide, or pomalidomide is given to treat amyloidosis. - Targeted therapy: Targeted therapy with proteasome inhibitors is given to treat amyloidosis. - Radiation therapy: Radiation therapy is given for bone lesions of the spine. - Chemotherapy: Chemotherapy is given to reduce back pain from osteoporosis or compression fractures of the spine. - Bisphosphonate therapy: Bisphosphonate therapy is given to slow bone loss and reduce bone pain. See the following PDQ summaries for more information on bisphosphonates and problems related to their use: - Cancer Pain - Oral Complications of Chemotherapy and Head/Neck Radiation Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Plasma Cell Neoplasms Monoclonal Gammopathy of Undetermined Significance Treatment of monoclonal gammopathy of undetermined significance (MGUS) is usually watchful waiting. Regular blood tests to check the level of M protein in the blood and physical exams to check for signs or symptoms of cancer will be done. Check the list of NCI-supported cancer clinical trials that are now accepting patients with monoclonal gammopathy of undetermined significance. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Isolated Plasmacytoma of Bone Treatment of isolated plasmacytoma of bone is usually radiation therapy to the bone lesion. Check the list of NCI-supported cancer clinical trials that are now accepting patients with isolated plasmacytoma of bone. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Extramedullary Plasmacytoma Treatment of extramedullary plasmacytoma may include the following: - Radiation therapy to the tumor and nearby lymph nodes. - Surgery, usually followed by radiation therapy. - Watchful waiting after initial treatment, followed by radiation therapy, surgery, or chemotherapy if the tumor grows or causes signs or symptoms. Check the list of NCI-supported cancer clinical trials that are now accepting patients with extramedullary plasmacytoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Multiple Myeloma Patients without signs or symptoms may not need treatment. When signs or symptoms appear, the treatment of multiple myeloma may be done in phases: - Induction therapy : This is the first phase of treatment. Its goal is to reduce the amount of disease, and may include one or more of the following: - Corticosteroid therapy. - Biologic therapy with lenalidomide, pomalidomide, or thalidomide therapy. - Targeted therapy with proteasome inhibitors (bortezomib, carfilzomib, ixazomib, daratumumab, and elotuzumab). - Chemotherapy. - Histone deacetylase inhibitor therapy with panobinostat. - A clinical trial of different combinations of treatment. - Consolidation chemotherapy : This is the second phase of treatment. Treatment in the consolidation phase is to kill any remaining cancer cells. High-dose chemotherapy is followed by either: - one autologous stem cell transplant, in which the patient's stem cells from the blood or bone marrow are used; or - two autologous stem cell transplants followed by an autologous or allogeneic stem cell transplant, in which the patient receives stem cells from the blood or bone marrow of a donor; or - one allogeneic stem cell transplant. - Maintenance therapy : After the initial treatment, maintenance therapy is often given to help keep the disease in remission for a longer time. Several types of treatment are being studied for this use, including the following: - Chemotherapy. - Biologic therapy with interferon. - Corticosteroid therapy. - Lenalidomide therapy. - Targeted therapy with a proteasome inhibitor (bortezomib). Check the list of NCI-supported cancer clinical trials that are now accepting patients with multiple myeloma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Refractory Multiple Myeloma Treatment of refractory multiple myeloma may include the following: - Watchful waiting for patients whose disease is stable. - A different treatment than treatment already given, for patients whose tumor kept growing during treatment. (See Multiple Myeloma treatment options.) - A clinical trial of a new therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with refractory multiple myeloma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Plasma Cell Neoplasms (Including Multiple Myeloma),0000030_1,CancerGov,https://www.cancer.gov/types/myeloma/patient/myeloma-treatment-pdq,C0026764,T191,Disorders what research (or clinical trials) is being done for Plasma Cell Neoplasms (Including Multiple Myeloma) ?,0000030_1-7,research,"New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. New combinations of therapies Clinical trials are studying different combinations of biologic therapy, chemotherapy, steroid therapy, and drugs. New treatment regimens using thalidomide or lenalidomide are also being studied. Supportive care is given to lessen the problems caused by the disease or its treatment. This therapy controls problems or side effects caused by the disease or its treatment, and improves quality of life. Supportive care is given to treat problems caused by multiple myeloma and other plasma cell neoplasms. Supportive care may include the following: - Plasmapheresis: If the blood becomes thick with extra antibody proteins and interferes with circulation, plasmapheresis is done to remove extra plasma and antibody proteins from the blood. In this procedure blood is removed from the patient and sent through a machine that separates the plasma (the liquid part of the blood) from the blood cells. The patient's plasma contains the unneeded antibodies and is not returned to the patient. The normal blood cells are returned to the bloodstream along with donated plasma or a plasma replacement. Plasmapheresis does not keep new antibodies from forming. - High-dose chemotherapy with stem cell transplant: If amyloidosis occurs, treatment may include high-dose chemotherapy followed by stem cell transplant using the patient's own stem cells. - Biologic therapy: Biologic therapy with thalidomide, lenalidomide, or pomalidomide is given to treat amyloidosis. - Targeted therapy: Targeted therapy with proteasome inhibitors is given to treat amyloidosis. - Radiation therapy: Radiation therapy is given for bone lesions of the spine. - Chemotherapy: Chemotherapy is given to reduce back pain from osteoporosis or compression fractures of the spine. - Bisphosphonate therapy: Bisphosphonate therapy is given to slow bone loss and reduce bone pain. See the following PDQ summaries for more information on bisphosphonates and problems related to their use: - Cancer Pain - Oral Complications of Chemotherapy and Head/Neck Radiation Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Plasma Cell Neoplasms (Including Multiple Myeloma),0000030_1,CancerGov,https://www.cancer.gov/types/myeloma/patient/myeloma-treatment-pdq,C0026764,T191,Disorders What is (are) Neuroblastoma ?,0000031_1-1,information,"Key Points - Neuroblastoma is a disease in which malignant (cancer) cells form in neuroblasts (immature nerve tissue) in the adrenal gland, neck, chest, or spinal cord. - Neuroblastoma is sometimes caused by a gene mutation (change) passed from the parent to the child. - Signs and symptoms of neuroblastoma include bone pain and a lump in the abdomen, neck, or chest. - Tests that examine many different body tissues and fluids are used to detect (find) and diagnose neuroblastoma. - A biopsy is done to diagnose neuroblastoma. - Certain factors affect prognosis (chance of recovery) and treatment options. Neuroblastoma is a disease in which malignant (cancer) cells form in neuroblasts (immature nerve tissue) in the adrenal gland, neck, chest, or spinal cord. Neuroblastoma often begins in the nerve tissue of the adrenal glands. There are two adrenal glands, one on top of each kidney in the back of the upper abdomen. The adrenal glands make important hormones that help control heart rate, blood pressure, blood sugar, and the way the body reacts to stress. Neuroblastoma may also begin in nerve tissue in the neck, chest, abdomen or pelvis. Neuroblastoma most often begins in infancy and may be diagnosed in the first month of life. It is found when the tumor begins to grow and cause signs or symptoms. Sometimes it forms before birth and is found during a fetal ultrasound. By the time neuroblastoma is diagnosed, the cancer has usually metastasized (spread). Neuroblastoma spreads most often to the lymph nodes, bones, bone marrow, and liver. In infants, it also spreads to the skin.",Neuroblastoma,0000031_1,CancerGov,https://www.cancer.gov/types/neuroblastoma/patient/neuroblastoma-treatment-pdq,C0027819,T191,Disorders Is Neuroblastoma inherited ?,0000031_1-2,inheritance,"Neuroblastoma is sometimes caused by a gene mutation (change) passed from the parent to the child. Gene mutations that increase the risk of neuroblastoma are sometimes inherited (passed from the parent to the child). In children with a gene mutation, neuroblastoma usually occurs at a younger age and more than one tumor may form in the adrenal glands.",Neuroblastoma,0000031_1,CancerGov,https://www.cancer.gov/types/neuroblastoma/patient/neuroblastoma-treatment-pdq,C0027819,T191,Disorders What are the symptoms of Neuroblastoma ?,0000031_1-3,symptoms,"Signs and symptoms of neuroblastoma include bone pain and a lump in the abdomen, neck, or chest.The most common signs and symptoms of neuroblastoma are caused by the tumor pressing on nearby tissues as it grows or by cancer spreading to the bone. These and other signs and symptoms may be caused by neuroblastoma or by other conditions. Check with your childs doctor if your child has any of the following: - Lump in the abdomen, neck, or chest. - Bulging eyes. - Dark circles around the eyes (""black eyes""). - Bone pain. - Swollen stomach and trouble breathing (in infants). - Painless, bluish lumps under the skin (in infants). - Weakness or paralysis (loss of ability to move a body part). Less common signs and symptoms of neuroblastoma include the following: - Fever. - Shortness of breath. - Feeling tired. - Easy bruising or bleeding. - Petechiae (flat, pinpoint spots under the skin caused by bleeding). - High blood pressure. - Severe watery diarrhea. - Horner syndrome (droopy eyelid, smaller pupil, and less sweating on one side of the face). - Jerky muscle movements. - Uncontrolled eye movements.",Neuroblastoma,0000031_1,CancerGov,https://www.cancer.gov/types/neuroblastoma/patient/neuroblastoma-treatment-pdq,C0027819,T191,Disorders How to diagnose Neuroblastoma ?,0000031_1-4,exams and tests,"Tests that examine many different body tissues and fluids are used to detect (find) and diagnose neuroblastoma. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Neurological exam : A series of questions and tests to check the brain, spinal cord, and nerve function. The exam checks a persons mental status, coordination, and ability to walk normally, and how well the muscles, senses, and reflexes work. This may also be called a neuro exam or a neurologic exam. - Urine catecholamine studies: A procedure in which a urine sample is checked to measure the amount of certain substances, vanillylmandelic acid (VMA) and homovanillic acid (HVA), that are made when catecholamines break down and are released into the urine. A higher than normal amount of VMA or HVA can be a sign of neuroblastoma. - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. - X-ray : An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - MRI (magnetic resonance imaging) with gadolinium : A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. A substance called gadolinium is injected into a vein. The gadolinium collects around the cancer cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI). - mIBG (metaiodobenzylguanidine) scan : A procedure used to find neuroendocrine tumors, such as neuroblastoma. A very small amount of a substance called radioactive mIBG is injected into a vein and travels through the bloodstream. Neuroendocrine tumor cells take up the radioactive mIBG and are detected by a scanner. Scans may be taken over 1-3 days. An iodine solution may be given before or during the test to keep the thyroid gland from absorbing too much of the mIBG. This test is also used to find out how well the tumor is responding to treatment. mIBG is used in high doses to treat neuroblastoma. - Bone marrow aspiration and biopsy : The removal of bone marrow, blood, and a small piece of bone by inserting a hollow needle into the hipbone or breastbone. A pathologist views the bone marrow, blood, and bone under a microscope to look for signs of cancer. - Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later. An ultrasound exam is not done if a CT/MRI has been done.",Neuroblastoma,0000031_1,CancerGov,https://www.cancer.gov/types/neuroblastoma/patient/neuroblastoma-treatment-pdq,C0027819,T191,Disorders What is the outlook for Neuroblastoma ?,0000031_1-5,outlook,"Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - Age of the child at the time of diagnosis. - The child's risk group. - Whether there are certain changes in the genes. - Where in the body the tumor started. - Tumor histology (the shape, function, and structure of the tumor cells). - Whether there is cancer in the lymph nodes on the same side of the body as the primary cancer or whether there is cancer in the lymph nodes on the opposite side of the body. - How the tumor responds to treatment. - How much time passed between diagnosis and when the cancer recurred (for recurrent cancer). Prognosis and treatment options for neuroblastoma are also affected by tumor biology, which includes: - The patterns of the tumor cells. - How different the tumor cells are from normal cells. - How fast the tumor cells are growing. - Whether the tumor shows MYCN amplification. - Whether the tumor has changes in the ALK gene. The tumor biology is said to be favorable or unfavorable, depending on these factors. A favorable tumor biology means there is a better chance of recovery. In some children up to 6 months old, neuroblastoma may disappear without treatment. This is called spontaneous regression. The child is closely watched for signs or symptoms of neuroblastoma. If signs or symptoms occur, treatment may be needed.",Neuroblastoma,0000031_1,CancerGov,https://www.cancer.gov/types/neuroblastoma/patient/neuroblastoma-treatment-pdq,C0027819,T191,Disorders What are the stages of Neuroblastoma ?,0000031_1-6,stages,"Key Points - After neuroblastoma has been diagnosed, tests are done to find out if cancer has spread from where it started to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - The following stages are used for neuroblastoma: - Stage 1 - Stage 2 - Stage 3 - Stage 4 - Treatment of neuroblastoma is based on risk groups. After neuroblastoma has been diagnosed, tests are done to find out if cancer has spread from where it started to other parts of the body. The process used to find out the extent or spread of cancer is called staging. The information gathered from the staging process helps determine the stage of the disease. For neuroblastoma, the stage of disease affects whether the cancer is low risk, intermediate risk, or high risk. It also affects the treatment plan. The results of some tests and procedures used to diagnose neuroblastoma may be used for staging. See the General Information section for a description of these tests and procedures. The following tests and procedures also may be used to determine the stage: - Lymph node biopsy : The removal of all or part of a lymph node. A pathologist views the tissue under a microscope to look for cancer cells. One of the following types of biopsies may be done: - Excisional biopsy : The removal of an entire lymph node. - Incisional biopsy : The removal of part of a lymph node. - Core biopsy : The removal of tissue from a lymph node using a wide needle. - Fine-needle aspiration (FNA) biopsy : The removal of tissue or fluid from a lymph node using a thin needle. - X-ray of the bone: An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. - Bone scan : A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones with cancer and is detected by a scanner. - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if neuroblastoma spreads to the liver, the cancer cells in the liver are actually neuroblastoma cells. The disease is metastatic neuroblastoma, not liver cancer. The following stages are used for neuroblastoma: Stage 1 In stage 1, the tumor is in only one area and all of the tumor that can be seen is completely removed during surgery. Stage 2 Stage 2 is divided into stages 2A and 2B. - Stage 2A: The tumor is in only one area and all of the tumor that can be seen cannot be completely removed during surgery. - Stage 2B: The tumor is in only one area and all of the tumor that can be seen may be completely removed during surgery. Cancer cells are found in the lymph nodes near the tumor. Stage 3 In stage 3, one of the following is true: - the tumor cannot be completely removed during surgery and has spread from one side of the body to the other side and may also have spread to nearby lymph nodes; or - the tumor is in only one area, on one side of the body, but has spread to lymph nodes on the other side of the body; or - the tumor is in the middle of the body and has spread to tissues or lymph nodes on both sides of the body, and the tumor cannot be removed by surgery. Stage 4 Stage 4 is divided into stages 4 and 4S. - In stage 4, the tumor has spread to distant lymph nodes or other parts of the body. - In stage 4S: - the child is younger than 12 months; and - the cancer has spread to the skin, liver, and/or bone marrow; and - the tumor is in only one area and all of the tumor that can be seen may be completely removed during surgery; and/or - cancer cells may be found in the lymph nodes near the tumor. Treatment of neuroblastoma is based on risk groups. For many types of cancer, stages are used to plan treatment. For neuroblastoma, treatment depends on risk groups. The stage of neuroblastoma is one factor used to determine risk group. Other factors are the age of the child, tumor histology, and tumor biology. There are three risk groups: low risk, intermediate risk, and high risk. - Low-risk and intermediate-risk neuroblastoma have a good chance of being cured. - High-risk neuroblastoma may be hard to cure.",Neuroblastoma,0000031_1,CancerGov,https://www.cancer.gov/types/neuroblastoma/patient/neuroblastoma-treatment-pdq,C0027819,T191,Disorders What are the treatments for Neuroblastoma ?,0000031_1-7,treatment,"Key Points - There are different types of treatment for patients with neuroblastoma. - Children with neuroblastoma should have their treatment planned by a team of doctors who are experts in treating childhood cancer, especially neuroblastoma. - Children who are treated for neuroblastoma may have late effects, including an increased risk of second cancers. - Seven types of standard treatment are used: - Observation - Surgery - Radiation therapy - Iodine 131-mIBG therapy - Chemotherapy - High-dose chemotherapy and radiation therapy with stem cell rescue - Targeted therapy - New types of treatment are being tested in clinical trials. - Immunotherapy - Other drug therapy - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with neuroblastoma. Different types of treatment are available for patients with neuroblastoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment. Children with neuroblastoma should have their treatment planned by a team of doctors who are experts in treating childhood cancer, especially neuroblastoma. Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other pediatric health care providers who are experts in treating children with neuroblastoma and who specialize in certain areas of medicine. These may include the following specialists: - Pediatric surgeon. - Pediatric radiation oncologist.. - Endocrinologist. - Neurologist. - Pediatric neuropathologist.. - Neuroradiologist. - Pediatrician. - Pediatric nurse specialist. - Social worker. - Child life professional. - Psychologist. Children who are treated for neuroblastoma may have late effects, including an increased risk of second cancers. Some cancer treatments cause side effects that continue or appear years after cancer treatment has ended. These are called late effects. Late effects of cancer treatment may include: - Physical problems. - Changes in mood, feelings, thinking, learning, or memory. - Second cancers (new types of cancer). Some late effects may be treated or controlled. It is important that parents of children who are treated for neuroblastoma talk with their doctors about the possible late effects caused by some treatments. See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information. Seven types of standard treatment are used: Observation Observation is closely monitoring a patient's condition without giving any treatment until signs or symptoms appear or change. Surgery Surgery is used to treat neuroblastoma unless it has spread to other parts of the body. Depending on where the tumor is, as much of the tumor as is safely possible will be removed. If the tumor cannot be removed, a biopsy may be done instead. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type of cancer being treated and the child's risk group. External radiation therapy is used to treat neuroblastoma. Iodine 131-mIBG therapy Iodine 131-mIBG therapy is a treatment with radioactive iodine. The radioactive iodine is given through an intravenous (IV) line and enters the bloodstream which carries radiation directly to tumor cells. Radioactive iodine collects in neuroblastoma cells and kills them with the radiation that is given off. Iodine 131-mIBG therapy is sometimes used to treat high-risk neuroblastoma that comes back after initial treatment. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type of cancer being treated and the child's risk group. The use of two or more anticancer drugs is called combination chemotherapy. See Drugs Approved for Neuroblastoma for more information. High-dose chemotherapy and radiation therapy with stem cell rescue High-dose chemotherapy and radiation therapy with stem cell rescue is a way of giving high doses of chemotherapy and radiation therapy and replacing blood -forming cells destroyed by cancer treatment for high-risk neuroblastoma. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient and are frozen and stored. After chemotherapy and radiation therapy are completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. Maintenance therapy is given after high-dose chemotherapy and radiation therapy with stem cell rescue to kill any cancer cells that may regrow and cause the disease to come back. Maintenance therapy is given for 6 months and includes the following treatments: - Isotretinoin: A vitamin -like drug that slows the cancer's ability to make more cancer cells and changes how these cells look and act. This drug is taken by mouth. - Dinutuximab: A type of monoclonal antibody therapy that uses an antibody made in the laboratory from a single type of immune system cell. Dinutuximab identifies and attaches to a substance, called GD2, on the surface of neuroblastoma cells. Once dinutuximab attaches to the GD2, a signal is sent to the immune system that a foreign substance has been found and needs to be killed. Then the body's immune system kills the neuroblastoma cell. Dinutuximab is given by infusion. It is a type of targeted therapy. - Granulocyte-macrophage colony-stimulating factor (GM-CSF): A cytokine that helps make more immune system cells, especially granulocytes and macrophages (white blood cells), which can attack and kill cancer cells. - Interleukin-2 (IL-2): A type of immunotherapy that boosts the growth and activity of many immune cells, especially lymphocytes (a type of white blood cell). Lymphocytes can attack and kill cancer cells. See Drugs Approved for Neuroblastoma for more information. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack cancer cells with less harm to normal cells. There are different types of targeted therapy: - Tyrosine kinase inhibitors are small-molecule drugs that go through the cell membrane and work inside cancer cells to block signals that cancer cells need to grow and divide. Crizotinib is used to treat neuroblastoma that has come back after treatment. - Antibody-drug conjugates are made up of a monoclonal antibody attached to a drug. The monoclonal antibody binds to specific proteins or receptors found on certain cells, including cancer cells. The drug enters these cells and kills them without harming other cells. Lorvotuzumab mertansine is an antibody-drug conjugate being studied to treat neuroblastoma that has come back after treatment. New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Immunotherapy Immunotherapy is a treatment that uses the patients immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the bodys natural defenses against cancer. This type of cancer treatment is also called biotherapy or biological therapy. Vaccine therapy uses a substance to stimulate the immune system to destroy a tumor. Vaccine therapy is being studied to treat neuroblastoma that has come back after treatment. Other drug therapy Lenalidomide is a type of angiogenesis inhibitor. It prevents the growth of new blood vessels that are needed by a tumor to grow. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Neuroblastoma Low-Risk Neuroblastoma Treatment of low-risk neuroblastoma may include the following: - Surgery followed by observation. - Chemotherapy with or without surgery, for children with symptoms, children whose tumor has continued to grow and cannot be removed by surgery, or children with unfavorable tumor biology. - Observation alone for infants younger than 6 months who have small adrenal tumors. - Observation alone for infants who do not have signs or symptoms of neuroblastoma. - Radiation therapy to treat tumors that are causing serious problems and do not respond quickly to chemotherapy or surgery. - A clinical trial of treatment based on the tumor's response to treatment and tumor biology. Intermediate-Risk Neuroblastoma Treatment of intermediate-risk neuroblastoma may include the following: - Chemotherapy for children with symptoms or to shrink a tumor that cannot be removed by surgery. Surgery may be done after chemotherapy. - Surgery alone for infants. - Observation alone for certain infants. - Radiation therapy to treat tumors that are causing serious problems and do not respond quickly to chemotherapy or surgery. - Radiation therapy for tumors that do not respond to other treatment. - A clinical trial of treatment based on the tumor's response to treatment and tumor biology. High-Risk Neuroblastoma Treatment of high-risk neuroblastoma may include the following: - A regimen of combination chemotherapy, surgery, stem cell rescue, radiation therapy, and monoclonal antibody therapy (dinutuximab) with interleukin-2 (IL-2), granulocyte-macrophage colony-stimulating factor (GM-CSF), and isotretinoin. Stage 4S Neuroblastoma There is no standard treatment for stage 4S neuroblastoma but treatment options include the following: - Observation with supportive care for certain children who have favorable tumor biology and do not have signs or symptoms. - Chemotherapy, for children who have signs or symptoms of neuroblastoma or unfavorable tumor biology, or for very young infants. - A clinical trial of treatment based on the tumor's response to treatment and tumor biology. Recurrent Neuroblastoma Patients First Treated for Low-Risk Neuroblastoma Treatment for recurrent neuroblastoma that is found only in the area where the cancer first formed may include the following: - Surgery followed by observation or chemotherapy. - Chemotherapy that may be followed by surgery. Treatment for recurrent neuroblastoma that has spread to other parts of the body may include the following: - Observation. - Chemotherapy. - Surgery followed by chemotherapy. - Treatment as for newly diagnosed high-risk neuroblastoma for children older than 1 year. Patients First Treated for Intermediate-Risk Neuroblastoma Treatment for recurrent neuroblastoma that is found only in the area where the cancer first formed may include the following: - Surgery that may be followed by chemotherapy. - Treatment as for newly diagnosed high-risk neuroblastoma for neuroblastoma that has spread to other parts of the body. Recurrent neuroblastoma that has spread to other parts of the body is treated the same way as newly diagnosed high-risk neuroblastoma. Patients First Treated for High-Risk Neuroblastoma Treatment for recurrent neuroblastoma may include the following: - Combination chemotherapy. - Iodine 131-mIBG therapy to relieve symptoms and improve quality of life. It may be given alone or in combination with other therapy, or before stem cell rescue. - A second course of high-dose chemotherapy and stem cell rescue. - Tyrosine kinase inhibitor therapy (crizotinib) for patients with changes in the ALK gene. Because there is no standard treatment for recurrent neuroblastoma in patients first treated for high-risk neuroblastoma, patients may want to consider a clinical trial. For information about clinical trials, please see the NCI website. Patients with Recurrent CNS Neuroblastoma Treatment for neuroblastoma that recurs (comes back) in the central nervous system (CNS; brain and spinal cord) may include the following: - Surgery to remove the tumor in the CNS followed by radiation therapy. - A clinical trial of a new therapy. Treatments Being Studied for Progressive/Recurrent Neuroblastoma Some of the treatments being studied in clinical trials for neuroblastoma that recurs (comes back) or progresses (grows, spreads, or does not respond to treatment) include the following: - Combination chemotherapy and monoclonal antibody therapy (dinutuximab). - Lenalidomide and monoclonal antibody therapy (dinutuximab) with or without isotretinoin. - Iodine 131-mIBG given alone or with other anticancer drugs. - Immunotherapy (vaccine therapy). - Tyrosine kinase inhibitor (crizotinib) and combination chemotherapy. - Targeted therapy with an antibody-drug conjugate (lorvotuzumab mertansine).",Neuroblastoma,0000031_1,CancerGov,https://www.cancer.gov/types/neuroblastoma/patient/neuroblastoma-treatment-pdq,C0027819,T191,Disorders what research (or clinical trials) is being done for Neuroblastoma ?,0000031_1-8,research,"New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Immunotherapy Immunotherapy is a treatment that uses the patients immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the bodys natural defenses against cancer. This type of cancer treatment is also called biotherapy or biological therapy. Vaccine therapy uses a substance to stimulate the immune system to destroy a tumor. Vaccine therapy is being studied to treat neuroblastoma that has come back after treatment. Other drug therapy Lenalidomide is a type of angiogenesis inhibitor. It prevents the growth of new blood vessels that are needed by a tumor to grow. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Neuroblastoma,0000031_1,CancerGov,https://www.cancer.gov/types/neuroblastoma/patient/neuroblastoma-treatment-pdq,C0027819,T191,Disorders What is (are) Neuroblastoma ?,0000031_2-1,information,"Key Points - Neuroblastoma is a disease in which malignant (cancer) cells form in nerve tissue. - Most cases of neuroblastoma are diagnosed before 1 year of age. - The risk factors for neuroblastoma are not known. Neuroblastoma is a disease in which malignant (cancer) cells form in nerve tissue. Neuroblastoma often begins in the nerve tissue of the adrenal glands. There are two adrenal glands, one on top of each kidney, in the back of the upper abdomen. The adrenal glands make important hormones that help control heart rate, blood pressure, blood sugar, and the way the body reacts to stress. Neuroblastoma may also begin in the abdomen, chest, spinal cord, or in nerve tissue near the spine in the neck. Neuroblastoma most often begins during early childhood, usually in children younger than 5 years of age. See the PDQ summary on Neuroblastoma Treatment for more information about neuroblastoma. Most cases of neuroblastoma are diagnosed before 1 year of age. Neuroblastoma is the most common type of cancer in infants. The number of new cases of neuroblastoma is greatest among children under 1 year of age. As children get older, the number of new cases decreases. Neuroblastoma is slightly more common in males than females. Neuroblastoma sometimes forms before birth but is usually found later, when the tumor begins to grow and cause symptoms. In rare cases, neuroblastoma may be found before birth, by fetal ultrasound.",Neuroblastoma,0000031_2,CancerGov,https://www.cancer.gov/types/neuroblastoma/patient/neuroblastoma-screening-pdq,C0027819,T191,Disorders Who is at risk for Neuroblastoma? ?,0000031_2-2,susceptibility,The risk factors for neuroblastoma are not known.,Neuroblastoma,0000031_2,CancerGov,https://www.cancer.gov/types/neuroblastoma/patient/neuroblastoma-screening-pdq,C0027819,T191,Disorders Who is at risk for Neuroblastoma? ?,0000031_2-4,susceptibility,"Key Points - Screening tests have risks. - The risks of neuroblastoma screening include the following: - Neuroblastoma may be overdiagnosed. - False-negative test results can occur. - False-positive test results can occur. Screening tests have risks. Decisions about screening tests can be difficult. Not all screening tests are helpful and most have risks. Before having any screening test, you may want to discuss the test with your doctor. It is important to know the risks of the test and whether it has been proven to reduce the risk of dying from cancer. The risks of neuroblastoma screening include the following: Neuroblastoma may be overdiagnosed. When a screening test result leads to the diagnosis and treatment of a disease that may never have caused symptoms or become life-threatening, it is called overdiagnosis. For example, when a urine test result shows a higher than normal amount of homovanillic acid (HMA) or vanillyl mandelic acid (VMA), tests and treatments for neuroblastoma are likely to be done, but may not be needed. At this time, it is not possible to know which neuroblastomas found by a screening test will cause symptoms and which neuroblastomas will not. Diagnostic tests (such as biopsies) and cancer treatments (such as surgery, radiation therapy, and chemotherapy) can have serious risks, including physical and emotional problems. False-negative test results can occur. Screening test results may appear to be normal even though neuroblastoma is present. A person who receives a false-negative test result (one that shows there is no cancer when there really is) may delay seeking medical care even if there are symptoms. False-positive test results can occur. Screening test results may appear to be abnormal even though no cancer is present. A false-positive test result (one that shows there is cancer when there really isn't) can cause anxiety and is usually followed by more tests and procedures, which also have risks.",Neuroblastoma,0000031_2,CancerGov,https://www.cancer.gov/types/neuroblastoma/patient/neuroblastoma-screening-pdq,C0027819,T191,Disorders What is (are) Non-Small Cell Lung Cancer ?,0000032_1-1,information,"Key Points - Non-small cell lung cancer is a disease in which malignant (cancer) cells form in the tissues of the lung. - There are several types of non-small cell lung cancer. - Smoking is the major risk factor for non-small cell lung cancer. - Signs of non-small cell lung cancer include a cough that doesn't go away and shortness of breath. - Tests that examine the lungs are used to detect (find), diagnose, and stage non-small cell lung cancer. - Certain factors affect prognosis (chance of recovery) and treatment options. - For most patients with non-small cell lung cancer, current treatments do not cure the cancer. Non-small cell lung cancer is a disease in which malignant (cancer) cells form in the tissues of the lung. The lungs are a pair of cone-shaped breathing organs in the chest. The lungs bring oxygen into the body as you breathe in. They release carbon dioxide, a waste product of the bodys cells, as you breathe out. Each lung has sections called lobes. The left lung has two lobes. The right lung is slightly larger and has three lobes. Two tubes called bronchi lead from the trachea (windpipe) to the right and left lungs. The bronchi are sometimes also involved in lung cancer. Tiny air sacs called alveoli and small tubes called bronchioles make up the inside of the lungs. A thin membrane called the pleura covers the outside of each lung and lines the inside wall of the chest cavity. This creates a sac called the pleural cavity. The pleural cavity normally contains a small amount of fluid that helps the lungs move smoothly in the chest when you breathe. There are two main types of lung cancer: non-small cell lung cancer and small cell lung cancer. See the following PDQ summaries for more information about lung cancer: - Small Cell Lung Cancer Treatment - Unusual Cancers of Childhood Treatment - Lung Cancer Prevention - Lung Cancer Screening There are several types of non-small cell lung cancer. Each type of non-small cell lung cancer has different kinds of cancer cells. The cancer cells of each type grow and spread in different ways. The types of non-small cell lung cancer are named for the kinds of cells found in the cancer and how the cells look under a microscope: - Squamous cell carcinoma: Cancer that begins in squamous cells, which are thin, flat cells that look like fish scales. This is also called epidermoid carcinoma. - Large cell carcinoma: Cancer that may begin in several types of large cells. - Adenocarcinoma: Cancer that begins in the cells that line the alveoli and make substances such as mucus. Other less common types of non-small cell lung cancer are: pleomorphic, carcinoid tumor, salivary gland carcinoma, and unclassified carcinoma. For most patients with non-small cell lung cancer, current treatments do not cure the cancer. If lung cancer is found, taking part in one of the many clinical trials being done to improve treatment should be considered. Clinical trials are taking place in most parts of the country for patients with all stages of non-small cell lung cancer. Information about ongoing clinical trials is available from the NCI website.",Non-Small Cell Lung Cancer,0000032_1,CancerGov,https://www.cancer.gov/types/lung/patient/non-small-cell-lung-treatment-pdq,C0007131,T191,Disorders Who is at risk for Non-Small Cell Lung Cancer? ?,0000032_1-2,susceptibility,"Smoking is the major risk factor for non-small cell lung cancer. Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk to your doctor if you think you may be at risk for lung cancer. Risk factors for lung cancer include the following: - Smoking cigarettes, pipes, or cigars, now or in the past. This is the most important risk factor for lung cancer. The earlier in life a person starts smoking, the more often a person smokes, and the more years a person smokes, the greater the risk of lung cancer. - Being exposed to secondhand smoke. - Being exposed to radiation from any of the following: - Radiation therapy to the breast or chest. - Radon in the home or workplace. - Imaging tests such as CT scans. - Atomic bomb radiation. - Being exposed to asbestos, chromium, nickel, beryllium, arsenic, soot, or tar in the workplace. - Living where there is air pollution. - Having a family history of lung cancer. - Being infected with the human immunodeficiency virus (HIV). - Taking beta carotene supplements and being a heavy smoker. Older age is the main risk factor for most cancers. The chance of getting cancer increases as you get older. When smoking is combined with other risk factors, the risk of lung cancer is increased.",Non-Small Cell Lung Cancer,0000032_1,CancerGov,https://www.cancer.gov/types/lung/patient/non-small-cell-lung-treatment-pdq,C0007131,T191,Disorders What are the symptoms of Non-Small Cell Lung Cancer ?,0000032_1-3,symptoms,Signs of non-small cell lung cancer include a cough that doesn't go away and shortness of breath. Sometimes lung cancer does not cause any signs or symptoms. It may be found during a chest x-ray done for another condition. Signs and symptoms may be caused by lung cancer or by other conditions. Check with your doctor if you have any of the following: - Chest discomfort or pain. - A cough that doesnt go away or gets worse over time. - Trouble breathing. - Wheezing. - Blood in sputum (mucus coughed up from the lungs). - Hoarseness. - Loss of appetite. - Weight loss for no known reason. - Feeling very tired. - Trouble swallowing. - Swelling in the face and/or veins in the neck.,Non-Small Cell Lung Cancer,0000032_1,CancerGov,https://www.cancer.gov/types/lung/patient/non-small-cell-lung-treatment-pdq,C0007131,T191,Disorders How to diagnose Non-Small Cell Lung Cancer ?,0000032_1-4,exams and tests,"Tests that examine the lungs are used to detect (find), diagnose, and stage non-small cell lung cancer. Tests and procedures to detect, diagnose, and stage non-small cell lung cancer are often done at the same time. Some of the following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits, including smoking, and past jobs, illnesses, and treatments will also be taken. - Laboratory tests : Medical procedures that test samples of tissue, blood, urine, or other substances in the body. These tests help to diagnose disease, plan and check treatment, or monitor the disease over time. - Chest x-ray: An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the chest, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - Sputum cytology : A procedure in which a pathologist views a sample of sputum (mucus coughed up from the lungs) under a microscope, to check for cancer cells. - Fine-needle aspiration (FNA) biopsy of the lung: The removal of tissue or fluid from the lung using a thin needle. A CT scan, ultrasound, or other imaging procedure is used to locate the abnormal tissue or fluid in the lung. A small incision may be made in the skin where the biopsy needle is inserted into the abnormal tissue or fluid. A sample is removed with the needle and sent to the laboratory. A pathologist then views the sample under a microscope to look for cancer cells. A chest x-ray is done after the procedure to make sure no air is leaking from the lung into the chest. - Bronchoscopy : A procedure to look inside the trachea and large airways in the lung for abnormal areas. A bronchoscope is inserted through the nose or mouth into the trachea and lungs. A bronchoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer. - Thoracoscopy : A surgical procedure to look at the organs inside the chest to check for abnormal areas. An incision (cut) is made between two ribs, and a thoracoscope is inserted into the chest. A thoracoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of cancer. In some cases, this procedure is used to remove part of the esophagus or lung. If certain tissues, organs, or lymph nodes cant be reached, a thoracotomy may be done. In this procedure, a larger incision is made between the ribs and the chest is opened. - Thoracentesis : The removal of fluid from the space between the lining of the chest and the lung, using a needle. A pathologist views the fluid under a microscope to look for cancer cells. - Light and electron microscopy : A laboratory test in which cells in a sample of tissue are viewed under regular and high-powered microscopes to look for certain changes in the cells. - Immunohistochemistry : A test that uses antibodies to check for certain antigens in a sample of tissue. The antibody is usually linked to a radioactive substance or a dye that causes the tissue to light up under a microscope. This type of test may be used to tell the difference between different types of cancer.",Non-Small Cell Lung Cancer,0000032_1,CancerGov,https://www.cancer.gov/types/lung/patient/non-small-cell-lung-treatment-pdq,C0007131,T191,Disorders What is the outlook for Non-Small Cell Lung Cancer ?,0000032_1-5,outlook,"Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - The stage of the cancer (the size of the tumor and whether it is in the lung only or has spread to other places in the body). - The type of lung cancer. - Whether the cancer has mutations (changes) in certain genes, such as the epidermal growth factor receptor (EGFR) gene or the anaplastic lymphoma kinase (ALK) gene. - Whether there are signs and symptoms such as coughing or trouble breathing. - The patients general health.",Non-Small Cell Lung Cancer,0000032_1,CancerGov,https://www.cancer.gov/types/lung/patient/non-small-cell-lung-treatment-pdq,C0007131,T191,Disorders What are the stages of Non-Small Cell Lung Cancer ?,0000032_1-6,stages,"Key Points - After lung cancer has been diagnosed, tests are done to find out if cancer cells have spread within the lungs or to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - The following stages are used for non-small cell lung cancer: - Occult (hidden) stage - Stage 0 (carcinoma in situ) - Stage I - Stage II - Stage IIIA - Stage IIIB - Stage IV After lung cancer has been diagnosed, tests are done to find out if cancer cells have spread within the lungs or to other parts of the body. The process used to find out if cancer has spread within the lungs or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. Some of the tests used to diagnose non-small cell lung cancer are also used to stage the disease. (See the General Information section.) Other tests and procedures that may be used in the staging process include the following: - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body, such as the brain. This procedure is also called nuclear magnetic resonance imaging (NMRI). - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the brain and abdomen, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. - Radionuclide bone scan : A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones and is detected by a scanner. - Pulmonary function test (PFT): A test to see how well the lungs are working. It measures how much air the lungs can hold and how quickly air moves into and out of the lungs. It also measures how much oxygen is used and how much carbon dioxide is given off during breathing. This is also called lung function test. - Endoscopic ultrasound (EUS): A procedure in which an endoscope is inserted into the body. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. A probe at the end of the endoscope is used to bounce high-energy sound waves (ultrasound) off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. This procedure is also called endosonography. EUS may be used to guide fine needle aspiration (FNA) biopsy of the lung, lymph nodes, or other areas. - Mediastinoscopy : A surgical procedure to look at the organs, tissues, and lymph nodes between the lungs for abnormal areas. An incision (cut) is made at the top of the breastbone and a mediastinoscope is inserted into the chest. A mediastinoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of cancer. - Anterior mediastinotomy : A surgical procedure to look at the organs and tissues between the lungs and between the breastbone and heart for abnormal areas. An incision (cut) is made next to the breastbone and a mediastinoscope is inserted into the chest. A mediastinoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of cancer. This is also called the Chamberlain procedure. - Lymph node biopsy : The removal of all or part of a lymph node. A pathologist views the tissue under a microscope to look for cancer cells. - Bone marrow aspiration and biopsy : The removal of bone marrow, blood, and a small piece of bone by inserting a hollow needle into the hipbone or breastbone. A pathologist views the bone marrow, blood, and bone under a microscope to look for signs of cancer. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if non-small cell lung cancer spreads to the brain, the cancer cells in the brain are actually lung cancer cells. The disease is metastatic lung cancer, not brain cancer. The following stages are used for non-small cell lung cancer: Occult (hidden) stage In the occult (hidden) stage, cancer cannot be seen by imaging or bronchoscopy. Cancer cells are found in sputum (mucus coughed up from the lungs) or bronchial washing (a sample of cells taken from inside the airways that lead to the lung). Cancer may have spread to other parts of the body. Stage 0 (carcinoma in situ) In stage 0, abnormal cells are found in the lining of the airways. These abnormal cells may become cancer and spread into nearby normal tissue. Stage 0 is also called carcinoma in situ. Stage I In stage I, cancer has formed. Stage I is divided into stages IA and IB: - Stage IA: The tumor is in the lung only and is 3 centimeters or smaller. - Stage IB: Cancer has not spread to the lymph nodes and one or more of the following is true: - The tumor is larger than 3 centimeters but not larger than 5 centimeters. - Cancer has spread to the main bronchus and is at least 2 centimeters below where the trachea joins the bronchus. - Cancer has spread to the innermost layer of the membrane that covers the lung. - Part of the lung has collapsed or developed pneumonitis (inflammation of the lung) in the area where the trachea joins the bronchus. Stage II Stage II is divided into stages IIA and IIB. Stage IIA and IIB are each divided into two sections depending on the size of the tumor, where the tumor is found, and whether there is cancer in the lymph nodes. - Stage IIA: (1) Cancer has spread to lymph nodes on the same side of the chest as the tumor. The lymph nodes with cancer are within the lung or near the bronchus. Also, one or more of the following is true: - The tumor is not larger than 5 centimeters. - Cancer has spread to the main bronchus and is at least 2 centimeters below where the trachea joins the bronchus. - Cancer has spread to the innermost layer of the membrane that covers the lung. - Part of the lung has collapsed or developed pneumonitis (inflammation of the lung) in the area where the trachea joins the bronchus. or (2) Cancer has not spread to lymph nodes and one or more of the following is true: - The tumor is larger than 5 centimeters but not larger than 7 centimeters. - Cancer has spread to the main bronchus and is at least 2 centimeters below where the trachea joins the bronchus. - Cancer has spread to the innermost layer of the membrane that covers the lung. - Part of the lung has collapsed or developed pneumonitis (inflammation of the lung) in the area where the trachea joins the bronchus. - Stage IIB: (1) Cancer has spread to nearby lymph nodes on the same side of the chest as the tumor. The lymph nodes with cancer are within the lung or near the bronchus. Also, one or more of the following is true: - The tumor is larger than 5 centimeters but not larger than 7 centimeters. - Cancer has spread to the main bronchus and is at least 2 centimeters below where the trachea joins the bronchus. - Cancer has spread to the innermost layer of the membrane that covers the lung. - Part of the lung has collapsed or developed pneumonitis (inflammation of the lung) in the area where the trachea joins the bronchus. or (2) Cancer has not spread to lymph nodes and one or more of the following is true: - The tumor is larger than 7 centimeters. - Cancer has spread to the main bronchus (and is less than 2 centimeters below where the trachea joins the bronchus), the chest wall, the diaphragm, or the nerve that controls the diaphragm. - Cancer has spread to the membrane around the heart or lining the chest wall. - The whole lung has collapsed or developed pneumonitis (inflammation of the lung). - There are one or more separate tumors in the same lobe of the lung. Stage IIIA Stage IIIA is divided into three sections depending on the size of the tumor, where the tumor is found, and which lymph nodes have cancer (if any). (1) Cancer has spread to lymph nodes on the same side of the chest as the tumor. The lymph nodes with cancer are near the sternum (chest bone) or where the bronchus enters the lung. Also: - The tumor may be any size. - Part of the lung (where the trachea joins the bronchus) or the whole lung may have collapsed or developed pneumonitis (inflammation of the lung). - There may be one or more separate tumors in the same lobe of the lung. - Cancer may have spread to any of the following: - Main bronchus, but not the area where the trachea joins the bronchus. - Chest wall. - Diaphragm and the nerve that controls it. - Membrane around the lung or lining the chest wall. - Membrane around the heart. or (2) Cancer has spread to lymph nodes on the same side of the chest as the tumor. The lymph nodes with cancer are within the lung or near the bronchus. Also: - The tumor may be any size. - The whole lung may have collapsed or developed pneumonitis (inflammation of the lung). - There may be one or more separate tumors in any of the lobes of the lung with cancer. - Cancer may have spread to any of the following: - Main bronchus, but not the area where the trachea joins the bronchus. - Chest wall. - Diaphragm and the nerve that controls it. - Membrane around the lung or lining the chest wall. - Heart or the membrane around it. - Major blood vessels that lead to or from the heart. - Trachea. - Esophagus. - Nerve that controls the larynx (voice box). - Sternum (chest bone) or backbone. - Carina (where the trachea joins the bronchi). or (3) Cancer has not spread to the lymph nodes and the tumor may be any size. Cancer has spread to any of the following: - Heart. - Major blood vessels that lead to or from the heart. - Trachea. - Esophagus. - Nerve that controls the larynx (voice box). - Sternum (chest bone) or backbone. - Carina (where the trachea joins the bronchi). Stage IIIB Stage IIIB is divided into two sections depending on the size of the tumor, where the tumor is found, and which lymph nodes have cancer. (1) Cancer has spread to lymph nodes above the collarbone or to lymph nodes on the opposite side of the chest as the tumor. Also: - The tumor may be any size. - Part of the lung (where the trachea joins the bronchus) or the whole lung may have collapsed or developed pneumonitis (inflammation of the lung). - There may be one or more separate tumors in any of the lobes of the lung with cancer. - Cancer may have spread to any of the following: - Main bronchus. - Chest wall. - Diaphragm and the nerve that controls it. - Membrane around the lung or lining the chest wall. - Heart or the membrane around it. - Major blood vessels that lead to or from the heart. - Trachea. - Esophagus. - Nerve that controls the larynx (voice box). - Sternum (chest bone) or backbone. - Carina (where the trachea joins the bronchi). or (2) Cancer has spread to lymph nodes on the same side of the chest as the tumor. The lymph nodes with cancer are near the sternum (chest bone) or where the bronchus enters the lung. Also: - The tumor may be any size. - There may be separate tumors in different lobes of the same lung. - Cancer has spread to any of the following: - Heart. - Major blood vessels that lead to or from the heart. - Trachea. - Esophagus. - Nerve that controls the larynx (voice box). - Sternum (chest bone) or backbone. - Carina (where the trachea joins the bronchi). Stage IV In stage IV, the tumor may be any size and cancer may have spread to lymph nodes. One or more of the following is true: - There are one or more tumors in both lungs. - Cancer is found in fluid around the lungs or the heart. - Cancer has spread to other parts of the body, such as the brain, liver, adrenal glands, kidneys, or bone.",Non-Small Cell Lung Cancer,0000032_1,CancerGov,https://www.cancer.gov/types/lung/patient/non-small-cell-lung-treatment-pdq,C0007131,T191,Disorders What are the treatments for Non-Small Cell Lung Cancer ?,0000032_1-7,treatment,"Key Points - There are different types of treatment for patients with non-small cell lung cancer. - Nine types of standard treatment are used: - Surgery - Radiation therapy - Chemotherapy - Targeted therapy - Laser therapy - Photodynamic therapy (PDT) - Cryosurgery - Electrocautery - Watchful waiting - New types of treatment are being tested in clinical trials. - Chemoprevention - Radiosensitizers - New combinations - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with non-small cell lung cancer. Different types of treatments are available for patients with non-small cell lung cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Nine types of standard treatment are used: Surgery Four types of surgery are used to treat lung cancer: - Wedge resection: Surgery to remove a tumor and some of the normal tissue around it. When a slightly larger amount of tissue is taken, it is called a segmental resection. - Lobectomy: Surgery to remove a whole lobe (section) of the lung. - Pneumonectomy: Surgery to remove one whole lung. - Sleeve resection: Surgery to remove part of the bronchus. Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. Stereotactic body radiation therapy is a type of external radiation therapy. Special equipment is used to place the patient in the same position for each radiation treatment. Once a day for several days, a radiation machine aims a larger than usual dose of radiation directly at the tumor. By having the patient in the same position for each treatment, there is less damage to nearby healthy tissue. This procedure is also called stereotactic external-beam radiation therapy and stereotaxic radiation therapy. Stereotactic radiosurgery is a type of external radiation therapy used to treat lung cancer that has spread to the brain. A rigid head frame is attached to the skull to keep the head still during the radiation treatment. A machine aims a single large dose of radiation directly at the tumor in the brain. This procedure does not involve surgery. It is also called stereotaxic radiosurgery, radiosurgery, and radiation surgery. For tumors in the airways, radiation is given directly to the tumor through an endoscope. The way the radiation therapy is given depends on the type and stage of the cancer being treated. It also depends on where the cancer is found. External and internal radiation therapy are used to treat non-small cell lung cancer. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. See Drugs Approved for Non-Small Cell Lung Cancer for more information. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. Monoclonal antibodies and tyrosine kinase inhibitors are the two main types of targeted therapy being used to treat advanced, metastatic, or recurrent non-small cell lung cancer. Monoclonal antibodies Monoclonal antibody therapy is a cancer treatment that uses antibodies made in the laboratory from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances in the blood or tissues that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. There are different types of monoclonal antibody therapy: - Vascular endothelial growth factor (VEGF) inhibitor therapy: Cancer cells make a substance called VEGF, which causes new blood vessels to form (angiogenesis) and helps the cancer grow. VEGF inhibitors block VEGF and stop new blood vessels from forming. This may kill cancer cells because they need new blood vessels to grow. Bevacizumab and ramucirumab are VEGF inhibitors and angiogenesis inhibitors. - Epidermal growth factor receptor (EGFR) inhibitor therapy: EGFRs are proteins found on the surface of certain cells, including cancer cells. Epidermal growth factor attaches to the EGFR on the surface of the cell and causes the cells to grow and divide. EGFR inhibitors block the receptor and stop the epidermal growth factor from attaching to the cancer cell. This stops the cancer cell from growing and dividing. Cetuximab and necitumumab are EGFR inhibitors. - Immune checkpoint inhibitor therapy: PD-1 is a protein on the surface of T cells that helps keep the bodys immune responses in check. When PD-1 attaches to another protein called PDL-1 on a cancer cell, it stops the T cell from killing the cancer cell. PD-1 inhibitors attach to PDL-1 and allow the T cells to kill cancer cells. Nivolumab, pembrolizumab, and atezolizumab are types of immune checkpoint inhibitors. Tyrosine kinase inhibitors Tyrosine kinase inhibitors are small-molecule drugs that go through the cell membrane and work inside cancer cells to block signals that cancer cells need to grow and divide. Some tyrosine kinase inhibitors also have angiogenesis inhibitor effects. There are different types of tyrosine kinase inhibitors: - Epidermal growth factor receptor (EGFR) tyrosine kinase inhibitors: EGFRs are proteins found on the surface and inside certain cells, including cancer cells. Epidermal growth factor attaches to the EGFR inside the cell and sends signals to the tyrosine kinase area of the cell, which tells the cell to grow and divide. EGFR tyrosine kinase inhibitors stop these signals and stop the cancer cell from growing and dividing. Erlotinib, gefitinib, and afatinib are types of EGFR tyrosine kinase inhibitors. Some of these drugs work better when there is also a mutation (change) in the EGFR gene. - Kinase inhibitors that affect cells with certain gene changes: Certain changes in the ALK and ROS1 genes cause too much protein to be made. Blocking these proteins may stop the cancer from growing and spreading. Crizotinib is used to stop proteins from being made by the ALK and ROS1 gene. Ceritinib is used to stop proteins from being made by the ALK gene. See Drugs Approved for Non-Small Cell Lung Cancer for more information. Laser therapy Laser therapy is a cancer treatment that uses a laser beam (a narrow beam of intense light) to kill cancer cells. Photodynamic therapy (PDT) Photodynamic therapy (PDT) is a cancer treatment that uses a drug and a certain type of laser light to kill cancer cells. A drug that is not active until it is exposed to light is injected into a vein. The drug collects more in cancer cells than in normal cells. Fiberoptic tubes are then used to carry the laser light to the cancer cells, where the drug becomes active and kills the cells. Photodynamic therapy causes little damage to healthy tissue. It is used mainly to treat tumors on or just under the skin or in the lining of internal organs. When the tumor is in the airways, PDT is given directly to the tumor through an endoscope. Cryosurgery Cryosurgery is a treatment that uses an instrument to freeze and destroy abnormal tissue, such as carcinoma in situ. This type of treatment is also called cryotherapy. For tumors in the airways, cryosurgery is done through an endoscope. Electrocautery Electrocautery is a treatment that uses a probe or needle heated by an electric current to destroy abnormal tissue. For tumors in the airways, electrocautery is done through an endoscope. Watchful waiting Watchful waiting is closely monitoring a patients condition without giving any treatment until signs or symptoms appear or change. This may be done in certain rare cases of non-small cell lung cancer. New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Chemoprevention Chemoprevention is the use of drugs, vitamins, or other substances to reduce the risk of cancer or to reduce the risk cancer will recur (come back). For lung cancer, chemoprevention is used to lessen the chance that a new tumor will form in the lung. Radiosensitizers Radiosensitizers are substances that make tumor cells easier to kill with radiation therapy. The combination of chemotherapy and radiation therapy given with a radiosensitizer is being studied in the treatment of non-small cell lung cancer. New combinations New combinations of treatments are being studied in clinical trials. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options by Stage Occult Non-Small Cell Lung Cancer Treatment of occult non-small cell lung cancer depends on the stage of the disease. Occult tumors are often found at an early stage (the tumor is in the lung only) and sometimes can be cured by surgery. Check the list of NCI-supported cancer clinical trials that are now accepting patients with occult non-small cell lung cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage 0 (Carcinoma in Situ) Treatment of stage 0 may include the following: - Surgery (wedge resection or segmental resection). - Photodynamic therapy for tumors in or near the bronchus. - Electrocautery, cryosurgery, or laser surgery for tumors in or near the bronchus. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage 0 non-small cell lung cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage I Non-Small Cell Lung Cancer Treatment of stage I non-small cell lung cancer may include the following: - Surgery (wedge resection, segmental resection, sleeve resection, or lobectomy). - External radiation therapy, including stereotactic body radiation therapy for patients who cannot have surgery or choose not to have surgery. - A clinical trial of chemotherapy or radiation therapy following surgery. - A clinical trial of treatment given through an endoscope, such as photodynamic therapy (PDT). - A clinical trial of surgery followed by chemoprevention. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I non-small cell lung cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage II Non-Small Cell Lung Cancer Treatment of stage II non-small cell lung cancer may include the following: - Surgery (wedge resection, segmental resection, sleeve resection, lobectomy, or pneumonectomy). - Chemotherapy followed by surgery. - Surgery followed by chemotherapy. - External radiation therapy for patients who cannot have surgery. - A clinical trial of radiation therapy following surgery. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage II non-small cell lung cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage IIIA Non-Small Cell Lung Cancer Treatment of stage IIIA non-small cell lung cancer that can be removed with surgery may include the following: - Surgery followed by chemotherapy. - Surgery followed by radiation therapy. - Chemotherapy followed by surgery. - Surgery followed by chemotherapy combined with radiation therapy. - Chemotherapy and radiation therapy followed by surgery. - A clinical trial of new combinations of treatments. Treatment of stage IIIA non-small cell lung cancer that cannot be removed with surgery may include the following: - Chemotherapy and radiation therapy given over the same period of time or one followed by the other. - External radiation therapy alone for patients who cannot be treated with combined therapy, or as palliative treatment to relieve symptoms and improve the quality of life. - Internal radiation therapy or laser surgery, as palliative treatment to relieve symptoms and improve the quality of life. - A clinical trial of new combinations of treatments. For more information about supportive care for signs and symptoms including cough, shortness of breath, and chest pain, see the PDQ summary on Cardiopulmonary Syndromes. Non-small cell lung cancer of the superior sulcus, often called Pancoast tumor, begins in the upper part of the lung and spreads to nearby tissues such as the chest wall, large blood vessels, and spine. Treatment of Pancoast tumors may include the following: - Radiation therapy alone. - Radiation therapy followed by surgery. - Chemotherapy and radiation therapy given as separate treatments over the same period of time. Surgery may also be done after chemotherapy and radiation therapy. - Surgery alone. - A clinical trial of new combinations of treatments. Some stage IIIA non-small cell lung tumors that have grown into the chest wall may be completely removed. Treatment of chest wall tumors may include the following: - Surgery. - Surgery and radiation therapy. - Radiation therapy alone. - Chemotherapy combined with radiation therapy and/or surgery. - A clinical trial of new combinations of treatments. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III non-small cell lung cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage IIIB Non-Small Cell Lung Cancer Treatment of stage IIIB non-small cell lung cancer may include the following: - Chemotherapy followed by external radiation therapy. - Chemotherapy and radiation therapy given as separate treatments over the same period of time. - Chemotherapy followed by surgery. - External radiation therapy alone for patients who cannot be treated with chemotherapy. - External radiation therapy as palliative therapy, to relieve symptoms and improve the quality of life. - Laser therapy and/or internal radiation therapy to relieve symptoms and improve the quality of life. - Clinical trials of new external radiation therapy schedules and new types of treatment. - A clinical trial of chemotherapy and radiation therapy combined with a radiosensitizer. - Clinical trials of targeted therapy combined with chemotherapy and radiation therapy. For more information about supportive care for signs and symptoms such as cough, shortness of breath, and chest pain, see the following PDQ summaries: - Cardiopulmonary Syndromes - Cancer Pain Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III non-small cell lung cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage IV Non-Small Cell Lung Cancer Treatment of stage IV non-small cell lung cancer may include the following: - Chemotherapy. - Chemotherapy followed by more chemotherapy as maintenance therapy to help keep cancer from progressing. - Combination chemotherapy and targeted therapy with a monoclonal antibody, such as bevacizumab, cetuximab, or necitumumab. - Targeted therapy with a monoclonal antibody, such as nivolumab, pembrolizumab, or atezolizumab. - Targeted therapy with a tyrosine kinase inhibitor, such as erlotinib, gefitinib, afatinib, crizotinib, or ceritinib. - External radiation therapy as palliative therapy, to relieve symptoms and improve the quality of life. - Laser therapy and/or internal radiation therapy for tumors that are blocking the airways. - A clinical trial of new drugs and combinations of treatments. For more information about supportive care for signs and symptoms including cough, shortness of breath, and chest pain, see the following PDQ summaries: - Cardiopulmonary Syndromes - Cancer Pain - Last Days of Life Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage IV non-small cell lung cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Non-Small Cell Lung Cancer,0000032_1,CancerGov,https://www.cancer.gov/types/lung/patient/non-small-cell-lung-treatment-pdq,C0007131,T191,Disorders what research (or clinical trials) is being done for Non-Small Cell Lung Cancer ?,0000032_1-8,research,"New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Chemoprevention Chemoprevention is the use of drugs, vitamins, or other substances to reduce the risk of cancer or to reduce the risk cancer will recur (come back). For lung cancer, chemoprevention is used to lessen the chance that a new tumor will form in the lung. Radiosensitizers Radiosensitizers are substances that make tumor cells easier to kill with radiation therapy. The combination of chemotherapy and radiation therapy given with a radiosensitizer is being studied in the treatment of non-small cell lung cancer. New combinations New combinations of treatments are being studied in clinical trials. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Non-Small Cell Lung Cancer,0000032_1,CancerGov,https://www.cancer.gov/types/lung/patient/non-small-cell-lung-treatment-pdq,C0007131,T191,Disorders What is (are) Small Cell Lung Cancer ?,0000032_2-1,information,"Key Points - Small cell lung cancer is a disease in which malignant (cancer) cells form in the tissues of the lung. - There are two main types of small cell lung cancer. - Smoking is the major risk factor for small cell lung cancer. - Signs and symptoms of small cell lung cancer include coughing, shortness of breath, and chest pain. - Tests and procedures that examine the lungs are used to detect (find), diagnose, and stage small cell lung cancer. - Certain factors affect prognosis (chance of recovery) and treatment options. - For most patients with small cell lung cancer, current treatments do not cure the cancer. Small cell lung cancer is a disease in which malignant (cancer) cells form in the tissues of the lung. The lungs are a pair of cone-shaped breathing organs that are found in the chest. The lungs bring oxygen into the body when you breathe in and take out carbon dioxide when you breathe out. Each lung has sections called lobes. The left lung has two lobes. The right lung, which is slightly larger, has three. A thin membrane called the pleura surrounds the lungs. Two tubes called bronchi lead from the trachea (windpipe) to the right and left lungs. The bronchi are sometimes also affected by lung cancer. Small tubes called bronchioles and tiny air sacs called alveoli make up the inside of the lungs. There are two types of lung cancer: small cell lung cancer and non-small cell lung cancer. This summary is about small cell lung cancer and its treatment. See the following PDQ summaries for more information about lung cancer: - Non-Small Cell Lung Cancer Treatment - Unusual Cancers of Childhood Treatment - Lung Cancer Prevention - Lung Cancer Screening There are two main types of small cell lung cancer. These two types include many different types of cells. The cancer cells of each type grow and spread in different ways. The types of small cell lung cancer are named for the kinds of cells found in the cancer and how the cells look when viewed under a microscope: - Small cell carcinoma (oat cell cancer). - Combined small cell carcinoma. For most patients with small cell lung cancer, current treatments do not cure the cancer. If lung cancer is found, patients should think about taking part in one of the many clinical trials being done to improve treatment. Clinical trials are taking place in most parts of the country for patients with all stages of small cell lung cancer. Information about ongoing clinical trials is available from the NCI website.",Small Cell Lung Cancer,0000032_2,CancerGov,https://www.cancer.gov/types/lung/patient/small-cell-lung-treatment-pdq,C0149925,T191,Disorders Who is at risk for Small Cell Lung Cancer? ?,0000032_2-2,susceptibility,"Smoking is the major risk factor for small cell lung cancer. Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk to your doctor if you think you may be at risk for lung cancer. Risk factors for lung cancer include the following: - Smoking cigarettes, pipes, or cigars, now or in the past. This is the most important risk factor for lung cancer. The earlier in life a person starts smoking, the more often a person smokes, and the more years a person smokes, the greater the risk of lung cancer. - Being exposed to secondhand smoke. - Being exposed to radiation from any of the following: - Radiation therapy to the breast or chest. - Radon in the home or workplace. - Imaging tests such as CT scans. - Atomic bomb radiation. - Being exposed to asbestos, chromium, nickel, beryllium, arsenic, soot, or tar in the workplace. - Living where there is air pollution. - Having a family history of lung cancer. - Being infected with the human immunodeficiency virus (HIV). - Taking beta carotene supplements and being a heavy smoker. Older age is the main risk factor for most cancers. The chance of getting cancer increases as you get older. When smoking is combined with other risk factors, the risk of lung cancer is increased.",Small Cell Lung Cancer,0000032_2,CancerGov,https://www.cancer.gov/types/lung/patient/small-cell-lung-treatment-pdq,C0149925,T191,Disorders What are the symptoms of Small Cell Lung Cancer ?,0000032_2-3,symptoms,"Signs and symptoms of small cell lung cancer include coughing, shortness of breath, and chest pain. These and other signs and symptoms may be caused by small cell lung cancer or by other conditions. Check with your doctor if you have any of the following: - Chest discomfort or pain. - A cough that doesnt go away or gets worse over time. - Trouble breathing. - Wheezing. - Blood in sputum (mucus coughed up from the lungs). - Hoarseness. - Trouble swallowing. - Loss of appetite. - Weight loss for no known reason. - Feeling very tired. - Swelling in the face and/or veins in the neck.",Small Cell Lung Cancer,0000032_2,CancerGov,https://www.cancer.gov/types/lung/patient/small-cell-lung-treatment-pdq,C0149925,T191,Disorders How to diagnose Small Cell Lung Cancer ?,0000032_2-4,exams and tests,"Tests and procedures that examine the lungs are used to detect (find), diagnose, and stage small cell lung cancer. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits, including smoking, and past jobs, illnesses, and treatments will also be taken. - Laboratory tests : Medical procedures that test samples of tissue, blood, urine, or other substances in the body. These tests help to diagnose disease, plan and check treatment, or monitor the disease over time. - Chest x-ray : An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. - CT scan (CAT scan) of the brain, chest, and abdomen : A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - Sputum cytology : A microscope is used to check for cancer cells in the sputum (mucus coughed up from the lungs). - Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. The different ways a biopsy can be done include the following: - Fine-needle aspiration (FNA) biopsy of the lung: The removal of tissue or fluid from the lung, using a thin needle. A CT scan, ultrasound, or other imaging procedure is used to find the abnormal tissue or fluid in the lung. A small incision may be made in the skin where the biopsy needle is inserted into the abnormal tissue or fluid. A sample is removed with the needle and sent to the laboratory. A pathologist then views the sample under a microscope to look for cancer cells. A chest x-ray is done after the procedure to make sure no air is leaking from the lung into the chest. - Bronchoscopy : A procedure to look inside the trachea and large airways in the lung for abnormal areas. A bronchoscope is inserted through the nose or mouth into the trachea and lungs. A bronchoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer. - Thoracoscopy : A surgical procedure to look at the organs inside the chest to check for abnormal areas. An incision (cut) is made between two ribs, and a thoracoscope is inserted into the chest. A thoracoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of cancer. In some cases, this procedure is used to remove part of the esophagus or lung. If certain tissues, organs, or lymph nodes cant be reached, a thoracotomy may be done. In this procedure, a larger incision is made between the ribs and the chest is opened. - Thoracentesis : The removal of fluid from the space between the lining of the chest and the lung, using a needle. A pathologist views the fluid under a microscope to look for cancer cells. - Mediastinoscopy : A surgical procedure to look at the organs, tissues, and lymph nodes between the lungs for abnormal areas. An incision (cut) is made at the top of the breastbone and a mediastinoscope is inserted into the chest. A mediastinoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of cancer. - Light and electron microscopy : A laboratory test in which cells in a sample of tissue are viewed under regular and high-powered microscopes to look for certain changes in the cells. - Immunohistochemistry : A test that uses antibodies to check for certain antigens in a sample of tissue. The antibody is usually linked to a radioactive substance or a dye that causes the tissue to light up under a microscope. This type of test may be used to tell the difference between different types of cancer.",Small Cell Lung Cancer,0000032_2,CancerGov,https://www.cancer.gov/types/lung/patient/small-cell-lung-treatment-pdq,C0149925,T191,Disorders What are the stages of Small Cell Lung Cancer ?,0000032_2-6,stages,"Key Points - After small cell lung cancer has been diagnosed, tests are done to find out if cancer cells have spread within the chest or to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - The following stages are used for small cell lung cancer: - Limited-Stage Small Cell Lung Cancer - Extensive-Stage Small Cell Lung Cancer After small cell lung cancer has been diagnosed, tests are done to find out if cancer cells have spread within the chest or to other parts of the body. The process used to find out if cancer has spread within the chest or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. Some of the tests used to diagnose small cell lung cancer are also used to stage the disease. (See the General Information section.) Other tests and procedures that may be used in the staging process include the following: - MRI (magnetic resonance imaging) of the brain: A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the brain, chest or upper abdomen, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. A PET scan and CT scan may be done at the same time. This is called a PET-CT. - Bone scan : A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones and is detected by a scanner. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if small cell lung cancer spreads to the brain, the cancer cells in the brain are actually lung cancer cells. The disease is metastatic small cell lung cancer, not brain cancer. The following stages are used for small cell lung cancer: Limited-Stage Small Cell Lung Cancer In limited-stage, cancer is in the lung where it started and may have spread to the area between the lungs or to the lymph nodes above the collarbone. Extensive-Stage Small Cell Lung Cancer In extensive-stage, cancer has spread beyond the lung or the area between the lungs or the lymph nodes above the collarbone to other places in the body.",Small Cell Lung Cancer,0000032_2,CancerGov,https://www.cancer.gov/types/lung/patient/small-cell-lung-treatment-pdq,C0149925,T191,Disorders What are the treatments for Small Cell Lung Cancer ?,0000032_2-7,treatment,"Key Points - There are different types of treatment for patients with small cell lung cancer. - Five types of standard treatment are used: - Surgery - Chemotherapy - Radiation therapy - Laser therapy - Endoscopic stent placement - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with small cell lung cancer. Different types of treatment are available for patients with small cell lung cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Five types of standard treatment are used: Surgery Surgery may be used if the cancer is found in one lung and in nearby lymph nodes only. Because this type of lung cancer is usually found in both lungs, surgery alone is not often used. During surgery, the doctor will also remove lymph nodes to find out if they have cancer in them. Sometimes, surgery may be used to remove a sample of lung tissue to find out the exact type of lung cancer. Even if the doctor removes all the cancer that can be seen at the time of the operation, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. See Drugs Approved for Small Cell Lung Cancer for more information. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External radiation therapy is used to treat small cell lung cancer, and may also be used as palliative therapy to relieve symptoms and improve quality of life. Radiation therapy to the brain to lessen the risk that cancer will spread to the brain may also be given. Laser therapy Laser therapy is a cancer treatment that uses a laser beam (a narrow beam of intense light) to kill cancer cells. Endoscopic stent placement An endoscope is a thin, tube-like instrument used to look at tissues inside the body. An endoscope has a light and a lens for viewing and may be used to place a stent in a body structure to keep the structure open. An endoscopic stent can be used to open an airway blocked by abnormal tissue. New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options by Stage Limited-Stage Small Cell Lung Cancer Treatment of limited-stage small cell lung cancer may include the following: - Combination chemotherapy and radiation therapy to the chest. Radiation therapy to the brain may later be given to patients with complete responses. - Combination chemotherapy alone for patients who cannot be given radiation therapy. - Surgery followed by chemotherapy. - Surgery followed by chemotherapy and radiation therapy. - Radiation therapy to the brain may be given to patients who have had a complete response, to prevent the spread of cancer to the brain. - Clinical trials of new chemotherapy, surgery, and radiation treatments. Check the list of NCI-supported cancer clinical trials that are now accepting patients with limited stage small cell lung cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Extensive-Stage Small Cell Lung Cancer Treatment of extensive-stage small cell lung cancer may include the following: - Combination chemotherapy. - Radiation therapy to the brain, spine, bone, or other parts of the body where the cancer has spread, as palliative therapy to relieve symptoms and improve quality of life. - Radiation therapy to the chest may be given to patients who respond to chemotherapy. - Radiation therapy to the brain may be given to patients who have had a complete response, to prevent the spread of cancer to the brain. - Clinical trials of new chemotherapy treatments. Check the list of NCI-supported cancer clinical trials that are now accepting patients with extensive stage small cell lung cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Small Cell Lung Cancer,0000032_2,CancerGov,https://www.cancer.gov/types/lung/patient/small-cell-lung-treatment-pdq,C0149925,T191,Disorders what research (or clinical trials) is being done for Small Cell Lung Cancer ?,0000032_2-8,research,"New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Small Cell Lung Cancer,0000032_2,CancerGov,https://www.cancer.gov/types/lung/patient/small-cell-lung-treatment-pdq,C0149925,T191,Disorders What is (are) Lung Cancer ?,0000032_3-1,information,"Key Points - Lung cancer is a disease in which malignant (cancer) cells form in the tissues of the lung. - Lung cancer is the leading cause of cancer death in both men and women. Lung cancer is a disease in which malignant (cancer) cells form in the tissues of the lung. The lungs are a pair of cone-shaped breathing organs in the chest. The lungs bring oxygen into the body as you breathe in. They release carbon dioxide, a waste product of the body's cells, as you breathe out. Each lung has sections called lobes. The left lung has two lobes. The right lung is slightly larger, and has three lobes. A thin membrane called the pleura surrounds the lungs. Two tubes called bronchi lead from the trachea (windpipe) to the right and left lungs. The bronchi are sometimes also involved in lung cancer. Tiny air sacs called alveoli and small tubes called bronchioles make up the inside of the lungs. There are two types of lung cancer: small cell lung cancer and non-small cell lung cancer. See the following PDQ summaries for more information about lung cancer: - Lung Cancer Screening - Non-Small Cell Lung Cancer Treatment - Small Cell Lung Cancer Treatment Lung cancer is the leading cause of cancer death in both men and women. More people die from lung cancer than from any other type of cancer. Lung cancer is the second most common cancer in the United States, after skin cancer. The number of new cases and deaths from lung cancer is highest in black men.",Lung Cancer,0000032_3,CancerGov,https://www.cancer.gov/types/lung/patient/lung-prevention-pdq,C0242379,T191,Disorders How to prevent Lung Cancer ?,0000032_3-2,prevention,"Key Points - Avoiding risk factors and increasing protective factors may help prevent lung cancer. - The following are risk factors for lung cancer: - Cigarette, cigar, and pipe smoking - Secondhand smoke - Family history - HIV infection - Environmental risk factors - Beta carotene supplements in heavy smokers - The following are protective factors for lung cancer: - Not smoking - Quitting smoking - Lower exposure to workplace risk factors - Lower exposure to radon - It is not clear if the following decrease the risk of lung cancer: - Diet - Physical activity - The following do not decrease the risk of lung cancer: - Beta carotene supplements in nonsmokers - Vitamin E supplements - Cancer prevention clinical trials are used to study ways to prevent cancer. - New ways to prevent lung cancer are being studied in clinical trials. Avoiding risk factors and increasing protective factors may help prevent lung cancer. Avoiding cancer risk factors may help prevent certain cancers. Risk factors include smoking, being overweight, and not getting enough exercise. Increasing protective factors such as quitting smoking and exercising may also help prevent some cancers. Talk to your doctor or other health care professional about how you might lower your risk of cancer. The following are risk factors for lung cancer: Cigarette, cigar, and pipe smoking Tobacco smoking is the most important risk factor for lung cancer. Cigarette, cigar, and pipe smoking all increase the risk of lung cancer. Tobacco smoking causes about 9 out of 10 cases of lung cancer in men and about 8 out of 10 cases of lung cancer in women. Studies have shown that smoking low tar or low nicotine cigarettes does not lower the risk of lung cancer. Studies also show that the risk of lung cancer from smoking cigarettes increases with the number of cigarettes smoked per day and the number of years smoked. People who smoke have about 20 times the risk of lung cancer compared to those who do not smoke. Secondhand smoke Being exposed to secondhand tobacco smoke is also a risk factor for lung cancer. Secondhand smoke is the smoke that comes from a burning cigarette or other tobacco product, or that is exhaled by smokers. People who inhale secondhand smoke are exposed to the same cancer -causing agents as smokers, although in smaller amounts. Inhaling secondhand smoke is called involuntary or passive smoking. Family history Having a family history of lung cancer is a risk factor for lung cancer. People with a relative who has had lung cancer may be twice as likely to have lung cancer as people who do not have a relative who has had lung cancer. Because cigarette smoking tends to run in families and family members are exposed to secondhand smoke, it is hard to know whether the increased risk of lung cancer is from the family history of lung cancer or from being exposed to cigarette smoke. HIV infection Being infected with the human immunodeficiency virus (HIV), the cause of acquired immunodeficiency syndrome (AIDS), is linked with a higher risk of lung cancer. People infected with HIV may have more than twice the risk of lung cancer than those who are not infected. Since smoking rates are higher in those infected with HIV than in those not infected, it is not clear whether the increased risk of lung cancer is from HIV infection or from being exposed to cigarette smoke. Environmental risk factors - Radiation exposure: Being exposed to radiation is a risk factor for lung cancer. Atomic bomb radiation, radiation therapy, imaging tests, and radon are sources of radiation exposure: - Atomic bomb radiation: Being exposed to radiation after an atomic bomb explosion increases the risk of lung cancer. - Radiation therapy: Radiation therapy to the chest may be used to treat certain cancers, including breast cancer and Hodgkin lymphoma. Radiation therapy uses x-rays, gamma rays, or other types of radiation that may increase the risk of lung cancer. The higher the dose of radiation received, the higher the risk. The risk of lung cancer following radiation therapy is higher in patients who smoke than in nonsmokers. - Imaging tests: Imaging tests, such as CT scans, expose patients to radiation. Low-dose spiral CT scans expose patients to less radiation than higher dose CT scans. In lung cancer screening, the use of low-dose spiral CT scans can lessen the harmful effects of radiation. - Radon: Radon is a radioactive gas that comes from the breakdown of uranium in rocks and soil. It seeps up through the ground, and leaks into the air or water supply. Radon can enter homes through cracks in floors, walls, or the foundation, and levels of radon can build up over time. Studies show that high levels of radon gas inside the home or workplace increase the number of new cases of lung cancer and the number of deaths caused by lung cancer. The risk of lung cancer is higher in smokers exposed to radon than in nonsmokers who are exposed to it. In people who have never smoked, about 30% of deaths caused by lung cancer have been linked to being exposed to radon. - Workplace exposure: Studies show that being exposed to the following substances increases the risk of lung cancer: - Asbestos. - Arsenic. - Chromium. - Nickel. - Beryllium. - Cadmium. - Tar and soot. These substances can cause lung cancer in people who are exposed to them in the workplace and have never smoked. As the level of exposure to these substances increases, the risk of lung cancer also increases. The risk of lung cancer is even higher in people who are exposed and also smoke. - Air pollution: Studies show that living in areas with higher levels of air pollution increases the risk of lung cancer. Beta carotene supplements in heavy smokers Taking beta carotene supplements (pills) increases the risk of lung cancer, especially in smokers who smoke one or more packs a day. The risk is higher in smokers who have at least one alcoholic drink every day. The following are protective factors for lung cancer: Not smoking The best way to prevent lung cancer is to not smoke. Quitting smoking Smokers can decrease their risk of lung cancer by quitting. In smokers who have been treated for lung cancer, quitting smoking lowers the risk of new lung cancers. Counseling, the use of nicotine replacement products, and antidepressant therapy have helped smokers quit for good. In a person who has quit smoking, the chance of preventing lung cancer depends on how many years and how much the person smoked and the length of time since quitting. After a person has quit smoking for 10 years, the risk of lung cancer decreases 30% to 50%. See the following for more information on quitting smoking: - Tobacco (includes help with quitting) - Cigarette Smoking: Health Risks and How to Quit Lower exposure to workplace risk factors Laws that protect workers from being exposed to cancer-causing substances, such as asbestos, arsenic, nickel, and chromium, may help lower their risk of developing lung cancer. Laws that prevent smoking in the workplace help lower the risk of lung cancer caused by secondhand smoke. Lower exposure to radon Lowering radon levels may lower the risk of lung cancer, especially among cigarette smokers. High levels of radon in homes may be reduced by taking steps to prevent radon leakage, such as sealing basements. It is not clear if the following decrease the risk of lung cancer: Diet Some studies show that people who eat high amounts of fruits or vegetables have a lower risk of lung cancer than those who eat low amounts. However, since smokers tend to have less healthy diets than nonsmokers, it is hard to know whether the decreased risk is from having a healthy diet or from not smoking. Physical activity Some studies show that people who are physically active have a lower risk of lung cancer than people who are not. However, since smokers tend to have different levels of physical activity than nonsmokers, it is hard to know if physical activity affects the risk of lung cancer. The following do not decrease the risk of lung cancer: Beta carotene supplements in nonsmokers Studies of nonsmokers show that taking beta carotene supplements does not lower their risk of lung cancer. Vitamin E supplements Studies show that taking vitamin E supplements does not affect the risk of lung cancer. Cancer prevention clinical trials are used to study ways to prevent cancer. Cancer prevention clinical trials are used to study ways to lower the risk of developing certain types of cancer. Some cancer prevention trials are conducted with healthy people who have not had cancer but who have an increased risk for cancer. Other prevention trials are conducted with people who have had cancer and are trying to prevent another cancer of the same type or to lower their chance of developing a new type of cancer. Other trials are done with healthy volunteers who are not known to have any risk factors for cancer. The purpose of some cancer prevention clinical trials is to find out whether actions people take can prevent cancer. These may include eating fruits and vegetables, exercising, quitting smoking, or taking certain medicines, vitamins, minerals, or food supplements. New ways to prevent lung cancer are being studied in clinical trials. Clinical trials are taking place in many parts of the country. Information about clinical trials can be found in the Clinical Trials section of the NCI website. Check the list of NCI-supported cancer clinical trials for prevention trials for non-small cell lung cancer and small cell lung cancer that are now accepting patients. These include trials for quitting smoking.",Lung Cancer,0000032_3,CancerGov,https://www.cancer.gov/types/lung/patient/lung-prevention-pdq,C0242379,T191,Disorders Who is at risk for Lung Cancer? ?,0000032_3-3,susceptibility,"Key Points - Avoiding risk factors and increasing protective factors may help prevent lung cancer. - The following are risk factors for lung cancer: - Cigarette, cigar, and pipe smoking - Secondhand smoke - Family history - HIV infection - Environmental risk factors - Beta carotene supplements in heavy smokers - The following are protective factors for lung cancer: - Not smoking - Quitting smoking - Lower exposure to workplace risk factors - Lower exposure to radon - It is not clear if the following decrease the risk of lung cancer: - Diet - Physical activity - The following do not decrease the risk of lung cancer: - Beta carotene supplements in nonsmokers - Vitamin E supplements - Cancer prevention clinical trials are used to study ways to prevent cancer. - New ways to prevent lung cancer are being studied in clinical trials. Avoiding risk factors and increasing protective factors may help prevent lung cancer. Avoiding cancer risk factors may help prevent certain cancers. Risk factors include smoking, being overweight, and not getting enough exercise. Increasing protective factors such as quitting smoking and exercising may also help prevent some cancers. Talk to your doctor or other health care professional about how you might lower your risk of cancer. The following are risk factors for lung cancer: Cigarette, cigar, and pipe smoking Tobacco smoking is the most important risk factor for lung cancer. Cigarette, cigar, and pipe smoking all increase the risk of lung cancer. Tobacco smoking causes about 9 out of 10 cases of lung cancer in men and about 8 out of 10 cases of lung cancer in women. Studies have shown that smoking low tar or low nicotine cigarettes does not lower the risk of lung cancer. Studies also show that the risk of lung cancer from smoking cigarettes increases with the number of cigarettes smoked per day and the number of years smoked. People who smoke have about 20 times the risk of lung cancer compared to those who do not smoke. Secondhand smoke Being exposed to secondhand tobacco smoke is also a risk factor for lung cancer. Secondhand smoke is the smoke that comes from a burning cigarette or other tobacco product, or that is exhaled by smokers. People who inhale secondhand smoke are exposed to the same cancer -causing agents as smokers, although in smaller amounts. Inhaling secondhand smoke is called involuntary or passive smoking. Family history Having a family history of lung cancer is a risk factor for lung cancer. People with a relative who has had lung cancer may be twice as likely to have lung cancer as people who do not have a relative who has had lung cancer. Because cigarette smoking tends to run in families and family members are exposed to secondhand smoke, it is hard to know whether the increased risk of lung cancer is from the family history of lung cancer or from being exposed to cigarette smoke. HIV infection Being infected with the human immunodeficiency virus (HIV), the cause of acquired immunodeficiency syndrome (AIDS), is linked with a higher risk of lung cancer. People infected with HIV may have more than twice the risk of lung cancer than those who are not infected. Since smoking rates are higher in those infected with HIV than in those not infected, it is not clear whether the increased risk of lung cancer is from HIV infection or from being exposed to cigarette smoke. Environmental risk factors - Radiation exposure: Being exposed to radiation is a risk factor for lung cancer. Atomic bomb radiation, radiation therapy, imaging tests, and radon are sources of radiation exposure: - Atomic bomb radiation: Being exposed to radiation after an atomic bomb explosion increases the risk of lung cancer. - Radiation therapy: Radiation therapy to the chest may be used to treat certain cancers, including breast cancer and Hodgkin lymphoma. Radiation therapy uses x-rays, gamma rays, or other types of radiation that may increase the risk of lung cancer. The higher the dose of radiation received, the higher the risk. The risk of lung cancer following radiation therapy is higher in patients who smoke than in nonsmokers. - Imaging tests: Imaging tests, such as CT scans, expose patients to radiation. Low-dose spiral CT scans expose patients to less radiation than higher dose CT scans. In lung cancer screening, the use of low-dose spiral CT scans can lessen the harmful effects of radiation. - Radon: Radon is a radioactive gas that comes from the breakdown of uranium in rocks and soil. It seeps up through the ground, and leaks into the air or water supply. Radon can enter homes through cracks in floors, walls, or the foundation, and levels of radon can build up over time. Studies show that high levels of radon gas inside the home or workplace increase the number of new cases of lung cancer and the number of deaths caused by lung cancer. The risk of lung cancer is higher in smokers exposed to radon than in nonsmokers who are exposed to it. In people who have never smoked, about 30% of deaths caused by lung cancer have been linked to being exposed to radon. - Workplace exposure: Studies show that being exposed to the following substances increases the risk of lung cancer: - Asbestos. - Arsenic. - Chromium. - Nickel. - Beryllium. - Cadmium. - Tar and soot. These substances can cause lung cancer in people who are exposed to them in the workplace and have never smoked. As the level of exposure to these substances increases, the risk of lung cancer also increases. The risk of lung cancer is even higher in people who are exposed and also smoke. - Air pollution: Studies show that living in areas with higher levels of air pollution increases the risk of lung cancer. Beta carotene supplements in heavy smokers Taking beta carotene supplements (pills) increases the risk of lung cancer, especially in smokers who smoke one or more packs a day. The risk is higher in smokers who have at least one alcoholic drink every day. The following are protective factors for lung cancer: Not smoking The best way to prevent lung cancer is to not smoke. Quitting smoking Smokers can decrease their risk of lung cancer by quitting. In smokers who have been treated for lung cancer, quitting smoking lowers the risk of new lung cancers. Counseling, the use of nicotine replacement products, and antidepressant therapy have helped smokers quit for good. In a person who has quit smoking, the chance of preventing lung cancer depends on how many years and how much the person smoked and the length of time since quitting. After a person has quit smoking for 10 years, the risk of lung cancer decreases 30% to 50%. See the following for more information on quitting smoking: - Tobacco (includes help with quitting) - Cigarette Smoking: Health Risks and How to Quit Lower exposure to workplace risk factors Laws that protect workers from being exposed to cancer-causing substances, such as asbestos, arsenic, nickel, and chromium, may help lower their risk of developing lung cancer. Laws that prevent smoking in the workplace help lower the risk of lung cancer caused by secondhand smoke. Lower exposure to radon Lowering radon levels may lower the risk of lung cancer, especially among cigarette smokers. High levels of radon in homes may be reduced by taking steps to prevent radon leakage, such as sealing basements. It is not clear if the following decrease the risk of lung cancer: Diet Some studies show that people who eat high amounts of fruits or vegetables have a lower risk of lung cancer than those who eat low amounts. However, since smokers tend to have less healthy diets than nonsmokers, it is hard to know whether the decreased risk is from having a healthy diet or from not smoking. Physical activity Some studies show that people who are physically active have a lower risk of lung cancer than people who are not. However, since smokers tend to have different levels of physical activity than nonsmokers, it is hard to know if physical activity affects the risk of lung cancer. The following do not decrease the risk of lung cancer: Beta carotene supplements in nonsmokers Studies of nonsmokers show that taking beta carotene supplements does not lower their risk of lung cancer. Vitamin E supplements Studies show that taking vitamin E supplements does not affect the risk of lung cancer. Cancer prevention clinical trials are used to study ways to prevent cancer. Cancer prevention clinical trials are used to study ways to lower the risk of developing certain types of cancer. Some cancer prevention trials are conducted with healthy people who have not had cancer but who have an increased risk for cancer. Other prevention trials are conducted with people who have had cancer and are trying to prevent another cancer of the same type or to lower their chance of developing a new type of cancer. Other trials are done with healthy volunteers who are not known to have any risk factors for cancer. The purpose of some cancer prevention clinical trials is to find out whether actions people take can prevent cancer. These may include eating fruits and vegetables, exercising, quitting smoking, or taking certain medicines, vitamins, minerals, or food supplements. New ways to prevent lung cancer are being studied in clinical trials. Clinical trials are taking place in many parts of the country. Information about clinical trials can be found in the Clinical Trials section of the NCI website. Check the list of NCI-supported cancer clinical trials for prevention trials for non-small cell lung cancer and small cell lung cancer that are now accepting patients. These include trials for quitting smoking.",Lung Cancer,0000032_3,CancerGov,https://www.cancer.gov/types/lung/patient/lung-prevention-pdq,C0242379,T191,Disorders What is (are) Lung Cancer ?,0000032_4-1,information,"Key Points - Lung cancer is a disease in which malignant (cancer) cells form in the tissues of the lung. - Lung cancer is the leading cause of cancer death in the United States. - Different factors increase or decrease the risk of lung cancer. Lung cancer is a disease in which malignant (cancer) cells form in the tissues of the lung. The lungs are a pair of cone-shaped breathing organs inside the chest. The lungs bring oxygen into the body when breathing in and send carbon dioxide out of the body when breathing out. Each lung has sections called lobes. The left lung has two lobes. The right lung, which is slightly larger, has three. A thin membrane called the pleura surrounds the lungs. Two tubes called bronchi lead from the trachea (windpipe) to the right and left lungs. The bronchi are sometimes involved in lung cancer. Small tubes called bronchioles and tiny air sacs called alveoli make up the inside of the lungs. There are two types of lung cancer: small cell lung cancer and non-small cell lung cancer. See the following PDQ summaries for more information about lung cancer: - Lung Cancer Prevention - Non-Small Cell Lung Cancer Treatment - Small Cell Lung Cancer Treatment Lung cancer is the leading cause of cancer death in the United States. Lung cancer is the third most common type of non-skin cancer in the United States. Lung cancer is the leading cause of cancer death in men and in women.",Lung Cancer,0000032_4,CancerGov,https://www.cancer.gov/types/lung/patient/lung-screening-pdq,C0242379,T191,Disorders Who is at risk for Lung Cancer? ?,0000032_4-2,susceptibility,"Different factors increase or decrease the risk of lung cancer. Anything that increases your chance of getting a disease is called a risk factor. Anything that decreases your chance of getting a disease is called a protective factor. For information about risk factors and protective factors for lung cancer, see the PDQ summary on Lung Cancer Prevention. Key Points - Screening tests have risks. - The risks of lung cancer screening tests include the following: - Finding lung cancer may not improve health or help you live longer. - False-negative test results can occur. - False-positive test results can occur. - Chest x-rays and low-dose spiral CT scans expose the chest to radiation. - Talk to your doctor about your risk for lung cancer and your need for screening tests. Screening tests have risks. Decisions about screening tests can be difficult. Not all screening tests are helpful and most have risks. Before having any screening test, you may want to discuss the test with your doctor. It is important to know the risks of the test and whether it has been proven to reduce the risk of dying from cancer. The risks of lung cancer screening tests include the following: Finding lung cancer may not improve health or help you live longer. Screening may not improve your health or help you live longer if you have lung cancer that has already spread to other places in your body. When a screening test result leads to the diagnosis and treatment of a disease that may never have caused symptoms or become life-threatening, it is called overdiagnosis. It is not known if treatment of these cancers would help you live longer than if no treatment were given, and treatments for cancer may have serious side effects. Harms of treatment may happen more often in people who have medical problems caused by heavy or long-term smoking. False-negative test results can occur. Screening test results may appear to be normal even though lung cancer is present. A person who receives a false-negative test result (one that shows there is no cancer when there really is) may delay seeking medical care even if there are symptoms. False-positive test results can occur. Screening test results may appear to be abnormal even though no cancer is present. A false-positive test result (one that shows there is cancer when there really isn't) can cause anxiety and is usually followed by more tests (such as biopsy), which also have risks. A biopsy to diagnose lung cancer can cause part of the lung to collapse. Sometimes surgery is needed to reinflate the lung. Harms of diagnostic tests may happen more often in patients who have medical problems caused by heavy or long-term smoking. Chest x-rays and low-dose spiral CT scans expose the chest to radiation. Radiation exposure from chest x-rays and low-dose spiral CT scans may increase the risk of cancer. Younger people and people at low risk for lung cancer are more likely to develop lung cancer caused by radiation exposure. . Talk to your doctor about your risk for lung cancer and your need for screening tests. Talk to your doctor or other health care provider about your risk for lung cancer, whether a screening test is right for you, and about the benefits and harms of the screening test. You should take part in the decision about whether a screening test is right for you. (See the PDQ summary on Cancer Screening Overview for more information.)",Lung Cancer,0000032_4,CancerGov,https://www.cancer.gov/types/lung/patient/lung-screening-pdq,C0242379,T191,Disorders What is (are) Parathyroid Cancer ?,0000033_1-1,information,"Key Points - Parathyroid cancer is a rare disease in which malignant (cancer) cells form in the tissues of a parathyroid gland. - Having certain inherited disorders can increase the risk of developing parathyroid cancer. - Signs and symptoms of parathyroid cancer include weakness, feeling tired, and a lump in the neck. - Tests that examine the neck and blood are used to detect (find) and diagnose parathyroid cancer. - Certain factors affect prognosis (chance of recovery) and treatment options. Parathyroid cancer is a rare disease in which malignant (cancer) cells form in the tissues of a parathyroid gland. The parathyroid glands are four pea-sized organs found in the neck near the thyroid gland. The parathyroid glands make parathyroid hormone (PTH or parathormone). PTH helps the body use and store calcium to keep the calcium in the blood at normal levels. A parathyroid gland may become overactive and make too much PTH, a condition called hyperparathyroidism. Hyperparathyroidism can occur when a benign tumor (noncancer), called an adenoma, forms on one of the parathyroid glands, and causes it to grow and become overactive. Sometimes hyperparathyroidism can be caused by parathyroid cancer, but this is very rare. The extra PTH causes: - The calcium stored in the bones to move into the blood. - The intestines to absorb more calcium from the food we eat. This condition is called hypercalcemia (too much calcium in the blood). The hypercalcemia caused by hyperparathyroidism is more serious and life-threatening than parathyroid cancer itself and treating hypercalcemia is as important as treating the cancer.",Parathyroid Cancer,0000033_1,CancerGov,https://www.cancer.gov/types/parathyroid/patient/parathyroid-treatment-pdq,C0153653,T191,Disorders Who is at risk for Parathyroid Cancer? ?,0000033_1-2,susceptibility,Having certain inherited disorders can increase the risk of developing parathyroid cancer. Anything that increases the chance of getting a disease is called a risk factor. Risk factors for parathyroid cancer include the following rare disorders that are inherited (passed down from parent to child): - Familial isolated hyperparathyroidism (FIHP). - Multiple endocrine neoplasia type 1 (MEN1) syndrome. Treatment with radiation therapy may increase the risk of developing a parathyroid adenoma.,Parathyroid Cancer,0000033_1,CancerGov,https://www.cancer.gov/types/parathyroid/patient/parathyroid-treatment-pdq,C0153653,T191,Disorders What are the symptoms of Parathyroid Cancer ?,0000033_1-3,symptoms,"Signs and symptoms of parathyroid cancer include weakness, feeling tired, and a lump in the neck. Most parathyroid cancer signs and symptoms are caused by the hypercalcemia that develops. Signs and symptoms of hypercalcemia include the following: - Weakness. - Feeling very tired. - Nausea and vomiting. - Loss of appetite. - Weight loss for no known reason. - Being much more thirsty than usual. - Urinating much more than usual. - Constipation. - Trouble thinking clearly. Other signs and symptoms of parathyroid cancer include the following: - Pain in the abdomen, side, or back that doesn't go away. - Pain in the bones. - A broken bone. - A lump in the neck. - Change in voice such as hoarseness. - Trouble swallowing. Other conditions may cause the same signs and symptoms as parathyroid cancer. Check with your doctor if you have any of these problems.",Parathyroid Cancer,0000033_1,CancerGov,https://www.cancer.gov/types/parathyroid/patient/parathyroid-treatment-pdq,C0153653,T191,Disorders How to diagnose Parathyroid Cancer ?,0000033_1-4,exams and tests,"Tests that examine the neck and blood are used to detect (find) and diagnose parathyroid cancer. Once blood tests are done and hyperparathyroidism is diagnosed, imaging tests may be done to help find which of the parathyroid glands is overactive. Sometimes the parathyroid glands are hard to find and imaging tests are done to find exactly where they are. Parathyroid cancer may be hard to diagnose because the cells of a benign parathyroid adenoma and a malignant parathyroid cancer look alike. The patient's symptoms, blood levels of calcium and parathyroid hormone, and characteristics of the tumor are also used to make a diagnosis. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. To diagnose parathyroid cancer, the sample of blood is checked for its calcium level. - Parathyroid hormone test: A procedure in which a blood sample is checked to measure the amount of parathyroid hormone released into the blood by the parathyroid glands. A higher than normal amount of parathyroid hormone can be a sign of disease. - Sestamibi scan : A type of radionuclide scan used to find an overactive parathyroid gland. A very small amount of a radioactive substance called technetium 99 is injected into a vein and travels through the bloodstream to the parathyroid gland. The radioactive substance will collect in the overactive gland and show up brightly on a special camera that detects radioactivity. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - SPECT scan (single photon emission computed tomography scan): A procedure that uses a special camera linked to a computer to make a 3-dimensional (3-D) picture. A very small amount of a radioactive substance is injected into a vein. As the substance travels through the blood, the camera rotates around the neck and takes pictures. Blood flow and metabolism are higher than normal in areas where cancer cells are growing. These areas will show up brighter in the picture. This procedure may be done just before or after a CT scan. - Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. - Angiogram : A procedure to look at blood vessels and the flow of blood. A contrast dye is injected into the blood vessel. As the contrast dye moves through the blood vessel, x-rays are taken to see if there are any blockages. - Venous sampling : A procedure in which a sample of blood is taken from specific veins and checked to measure the amounts of certain substances released into the blood by nearby organs and tissues. If imaging tests do not show which parathyroid gland is overactive, blood samples may be taken from veins near each parathyroid gland to find which one is making too much PTH.",Parathyroid Cancer,0000033_1,CancerGov,https://www.cancer.gov/types/parathyroid/patient/parathyroid-treatment-pdq,C0153653,T191,Disorders What is the outlook for Parathyroid Cancer ?,0000033_1-5,outlook,Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - Whether the calcium level in the blood can be controlled. - The stage of the cancer. - Whether the tumor and the capsule around the tumor can be completely removed by surgery. - The patient's general health.,Parathyroid Cancer,0000033_1,CancerGov,https://www.cancer.gov/types/parathyroid/patient/parathyroid-treatment-pdq,C0153653,T191,Disorders What are the stages of Parathyroid Cancer ?,0000033_1-6,stages,"Key Points - After parathyroid cancer has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - There is no standard staging process for parathyroid cancer. After parathyroid cancer has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body. The process used to find out if cancer has spread to other parts of the body is called staging. The following imaging tests may be used to determine if cancer has spread to other parts of the body such as the lungs, liver, bone, heart, pancreas, or lymph nodes: - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if parathyroid cancer spreads to the lung, the cancer cells in the lung are actually parathyroid cancer cells. The disease is metastatic parathyroid cancer, not lung cancer. There is no standard staging process for parathyroid cancer. Parathyroid cancer is described as either localized or metastatic: - Localized parathyroid cancer is found in a parathyroid gland and may have spread to nearby tissues. - Metastatic parathyroid cancer has spread to other parts of the body, such as the lungs, liver, bone, sac around the heart, pancreas, or lymph nodes.",Parathyroid Cancer,0000033_1,CancerGov,https://www.cancer.gov/types/parathyroid/patient/parathyroid-treatment-pdq,C0153653,T191,Disorders What are the treatments for Parathyroid Cancer ?,0000033_1-7,treatment,"Key Points - There are different types of treatment for patients with parathyroid cancer. - Treatment includes control of hypercalcemia (too much calcium in the blood) in patients who have an overactive parathyroid gland. - Four types of standard treatment are used: - Surgery - Radiation therapy - Chemotherapy - Supportive care - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with parathyroid cancer. Different types of treatment are available for patients with parathyroid cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Treatment includes control of hypercalcemia (too much calcium in the blood) in patients who have an overactive parathyroid gland. In order to reduce the amount of parathyroid hormone that is being made and control the level of calcium in the blood, as much of the tumor as possible is removed in surgery. For patients who cannot have surgery, medication may be used. Four types of standard treatment are used: Surgery Surgery (removing the cancer in an operation) is the most common treatment for parathyroid cancer that is in the parathyroid glands or has spread to other parts of the body. Because parathyroid cancer grows very slowly, cancer that has spread to other parts of the body may be removed by surgery in order to cure the patient or control the effects of the disease for a long time. Before surgery, treatment is given to control hypercalcemia. The following surgical procedures may be used: - En bloc resection: Surgery to remove the entire parathyroid gland and the capsule around it. Sometimes lymph nodes, half of the thyroid gland on the same side of the body as the cancer, and muscles, tissues, and a nerve in the neck are also removed. - Tumor debulking: A surgical procedure in which as much of the tumor as possible is removed. Some tumors cannot be completely removed. - Metastasectomy: Surgery to remove any cancer that has spread to distant organs such as the lung. Surgery for parathyroid cancer sometimes damages nerves of the vocal cords. There are treatments to help with speech problems caused by this nerve damage. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External radiation therapy is used to treat parathyroid cancer. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. Supportive care Supportive care is given to lessen the problems caused by the disease or its treatment. Supportive care for hypercalcemia caused by parathyroid cancer may include the following: - Intravenous (IV) fluids. - Drugs that increase how much urine the body makes. - Drugs that stop the body from absorbing calcium from the food we eat. - Drugs that stop the parathyroid gland from making parathyroid hormone. New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Parathyroid cancer often recurs. Patients should have regular check-ups for the rest of their lives, to find and treat recurrences early. Treatment Options for Parathyroid Cancer Localized Parathyroid Cancer Treatment of localized parathyroid cancer may include the following: - Surgery (en bloc resection). - Surgery followed by radiation therapy. - Radiation therapy. - Supportive care to treat hypercalcemia (too much calcium in the blood). Check the list of NCI-supported cancer clinical trials that are now accepting patients with localized parathyroid cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Metastatic Parathyroid Cancer Treatment of metastatic parathyroid cancer may include the following: - Surgery (metastasectomy) to remove cancer from the places where it has spread. - Surgery followed by radiation therapy. - Radiation therapy. - Chemotherapy. - Supportive care to treat hypercalcemia (too much calcium in the blood). Check the list of NCI-supported cancer clinical trials that are now accepting patients with metastatic parathyroid cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Recurrent Parathyroid Cancer Treatment of recurrent parathyroid cancer may include the following: - Surgery (metastasectomy) to remove cancer from the places where it has recurred. - Surgery (tumor debulking). - Surgery followed by radiation therapy. - Radiation therapy. - Chemotherapy. - Supportive care to treat hypercalcemia (too much calcium in the blood). Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent parathyroid cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Parathyroid Cancer,0000033_1,CancerGov,https://www.cancer.gov/types/parathyroid/patient/parathyroid-treatment-pdq,C0153653,T191,Disorders what research (or clinical trials) is being done for Parathyroid Cancer ?,0000033_1-8,research,"New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Parathyroid Cancer,0000033_1,CancerGov,https://www.cancer.gov/types/parathyroid/patient/parathyroid-treatment-pdq,C0153653,T191,Disorders What is (are) Penile Cancer ?,0000034_1-1,information,"Key Points - Penile cancer is a disease in which malignant (cancer) cells form in the tissues of the penis. - Human papillomavirus infection may increase the risk of developing penile cancer. - Signs of penile cancer include sores, discharge, and bleeding. - Tests that examine the penis are used to detect (find) and diagnose penile cancer. - Certain factors affect prognosis (chance of recovery) and treatment options. Penile cancer is a disease in which malignant (cancer) cells form in the tissues of the penis. The penis is a rod-shaped male reproductive organ that passes sperm and urine from the body. It contains two types of erectile tissue (spongy tissue with blood vessels that fill with blood to make an erection): - Corpora cavernosa: The two columns of erectile tissue that form most of the penis. - Corpus spongiosum: The single column of erectile tissue that forms a small portion of the penis. The corpus spongiosum surrounds the urethra (the tube through which urine and sperm pass from the body). The erectile tissue is wrapped in connective tissue and covered with skin. The glans (head of the penis) is covered with loose skin called the foreskin.",Penile Cancer,0000034_1,CancerGov,https://www.cancer.gov/types/penile/patient/penile-treatment-pdq,C0853105,T191,Disorders Who is at risk for Penile Cancer? ?,0000034_1-2,susceptibility,Human papillomavirus infection may increase the risk of developing penile cancer. Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for penile cancer include the following: Circumcision may help prevent infection with the human papillomavirus (HPV). A circumcision is an operation in which the doctor removes part or all of the foreskin from the penis. Many boys are circumcised shortly after birth. Men who were not circumcised at birth may have a higher risk of developing penile cancer. Other risk factors for penile cancer include the following: - Being age 60 or older. - Having phimosis (a condition in which the foreskin of the penis cannot be pulled back over the glans). - Having poor personal hygiene. - Having many sexual partners. - Using tobacco products.,Penile Cancer,0000034_1,CancerGov,https://www.cancer.gov/types/penile/patient/penile-treatment-pdq,C0853105,T191,Disorders What are the symptoms of Penile Cancer ?,0000034_1-3,symptoms,"Signs of penile cancer include sores, discharge, and bleeding. These and other signs may be caused by penile cancer or by other conditions. Check with your doctor if you have any of the following: - Redness, irritation, or a sore on the penis. - A lump on the penis.",Penile Cancer,0000034_1,CancerGov,https://www.cancer.gov/types/penile/patient/penile-treatment-pdq,C0853105,T191,Disorders How to diagnose Penile Cancer ?,0000034_1-4,exams and tests,"Tests that examine the penis are used to detect (find) and diagnose penile cancer. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking the penis for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. The tissue sample is removed during one of the following procedures: - Fine-needle aspiration (FNA) biopsy : The removal of tissue or fluid using a thin needle. - Incisional biopsy : The removal of part of a lump or a sample of tissue that doesn't look normal. - Excisional biopsy : The removal of an entire lump or area of tissue that doesnt look normal.",Penile Cancer,0000034_1,CancerGov,https://www.cancer.gov/types/penile/patient/penile-treatment-pdq,C0853105,T191,Disorders What is the outlook for Penile Cancer ?,0000034_1-5,outlook,Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - The stage of the cancer. - The location and size of the tumor. - Whether the cancer has just been diagnosed or has recurred (come back).,Penile Cancer,0000034_1,CancerGov,https://www.cancer.gov/types/penile/patient/penile-treatment-pdq,C0853105,T191,Disorders What are the stages of Penile Cancer ?,0000034_1-6,stages,"Key Points - After penile cancer has been diagnosed, tests are done to find out if cancer cells have spread within the penis or to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - The following stages are used for penile cancer: - Stage 0 (Carcinoma in Situ) - Stage I - Stage II - Stage III - Stage IV After penile cancer has been diagnosed, tests are done to find out if cancer cells have spread within the penis or to other parts of the body. The process used to find out if cancer has spread within the penis or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following tests and procedures may be used in the staging process: - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. A substance called gadolinium is injected into a vein. The gadolinium collects around the cancer cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. - Chest x-ray : An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. - Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. The tissue sample is removed during one of the following procedures: - Sentinel lymph node biopsy : The removal of the sentinel lymph node during surgery. The sentinel lymph node is the first lymph node to receive lymphatic drainage from a tumor. It is the first lymph node the cancer is likely to spread to from the tumor. A radioactive substance and/or blue dye is injected near the tumor. The substance or dye flows through the lymph ducts to the lymph nodes. The first lymph node to receive the substance or dye is removed. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are not found, it may not be necessary to remove more lymph nodes. - Lymph node dissection : A procedure to remove one or more lymph nodes during surgery. A sample of tissue is checked under a microscope for signs of cancer. This procedure is also called a lymphadenectomy. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if penile cancer spreads to the lung, the cancer cells in the lung are actually penile cancer cells. The disease is metastatic penile cancer, not lung cancer. The following stages are used for penile cancer: Stage 0 (Carcinoma in Situ) In stage 0, abnormal cells or growths that look like warts are found on the surface of the skin of the penis. These abnormal cells or growths may become cancer and spread into nearby normal tissue. Stage 0 is also called carcinoma in situ. Stage I In stage I, cancer has formed and spread to connective tissue just under the skin of the penis. Cancer has not spread to lymph vessels or blood vessels. The tumor cells look a lot like normal cells under a microscope. Stage II In stage II, cancer has spread: - to connective tissue just under the skin of the penis. Also, cancer has spread to lymph vessels or blood vessels or the tumor cells may look very different from normal cells under a microscope; or - through connective tissue to erectile tissue (spongy tissue that fills with blood to make an erection); or - beyond erectile tissue to the urethra. Stage III Stage III is divided into stage IIIa and stage IIIb. In stage IIIa, cancer has spread to one lymph node in the groin. Cancer has also spread: - to connective tissue just under the skin of the penis. Also, cancer may have spread to lymph vessels or blood vessels or the tumor cells may look very different from normal cells under a microscope; or - through connective tissue to erectile tissue (spongy tissue that fills with blood to make an erection); or - beyond erectile tissue to the urethra. In stage IIIb, cancer has spread to more than one lymph node on one side of the groin or to lymph nodes on both sides of the groin. Cancer has also spread: - to connective tissue just under the skin of the penis. Also, cancer may have spread to lymph vessels or blood vessels or the tumor cells may look very different from normal cells under a microscope; or - through connective tissue to erectile tissue (spongy tissue that fills with blood to make an erection); or - beyond erectile tissue to the urethra. Stage IV In stage IV, cancer has spread: - to tissues near the penis such as the prostate, and may have spread to lymph nodes in the groin or pelvis; or - to one or more lymph nodes in the pelvis, or cancer has spread from the lymph nodes to the tissues around the lymph nodes; or - to distant parts of the body.",Penile Cancer,0000034_1,CancerGov,https://www.cancer.gov/types/penile/patient/penile-treatment-pdq,C0853105,T191,Disorders What are the treatments for Penile Cancer ?,0000034_1-7,treatment,"Key Points - There are different types of treatment for patients with penile cancer. - Four types of standard treatment are used: - Surgery - Radiation therapy - Chemotherapy - Biologic therapy - New types of treatment are being tested in clinical trials. - Radiosensitizers - Sentinel lymph node biopsy followed by surgery - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with penile cancer. Different types of treatments are available for patients with penile cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Four types of standard treatment are used: Surgery Surgery is the most common treatment for all stages of penile cancer. A doctor may remove the cancer using one of the following operations: - Mohs microsurgery: A procedure in which the tumor is cut from the skin in thin layers. During the surgery, the edges of the tumor and each layer of tumor removed are viewed through a microscope to check for cancer cells. Layers continue to be removed until no more cancer cells are seen. This type of surgery removes as little normal tissue as possible and is often used to remove cancer on the skin. It is also called Mohs surgery. - Laser surgery: A surgical procedure that uses a laser beam (a narrow beam of intense light) as a knife to make bloodless cuts in tissue or to remove a surface lesion such as a tumor. - Cryosurgery: A treatment that uses an instrument to freeze and destroy abnormal tissue. This type of treatment is also called cryotherapy. - Circumcision: Surgery to remove part or all of the foreskin of the penis. - Wide local excision: Surgery to remove only the cancer and some normal tissue around it. - Amputation of the penis: Surgery to remove part or all of the penis. If part of the penis is removed, it is a partial penectomy. If all of the penis is removed, it is a total penectomy. Lymph nodes in the groin may be taken out during surgery. Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External and internal radiation therapy are used to treat penile cancer. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly onto the skin (topical chemotherapy) or into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. Topical chemotherapy may be used to treat stage 0 penile cancer. See Drugs Approved for Penile Cancer for more information. Biologic therapy Biologic therapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy. Topical biologic therapy with imiquimod may be used to treat stage 0 penile cancer. New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Radiosensitizers Radiosensitizers are drugs that make tumor cells more sensitive to radiation therapy. Combining radiation therapy with radiosensitizers helps kill more tumor cells. Sentinel lymph node biopsy followed by surgery Sentinel lymph node biopsy is the removal of the sentinel lymph node during surgery. The sentinel lymph node is the first lymph node to receive lymphatic drainage from a tumor. It is the first lymph node the cancer is likely to spread to from the tumor. A radioactive substance and/or blue dye is injected near the tumor. The substance or dye flows through the lymph ducts to the lymph nodes. The first lymph node to receive the substance or dye is removed. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are not found, it may not be necessary to remove more lymph nodes. After the sentinel lymph node biopsy, the surgeon removes the cancer. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options by Stage Stage 0 (Carcinoma in Situ) Treatment of stage 0 may be one of the following: - Mohs microsurgery. - Topical chemotherapy. - Topical biologic therapy with imiquimod. - Laser surgery. - Cryosurgery. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage 0 penile cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage I Penile Cancer If the cancer is only in the foreskin, wide local excision and circumcision may be the only treatment needed. Treatment of stage I penile cancer may include the following: - Surgery (partial or total penectomy with or without removal of lymph nodes in the groin. - External or internal radiation therapy. - Mohs microsurgery. - A clinical trial of laser therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I penile cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage II Penile Cancer Treatment of stage II penile cancer may include the following: - Surgery (partial or total penectomy, with or without removal of lymph nodes in the groin). - External or internal radiation therapy followed by surgery. - A clinical trial of sentinel lymph node biopsy followed by surgery. - A clinical trial of laser surgery. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage II penile cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage III Penile Cancer Treatment of stage III penile cancer may include the following: - Surgery (penectomy and removal of lymph nodes in the groin) with or without radiation therapy. - Radiation therapy. - A clinical trial of sentinel lymph node biopsy followed by surgery. - A clinical trial of radiosensitizers. - A clinical trial of chemotherapy before or after surgery. - A clinical trial of new drugs, biologic therapy, or new kinds of surgery. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III penile cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage IV Penile Cancer Treatment of stage IV penile cancer is usually palliative (to relieve symptoms and improve the quality of life). Treatment may include the following: - Surgery (wide local excision and removal of lymph nodes in the groin). - Radiation therapy. - A clinical trial of chemotherapy before or after surgery. - A clinical trial of new drugs, biologic therapy, or new kinds of surgery. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage IV penile cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Penile Cancer,0000034_1,CancerGov,https://www.cancer.gov/types/penile/patient/penile-treatment-pdq,C0853105,T191,Disorders what research (or clinical trials) is being done for Penile Cancer ?,0000034_1-8,research,"New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Radiosensitizers Radiosensitizers are drugs that make tumor cells more sensitive to radiation therapy. Combining radiation therapy with radiosensitizers helps kill more tumor cells. Sentinel lymph node biopsy followed by surgery Sentinel lymph node biopsy is the removal of the sentinel lymph node during surgery. The sentinel lymph node is the first lymph node to receive lymphatic drainage from a tumor. It is the first lymph node the cancer is likely to spread to from the tumor. A radioactive substance and/or blue dye is injected near the tumor. The substance or dye flows through the lymph ducts to the lymph nodes. The first lymph node to receive the substance or dye is removed. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are not found, it may not be necessary to remove more lymph nodes. After the sentinel lymph node biopsy, the surgeon removes the cancer. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Penile Cancer,0000034_1,CancerGov,https://www.cancer.gov/types/penile/patient/penile-treatment-pdq,C0853105,T191,Disorders What is (are) Pituitary Tumors ?,0000035_1-1,information,"Key Points - A pituitary tumor is a growth of abnormal cells in the tissues of the pituitary gland. - The pituitary gland hormones control many other glands in the body. - Having certain genetic conditions increases the risk of developing a pituitary tumor. - Signs of a pituitary tumor include problems with vision and certain physical changes. - Imaging studies and tests that examine the blood and urine are used to detect (find) and diagnose a pituitary tumor. - Certain factors affect prognosis (chance of recovery) and treatment options. A pituitary tumor is a growth of abnormal cells in the tissues of the pituitary gland. Pituitary tumors form in the pituitary gland, a pea-sized organ in the center of the brain, just above the back of the nose. The pituitary gland is sometimes called the ""master endocrine gland"" because it makes hormones that affect the way many parts of the body work. It also controls hormones made by many other glands in the body. Pituitary tumors are divided into three groups: - Benign pituitary adenomas: Tumors that are not cancer. These tumors grow very slowly and do not spread from the pituitary gland to other parts of the body. - Invasive pituitary adenomas: Benign tumors that may spread to bones of the skull or the sinus cavity below the pituitary gland. - Pituitary carcinomas: Tumors that are malignant (cancer). These pituitary tumors spread into other areas of the central nervous system (brain and spinal cord) or outside of the central nervous system. Very few pituitary tumors are malignant. Pituitary tumors may be either non-functioning or functioning. - Non-functioning pituitary tumors do not make extra amounts of hormones. - Functioning pituitary tumors make more than the normal amount of one or more hormones. Most pituitary tumors are functioning tumors. The extra hormones made by pituitary tumors may cause certain signs or symptoms of disease. The pituitary gland hormones control many other glands in the body. Hormones made by the pituitary gland include: - Prolactin: A hormone that causes a womans breasts to make milk during and after pregnancy. - Adrenocorticotropic hormone (ACTH): A hormone that causes the adrenal glands to make a hormone called cortisol. Cortisol helps control the use of sugar, protein, and fats in the body and helps the body deal with stress. - Growth hormone: A hormone that helps control body growth and the use of sugar and fat in the body. Growth hormone is also called somatotropin. - Thyroid-stimulating hormone: A hormone that causes the thyroid gland to make other hormones that control growth, body temperature, and heart rate. Thyroid-stimulating hormone is also called thyrotropin. - Luteinizing hormone (LH) and follicle-stimulating hormone (FSH): Hormones that control the menstrual cycle in women and the making of sperm in men.",Pituitary Tumors,0000035_1,CancerGov,https://www.cancer.gov/types/pituitary/patient/pituitary-treatment-pdq,C0032019,T191,Disorders Who is at risk for Pituitary Tumors? ?,0000035_1-2,susceptibility,Having certain genetic conditions increases the risk of developing a pituitary tumor.Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesnt mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for pituitary tumors include having the following hereditary diseases: - Multiple endocrine neoplasia type 1 (MEN1) syndrome. - Carney complex. - Isolated familial acromegaly.,Pituitary Tumors,0000035_1,CancerGov,https://www.cancer.gov/types/pituitary/patient/pituitary-treatment-pdq,C0032019,T191,Disorders What are the symptoms of Pituitary Tumors ?,0000035_1-3,symptoms,"Signs of a pituitary tumor include problems with vision and certain physical changes. Signs and symptoms can be caused by the growth of the tumor and/or by hormones the tumor makes or by other conditions. Some tumors may not cause signs or symptoms. Check with your doctor if you have any of these problems. Signs and symptoms of a non-functioning pituitary tumor Sometimes, a pituitary tumor may press on or damage parts of the pituitary gland, causing it to stop making one or more hormones. Too little of a certain hormone will affect the work of the gland or organ that the hormone controls. The following signs and symptoms may occur: - Headache. - Some loss of vision. - Loss of body hair. - In women, less frequent or no menstrual periods or no milk from the breasts. - In men, loss of facial hair, growth of breast tissue, and impotence. - In women and men, lower sex drive. - In children, slowed growth and sexual development. Most of the tumors that make LH and FSH do not make enough extra hormone to cause signs and symptoms. These tumors are considered to be non-functioning tumors. Signs and symptoms of a functioning pituitary tumor When a functioning pituitary tumor makes extra hormones, the signs and symptoms will depend on the type of hormone being made. Too much prolactin may cause: - Headache. - Some loss of vision. - Less frequent or no menstrual periods or menstrual periods with a very light flow. - Trouble becoming pregnant or an inability to become pregnant. - Impotence in men. - Lower sex drive. - Flow of breast milk in a woman who is not pregnant or breast-feeding. Too much ACTH may cause: - Headache. - Some loss of vision. - Weight gain in the face, neck, and trunk of the body, and thin arms and legs. - A lump of fat on the back of the neck. - Thin skin that may have purple or pink stretch marks on the chest or abdomen. - Easy bruising. - Growth of fine hair on the face, upper back, or arms. - Bones that break easily. - Anxiety, irritability, and depression. Too much growth hormone may cause: - Headache. - Some loss of vision. - In adults, acromegaly (growth of the bones in the face, hands, and feet). In children, the whole body may grow much taller and larger than normal. - Tingling or numbness in the hands and fingers. - Snoring or pauses in breathing during sleep. - Joint pain. - Sweating more than usual. - Dysmorphophobia (extreme dislike of or concern about one or more parts of the body). Too much thyroid-stimulating hormone may cause: - Irregular heartbeat. - Shakiness. - Weight loss. - Trouble sleeping. - Frequent bowel movements. - Sweating. Other general signs and symptoms of pituitary tumors: - Nausea and vomiting. - Confusion. - Dizziness. - Seizures. - Runny or ""drippy"" nose (cerebrospinal fluid that surrounds the brain and spinal cord leaks into the nose).",Pituitary Tumors,0000035_1,CancerGov,https://www.cancer.gov/types/pituitary/patient/pituitary-treatment-pdq,C0032019,T191,Disorders How to diagnose Pituitary Tumors ?,0000035_1-4,exams and tests,"Imaging studies and tests that examine the blood and urine are used to detect (find) and diagnose a pituitary tumor. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Eye exam: An exam to check vision and the general health of the eyes. - Visual field exam: An exam to check a persons field of vision (the total area in which objects can be seen). This test measures both central vision (how much a person can see when looking straight ahead) and peripheral vision (how much a person can see in all other directions while staring straight ahead). The eyes are tested one at a time. The eye not being tested is covered. - Neurological exam : A series of questions and tests to check the brain, spinal cord, and nerve function. The exam checks a persons mental status, coordination, and ability to walk normally, and how well the muscles, senses, and reflexes work. This may also be called a neuro exam or a neurologic exam. - MRI (magnetic resonance imaging) with gadolinium : A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the brain and spinal cord. A substance called gadolinium is injected into a vein. The gadolinium collects around the cancer cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Blood chemistry study : A procedure in which a blood sample is checked to measure the amounts of certain substances, such as glucose (sugar), released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. - Blood tests: Tests to measure the levels of testosterone or estrogen in the blood. A higher or lower than normal amount of these hormones may be a sign of pituitary tumor. - Twenty-four-hour urine test: A test in which urine is collected for 24 hours to measure the amounts of certain substances. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. A higher than normal amount of the hormone cortisol may be a sign of a pituitary tumor and Cushing syndrome. - High-dose dexamethasone suppression test: A test in which one or more high doses of dexamethasone are given. The level of cortisol is checked from a sample of blood or from urine that is collected for three days. This test is done to check if the adrenal gland is making too much cortisol or if the pituitary gland is telling the adrenal glands to make too much cortisol. - Low-dose dexamethasone suppression test: A test in which one or more small doses of dexamethasone are given. The level of cortisol is checked from a sample of blood or from urine that is collected for three days. This test is done to check if the adrenal gland is making too much cortisol. - Venous sampling for pituitary tumors: A procedure in which a sample of blood is taken from veins coming from the pituitary gland. The sample is checked to measure the amount of ACTH released into the blood by the gland. Venous sampling may be done if blood tests show there is a tumor making ACTH, but the pituitary gland looks normal in the imaging tests. - Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. The following tests may be done on the sample of tissue that is removed: - Immunohistochemistry : A test that uses antibodies to check for certain antigens in a sample of tissue. The antibody is usually linked to a radioactive substance or a dye that causes the tissue to light up under a microscope. This type of test may be used to tell the difference between different types of cancer. - Immunocytochemistry : A test that uses antibodies to check for certain antigens in a sample of cells. The antibody is usually linked to a radioactive substance or a dye that causes the cells to light up under a microscope. This type of test may be used to tell the difference between different types of cancer. - Light and electron microscopy : A laboratory test in which cells in a sample of tissue are viewed under regular and high-powered microscopes to look for certain changes in the cells.",Pituitary Tumors,0000035_1,CancerGov,https://www.cancer.gov/types/pituitary/patient/pituitary-treatment-pdq,C0032019,T191,Disorders What is the outlook for Pituitary Tumors ?,0000035_1-5,outlook,Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) depends on the type of tumor and whether the tumor has spread into other areas of the central nervous system (brain and spinal cord) or outside of the central nervous system to other parts of the body. Treatment options depend on the following: - The type and size of the tumor. - Whether the tumor is making hormones. - Whether the tumor is causing problems with vision or other signs or symptoms. - Whether the tumor has spread into the brain around the pituitary gland or to other parts of the body. - Whether the tumor has just been diagnosed or has recurred (come back).,Pituitary Tumors,0000035_1,CancerGov,https://www.cancer.gov/types/pituitary/patient/pituitary-treatment-pdq,C0032019,T191,Disorders What are the stages of Pituitary Tumors ?,0000035_1-6,stages,"Key Points - Once a pituitary tumor has been diagnosed, tests are done to find out if it has spread within the central nervous system (brain and spinal cord) or to other parts of the body. - Pituitary tumors are described in several ways. Once a pituitary tumor has been diagnosed, tests are done to find out if it has spread within the central nervous system (brain and spinal cord) or to other parts of the body. The extent or spread of cancer is usually described as stages. There is no standard staging system for pituitary tumors. Once a pituitary tumor is found, tests are done to find out if the tumor has spread into the brain or to other parts of the body. The following test may be used: - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). Pituitary tumors are described in several ways. Pituitary tumors are described by their size and grade, whether or not they make extra hormones, and whether the tumor has spread to other parts of the body. The following sizes are used: - Microadenoma: The tumor is smaller than 1 centimeter. - Macroadenoma: The tumor is 1 centimeter or larger. Most pituitary adenomas are microadenomas. The grade of a pituitary tumor is based on how far it has grown into the surrounding area of the brain, including the sella (the bone at the base of the skull, where the pituitary gland sits).",Pituitary Tumors,0000035_1,CancerGov,https://www.cancer.gov/types/pituitary/patient/pituitary-treatment-pdq,C0032019,T191,Disorders What are the treatments for Pituitary Tumors ?,0000035_1-7,treatment,"Key Points - There are different types of treatment for patients with pituitary tumors. - Four types of standard treatment are used: - Surgery - Radiation therapy - Drug therapy - Chemotherapy - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with pituitary tumors. Different types of treatments are available for patients with pituitary tumors. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Four types of standard treatment are used: Surgery Many pituitary tumors can be removed by surgery using one of the following operations: - Transsphenoidal surgery: A type of surgery in which the instruments are inserted into part of the brain by going through an incision (cut) made under the upper lip or at the bottom of the nose between the nostrils and then through the sphenoid bone (a butterfly-shaped bone at the base of the skull) to reach the pituitary gland. The pituitary gland lies just above the sphenoid bone. - Endoscopic transsphenoidal surgery: A type of surgery in which an endoscope is inserted through an incision (cut) made at the back of the inside of the nose and then through the sphenoid bone to reach the pituitary gland. An endoscope is a thin, tube-like instrument with a light, a lens for viewing, and a tool for removing tumor tissue. - Craniotomy: Surgery to remove the tumor through an opening made in the skull. Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. Certain ways of giving radiation therapy can help keep radiation from damaging nearby healthy tissue. This type of radiation therapy may include the following: - Stereotactic radiosurgery: A rigid head frame is attached to the skull to keep the head still during the radiation treatment. A machine aims a single large dose of radiation directly at the tumor. This procedure does not involve surgery. It is also called stereotaxic radiosurgery, radiosurgery, and radiation surgery. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type of the cancer being treated. External radiation therapy is used to treat pituitary tumors. Drug therapy Drugs may be given to stop a functioning pituitary tumor from making too many hormones. Chemotherapy Chemotherapy may be used as palliative treatment for pituitary carcinomas, to relieve symptoms and improve the patient's quality of life. Chemotherapy uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type of the cancer being treated. New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Pituitary Tumors Non-functioning Pituitary Tumors Treatment may include the following: - Surgery (transsphenoidal surgery, if possible) to remove the tumor, followed by watchful waiting (closely monitoring a patients condition without giving any treatment until signs or symptoms appear or change). Radiation therapy is given if the tumor comes back. - Radiation therapy alone. Treatment for luteinizing hormone -producing and follicle-stimulating hormone -producing tumors is usually transsphenoidal surgery to remove the tumor. Prolactin-Producing Pituitary Tumors Treatment may include the following: - Drug therapy to stop the tumor from making prolactin and to stop the tumor from growing. - Surgery to remove the tumor (transsphenoidal surgery or craniotomy) when the tumor does not respond to drug therapy or when the patient cannot take the drug. - Radiation therapy. - Surgery followed by radiation therapy. ACTH-Producing Pituitary Tumors Treatment may include the following: - Surgery (usually transsphenoidal surgery) to remove the tumor, with or without radiation therapy. - Radiation therapy alone. - Drug therapy to stop the tumor from making ACTH. - A clinical trial of stereotactic radiation surgery. Growth HormoneProducing Pituitary Tumors Treatment may include the following: - Surgery (usually transsphenoidal or endoscopic transsphenoidal surgery) to remove the tumor, with or without radiation therapy. - Drug therapy to stop the tumor from making growth hormone. Thyroid-Stimulating HormoneProducing Tumors Treatment may include the following: - Surgery (usually transsphenoidal surgery) to remove the tumor, with or without radiation therapy. - Drug therapy to stop the tumor from making hormones. Pituitary Carcinomas Treatment of pituitary carcinomas is palliative, to relieve symptoms and improve the quality of life. Treatment may include the following: - Surgery (transsphenoidal surgery or craniotomy) to remove the cancer, with or without radiation therapy. - Drug therapy to stop the tumor from making hormones. - Chemotherapy. Recurrent Pituitary Tumors Treatment may include the following: - Radiation therapy. - A clinical trial of stereotactic radiation surgery.",Pituitary Tumors,0000035_1,CancerGov,https://www.cancer.gov/types/pituitary/patient/pituitary-treatment-pdq,C0032019,T191,Disorders what research (or clinical trials) is being done for Pituitary Tumors ?,0000035_1-8,research,"New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Pituitary Tumors,0000035_1,CancerGov,https://www.cancer.gov/types/pituitary/patient/pituitary-treatment-pdq,C0032019,T191,Disorders What is (are) Prostate Cancer ?,0000036_1-1,information,"Key Points - Prostate cancer is a disease in which malignant (cancer) cells form in the tissues of the prostate. - Signs of prostate cancer include a weak flow of urine or frequent urination. - Tests that examine the prostate and blood are used to detect (find) and diagnose prostate cancer. - Certain factors affect prognosis (chance of recovery) and treatment options. Prostate cancer is a disease in which malignant (cancer) cells form in the tissues of the prostate. The prostate is a gland in the male reproductive system. It lies just below the bladder (the organ that collects and empties urine) and in front of the rectum (the lower part of the intestine). It is about the size of a walnut and surrounds part of the urethra (the tube that empties urine from the bladder). The prostate gland makes fluid that is part of the semen. Prostate cancer is most common in older men. In the U.S., about 1 out of 5 men will be diagnosed with prostate cancer.",Prostate Cancer,0000036_1,CancerGov,https://www.cancer.gov/types/prostate/patient/prostate-treatment-pdq,C0376358,T191,Disorders What are the symptoms of Prostate Cancer ?,0000036_1-2,symptoms,"Signs of prostate cancer include a weak flow of urine or frequent urination. These and other signs and symptoms may be caused by prostate cancer or by other conditions. Check with your doctor if you have any of the following: - Weak or interrupted (""stop-and-go"") flow of urine. - Sudden urge to urinate. - Frequent urination (especially at night). - Trouble starting the flow of urine. - Trouble emptying the bladder completely. - Pain or burning while urinating. - Blood in the urine or semen. - A pain in the back, hips, or pelvis that doesn't go away. - Shortness of breath, feeling very tired, fast heartbeat, dizziness, or pale skin caused by anemia. Other conditions may cause the same symptoms. As men age, the prostate may get bigger and block the urethra or bladder. This may cause trouble urinating or sexual problems. The condition is called benign prostatic hyperplasia (BPH), and although it is not cancer, surgery may be needed. The symptoms of benign prostatic hyperplasia or of other problems in the prostate may be like symptoms of prostate cancer.",Prostate Cancer,0000036_1,CancerGov,https://www.cancer.gov/types/prostate/patient/prostate-treatment-pdq,C0376358,T191,Disorders How to diagnose Prostate Cancer ?,0000036_1-3,exams and tests,"Tests that examine the prostate and blood are used to detect (find) and diagnose prostate cancer. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Digital rectal exam (DRE): An exam of the rectum. The doctor or nurse inserts a lubricated, gloved finger into the rectum and feels the prostate through the rectal wall for lumps or abnormal areas. - Prostate-specific antigen (PSA) test : A test that measures the level of PSA in the blood. PSA is a substance made by the prostate that may be found in an increased amount in the blood of men who have prostate cancer. PSA levels may also be high in men who have an infection or inflammation of the prostate or BPH (an enlarged, but noncancerous, prostate). - Transrectal ultrasound : A procedure in which a probe that is about the size of a finger is inserted into the rectum to check the prostate. The probe is used to bounce high-energy sound waves (ultrasound) off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. Transrectal ultrasound may be used during a biopsy procedure. - Transrectal magnetic resonance imaging (MRI): A procedure that uses a strong magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. A probe that gives off radio waves is inserted into the rectum near the prostate. This helps the MRI machine make clearer pictures of the prostate and nearby tissue. A transrectal MRI is done to find out if the cancer has spread outside the prostate into nearby tissues. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Biopsy: The removal of cells or tissues so they can be viewed under a microscope by a pathologist. The pathologist will check the tissue sample to see if there are cancer cells and find out the Gleason score. The Gleason score ranges from 2-10 and describes how likely it is that a tumor will spread. The lower the number, the less likely the tumor is to spread. A transrectal biopsy is used to diagnose prostate cancer. A transrectal biopsy is the removal of tissue from the prostate by inserting a thin needle through the rectum and into the prostate. This procedure is usually done using transrectal ultrasound to help guide where samples of tissue are taken from. A pathologist views the tissue under a microscope to look for cancer cells.",Prostate Cancer,0000036_1,CancerGov,https://www.cancer.gov/types/prostate/patient/prostate-treatment-pdq,C0376358,T191,Disorders What is the outlook for Prostate Cancer ?,0000036_1-4,outlook,"Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - The stage of the cancer (level of PSA, Gleason score, grade of the tumor, how much of the prostate is affected by the cancer, and whether the cancer has spread to other places in the body). - The patients age. - Whether the cancer has just been diagnosed or has recurred (come back). Treatment options also may depend on the following: - Whether the patient has other health problems. - The expected side effects of treatment. - Past treatment for prostate cancer. - The wishes of the patient. Most men diagnosed with prostate cancer do not die of it.",Prostate Cancer,0000036_1,CancerGov,https://www.cancer.gov/types/prostate/patient/prostate-treatment-pdq,C0376358,T191,Disorders What are the stages of Prostate Cancer ?,0000036_1-5,stages,"Key Points - After prostate cancer has been diagnosed, tests are done to find out if cancer cells have spread within the prostate or to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - The following stages are used for prostate cancer: - Stage I - Stage II - Stage III - Stage IV After prostate cancer has been diagnosed, tests are done to find out if cancer cells have spread within the prostate or to other parts of the body. The process used to find out if cancer has spread within the prostate or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The results of the tests used to diagnose prostate cancer are often also used to stage the disease. (See the General Information section.) In prostate cancer, staging tests may not be done unless the patient has symptoms or signs that the cancer has spread, such as bone pain, a high PSA level, or a high Gleason score. The following tests and procedures also may be used in the staging process: - Bone scan : A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones and is detected by a scanner. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - Pelvic lymphadenectomy : A surgical procedure to remove the lymph nodes in the pelvis. A pathologist views the tissue under a microscope to look for cancer cells. - Seminal vesicle biopsy : The removal of fluid from the seminal vesicles (glands that make semen) using a needle. A pathologist views the fluid under a microscope to look for cancer cells. - ProstaScint scan : A procedure to check for cancer that has spread from the prostate to other parts of the body, such as the lymph nodes. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material attaches to prostate cancer cells and is detected by a scanner. The radioactive material shows up as a bright spot on the picture in areas where there are a lot of prostate cancer cells. The stage of the cancer is based on the results of the staging and diagnostic tests, including the prostate-specific antigen (PSA) test and the Gleason score. The tissue samples removed during the biopsy are used to find out the Gleason score. The Gleason score ranges from 2-10 and describes how different the cancer cells look from normal cells and how likely it is that the tumor will spread. The lower the number, the less likely the tumor is to spread. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if prostate cancer spreads to the bone, the cancer cells in the bone are actually prostate cancer cells. The disease is metastatic prostate cancer, not bone cancer. Denosumab, a monoclonal antibody, may be used to prevent bone metastases. The following stages are used for prostate cancer: Stage I In stage I, cancer is found in the prostate only. The cancer: - is found by needle biopsy (done for a high PSA level) or in a small amount of tissue during surgery for other reasons (such as benign prostatic hyperplasia). The PSA level is lower than 10 and the Gleason score is 6 or lower; or - is found in one-half or less of one lobe of the prostate. The PSA level is lower than 10 and the Gleason score is 6 or lower; or - cannot be felt during a digital rectal exam and cannot be seen in imaging tests. Cancer is found in one-half or less of one lobe of the prostate. The PSA level and the Gleason score are not known. Stage II In stage II, cancer is more advanced than in stage I, but has not spread outside the prostate. Stage II is divided into stages IIA and IIB. In stage IIA, cancer: - is found by needle biopsy (done for a high PSA level) or in a small amount of tissue during surgery for other reasons (such as benign prostatic hyperplasia). The PSA level is lower than 20 and the Gleason score is 7; or - is found by needle biopsy (done for a high PSA level) or in a small amount of tissue during surgery for other reasons (such as benign prostatic hyperplasia). The PSA level is at least 10 but lower than 20 and the Gleason score is 6 or lower; or - is found in one-half or less of one lobe of the prostate. The PSA level is at least 10 but lower than 20 and the Gleason score is 6 or lower; or - is found in one-half or less of one lobe of the prostate. The PSA level is lower than 20 and the Gleason score is 7; or - is found in more than one-half of one lobe of the prostate. In stage IIB, cancer: - is found in opposite sides of the prostate. The PSA can be any level and the Gleason score can range from 2 to 10; or - cannot be felt during a digital rectal exam and cannot be seen in imaging tests. The PSA level is 20 or higher and the Gleason score can range from 2 to 10; or - cannot be felt during a digital rectal exam and cannot be seen in imaging tests. The PSA can be any level and the Gleason score is 8 or higher. Stage III In stage III, cancer has spread beyond the outer layer of the prostate and may have spread to the seminal vesicles. The PSA can be any level and the Gleason score can range from 2 to 10. Stage IV In stage IV, the PSA can be any level and the Gleason score can range from 2 to 10. Also, cancer: - has spread beyond the seminal vesicles to nearby tissue or organs, such as the rectum, bladder, or pelvic wall; or - may have spread to the seminal vesicles or to nearby tissue or organs, such as the rectum, bladder, or pelvic wall. Cancer has spread to nearby lymph nodes; or - has spread to distant parts of the body, which may include lymph nodes or bones. Prostate cancer often spreads to the bones.",Prostate Cancer,0000036_1,CancerGov,https://www.cancer.gov/types/prostate/patient/prostate-treatment-pdq,C0376358,T191,Disorders What are the treatments for Prostate Cancer ?,0000036_1-6,treatment,"Key Points - There are different types of treatment for patients with prostate cancer. - Seven types of standard treatment are used: - Watchful waiting or active surveillance - Surgery - Radiation therapy and radiopharmaceutical therapy - Hormone therapy - Chemotherapy - Biologic therapy - Bisphosphonate therapy - There are treatments for bone pain caused by bone metastases or hormone therapy. - New types of treatment are being tested in clinical trials. - Cryosurgery - High-intensityfocused ultrasound therapy - Proton beam radiation therapy - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with prostate cancer. Different types of treatment are available for patients with prostate cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Seven types of standard treatment are used: Watchful waiting or active surveillance Watchful waiting and active surveillance are treatments used for older men who do not have signs or symptoms or have other medical conditions and for men whose prostate cancer is found during a screening test. Watchful waiting is closely monitoring a patients condition without giving any treatment until signs or symptoms appear or change. Treatment is given to relieve symptoms and improve quality of life. Active surveillance is closely following a patient's condition without giving any treatment unless there are changes in test results. It is used to find early signs that the condition is getting worse. In active surveillance, patients are given certain exams and tests, including digital rectal exam, PSA test, transrectal ultrasound, and transrectal needle biopsy, to check if the cancer is growing. When the cancer begins to grow, treatment is given to cure the cancer. Other terms that are used to describe not giving treatment to cure prostate cancer right after diagnosis are observation, watch and wait, and expectant management. Surgery Patients in good health whose tumor is in the prostate gland only may be treated with surgery to remove the tumor. The following types of surgery are used: - Radical prostatectomy: A surgical procedure to remove the prostate, surrounding tissue, and seminal vesicles. There are two types of radical prostatectomy: - Retropubic prostatectomy: A surgical procedure to remove the prostate through an incision (cut) in the abdominal wall. Removal of nearby lymph nodes may be done at the same time. - Perineal prostatectomy: A surgical procedure to remove the prostate through an incision (cut) made in the perineum (area between the scrotum and anus). Nearby lymph nodes may also be removed through a separate incision in the abdomen. - Pelvic lymphadenectomy: A surgical procedure to remove the lymph nodes in the pelvis. A pathologist views the tissue under a microscope to look for cancer cells. If the lymph nodes contain cancer, the doctor will not remove the prostate and may recommend other treatment. - Transurethral resection of the prostate (TURP): A surgical procedure to remove tissue from the prostate using a resectoscope (a thin, lighted tube with a cutting tool) inserted through the urethra. This procedure is done to treat benign prostatic hypertrophy and it is sometimes done to relieve symptoms caused by a tumor before other cancer treatment is given. TURP may also be done in men whose tumor is in the prostate only and who cannot have a radical prostatectomy. In some cases, nerve-sparing surgery can be done. This type of surgery may save the nerves that control erection. However, men with large tumors or tumors that are very close to the nerves may not be able to have this surgery. Possible problems after prostate cancer surgery include the following: - Impotence. - Leakage of urine from the bladder or stool from the rectum. - Shortening of the penis (1 to 2 centimeters). The exact reason for this is not known. - Inguinal hernia (bulging of fat or part of the small intestine through weak muscles into the groin). Inguinal hernia may occur more often in men treated with radical prostatectomy than in men who have some other types of prostate surgery, radiation therapy, or prostate biopsy alone. It is most likely to occur within the first 2 years after radical prostatectomy. Radiation therapy and radiopharmaceutical therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are different types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. Conformal radiation is a type of external radiation therapy that uses a computer to make a 3-dimensional (3-D) picture of the tumor and shapes the radiation beams to fit the tumor. This allows a high dose of radiation to reach the tumor and causes less damage to nearby healthy tissue. Hypofractionated radiation therapy may be given because it has a more convenient treatment schedule. Hypofractionated radiation therapy is radiation treatment in which a larger than usual total dose of radiation is given once a day over a shorter period of time (fewer days) compared to standard radiation therapy. Hypofractionated radiation therapy may have worse side effects than standard radiation therapy, depending on the schedules used. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. In early-stage prostate cancer, the radioactive seeds are placed in the prostate using needles that are inserted through the skin between the scrotum and rectum. The placement of the radioactive seeds in the prostate is guided by images from transrectal ultrasound or computed tomography (CT). The needles are removed after the radioactive seeds are placed in the prostate. - Radiopharmaceutical therapy uses a radioactive substance to treat cancer. Radiopharmaceutical therapy includes the following: - Alpha emitter radiation therapy uses a radioactive substance to treat prostate cancer that has spread to the bone. A radioactive substance called radium-223 is injected into a vein and travels through the bloodstream. The radium-223 collects in areas of bone with cancer and kills the cancer cells. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External radiation therapy, internal radiation therapy, and radiopharmaceutical therapy are used to treat prostate cancer. Men treated with radiation therapy for prostate cancer have an increased risk of having bladder and/or gastrointestinal cancer. Radiation therapy can cause impotence and urinary problems. Hormone therapy Hormone therapy is a cancer treatment that removes hormones or blocks their action and stops cancer cells from growing. Hormones are substances made by glands in the body and circulated in the bloodstream. In prostate cancer, male sex hormones can cause prostate cancer to grow. Drugs, surgery, or other hormones are used to reduce the amount of male hormones or block them from working. Hormone therapy for prostate cancer may include the following: - Luteinizing hormone-releasing hormone agonists can stop the testicles from making testosterone. Examples are leuprolide, goserelin, and buserelin. - Antiandrogens can block the action of androgens (hormones that promote male sex characteristics), such as testosterone. Examples are flutamide, bicalutamide, enzalutamide, and nilutamide. - Drugs that can prevent the adrenal glands from making androgens include ketoconazole and aminoglutethimide. - Orchiectomy is a surgical procedure to remove one or both testicles, the main source of male hormones, such as testosterone, to decrease the amount of hormone being made. - Estrogens (hormones that promote female sex characteristics) can prevent the testicles from making testosterone. However, estrogens are seldom used today in the treatment of prostate cancer because of the risk of serious side effects. Hot flashes, impaired sexual function, loss of desire for sex, and weakened bones may occur in men treated with hormone therapy. Other side effects include diarrhea, nausea, and itching. See Drugs Approved for Prostate Cancer for more information. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. See Drugs Approved for Prostate Cancer for more information. Biologic therapy Biologic therapy is a treatment that uses the patients immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the bodys natural defenses against cancer. Sipuleucel-T is a type of biologic therapy used to treat prostate cancer that has metastasized (spread to other parts of the body). See Drugs Approved for Prostate Cancer for more information. Bisphosphonate therapy Bisphosphonate drugs, such as clodronate or zoledronate, reduce bone disease when cancer has spread to the bone. Men who are treated with antiandrogen therapy or orchiectomy are at an increased risk of bone loss. In these men, bisphosphonate drugs lessen the risk of bone fracture (breaks). The use of bisphosphonate drugs to prevent or slow the growth of bone metastases is being studied in clinical trials. There are treatments for bone pain caused by bone metastases or hormone therapy. Prostate cancer that has spread to the bone and certain types of hormone therapy can weaken bones and lead to bone pain. Treatments for bone pain include the following: - Pain medicine. - External radiation therapy. - Strontium-89 (a radioisotope). - Targeted therapy with a monoclonal antibody, such as denosumab. - Bisphosphonate therapy. - Corticosteroids. See the PDQ summary on Pain for more information. New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Cryosurgery Cryosurgery is a treatment that uses an instrument to freeze and destroy prostate cancer cells. Ultrasound is used to find the area that will be treated. This type of treatment is also called cryotherapy. Cryosurgery can cause impotence and leakage of urine from the bladder or stool from the rectum. High-intensityfocused ultrasound therapy High-intensityfocused ultrasound therapy is a treatment that uses ultrasound (high-energy sound waves) to destroy cancer cells. To treat prostate cancer, an endorectal probe is used to make the sound waves. Proton beam radiation therapy Proton beam radiation therapy is a type of high-energy, external radiation therapy that targets tumors with streams of protons (small, positively charged particles). This type of radiation therapy is being studied in the treatment of prostate cancer. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options by Stage Stage I Prostate Cancer Standard treatment of stage I prostate cancer may include the following: - Watchful waiting. - Active surveillance. If the cancer begins to grow, hormone therapy may be given. - Radical prostatectomy, usually with pelvic lymphadenectomy. Radiation therapy may be given after surgery. - External radiation therapy. Hormone therapy may be given after radiation therapy. - Internal radiation therapy with radioactive seeds. - A clinical trial of high-intensityfocused ultrasound therapy. - A clinical trial of cryosurgery. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I prostate cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage II Prostate Cancer Standard treatment of stage II prostate cancer may include the following: - Watchful waiting. - Active surveillance. If the cancer begins to grow, hormone therapy may be given. - Radical prostatectomy, usually with pelvic lymphadenectomy. Radiation therapy may be given after surgery. - External radiation therapy. Hormone therapy may be given after radiation therapy. - Internal radiation therapy with radioactive seeds. - A clinical trial of cryosurgery. - A clinical trial of high-intensityfocused ultrasound therapy. - A clinical trial of proton beam radiation therapy. - Clinical trials of new types of treatment, such as hormone therapy followed by radical prostatectomy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage II prostate cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage III Prostate Cancer Standard treatment of stage III prostate cancer may include the following: - External radiation therapy. Hormone therapy may be given after radiation therapy. - Hormone therapy. - Radical prostatectomy. Radiation therapy may be given after surgery. - Watchful waiting. - Active surveillance. If the cancer begins to grow, hormone therapy may be given. Treatment to control cancer that is in the prostate and lessen urinary symptoms may include the following: - External radiation therapy. - Internal radiation therapy with radioactive seeds. - Hormone therapy. - Transurethral resection of the prostate (TURP). - A clinical trial of new types of radiation therapy. - A clinical trial of cryosurgery. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III prostate cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage IV Prostate Cancer Standard treatment of stage IV prostate cancer may include the following: - Hormone therapy. - Hormone therapy combined with chemotherapy. - Bisphosphonate therapy. - External radiation therapy. Hormone therapy may be given after radiation therapy. - Alpha emitter radiation therapy. - Watchful waiting. - Active surveillance. If the cancer begins to grow, hormone therapy may be given. - A clinical trial of radical prostatectomy with orchiectomy. Treatment to control cancer that is in the prostate and lessen urinary symptoms may include the following: - Transurethral resection of the prostate (TURP). - Radiation therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage IV prostate cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Prostate Cancer,0000036_1,CancerGov,https://www.cancer.gov/types/prostate/patient/prostate-treatment-pdq,C0376358,T191,Disorders what research (or clinical trials) is being done for Prostate Cancer ?,0000036_1-7,research,"New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Cryosurgery Cryosurgery is a treatment that uses an instrument to freeze and destroy prostate cancer cells. Ultrasound is used to find the area that will be treated. This type of treatment is also called cryotherapy. Cryosurgery can cause impotence and leakage of urine from the bladder or stool from the rectum. High-intensityfocused ultrasound therapy High-intensityfocused ultrasound therapy is a treatment that uses ultrasound (high-energy sound waves) to destroy cancer cells. To treat prostate cancer, an endorectal probe is used to make the sound waves. Proton beam radiation therapy Proton beam radiation therapy is a type of high-energy, external radiation therapy that targets tumors with streams of protons (small, positively charged particles). This type of radiation therapy is being studied in the treatment of prostate cancer. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Prostate Cancer,0000036_1,CancerGov,https://www.cancer.gov/types/prostate/patient/prostate-treatment-pdq,C0376358,T191,Disorders What is (are) Prostate Cancer ?,0000036_2-1,information,"Key Points - Prostate cancer is a disease in which malignant (cancer) cells form in the tissues of the prostate. - Prostate cancer is the second most common cancer among men in the United States. Prostate cancer is a disease in which malignant (cancer) cells form in the tissues of the prostate. The prostate is a gland in the male reproductive system. The prostate is just below the bladder (the organ that collects and empties urine) and in front of the rectum (the lower part of the intestine). It is about the size of a walnut and surrounds part of the urethra (the tube that empties urine from the bladder). The prostate gland produces fluid that makes up part of the semen. As men age, the prostate may get bigger. A bigger prostate may block the flow of urine from the bladder and cause problems with sexual function. This condition is called benign prostatic hyperplasia (BPH). BPH is not cancer, but surgery may be needed to correct it. The symptoms of BPH or of other problems in the prostate may be like symptoms of prostate cancer.",Prostate Cancer,0000036_2,CancerGov,https://www.cancer.gov/types/prostate/patient/prostate-prevention-pdq,C0376358,T191,Disorders Who is at risk for Prostate Cancer? ?,0000036_2-2,susceptibility,"Prostate cancer is most common in older men. In the U.S., about one out of five men will be diagnosed with prostate cancer. Most men diagnosed with prostate cancer do not die of it. See the following PDQ summaries for more information about prostate cancer: - Prostate Cancer Screening - Prostate Cancer Treatment",Prostate Cancer,0000036_2,CancerGov,https://www.cancer.gov/types/prostate/patient/prostate-prevention-pdq,C0376358,T191,Disorders How to prevent Prostate Cancer ?,0000036_2-3,prevention,"Key Points - Avoiding risk factors and increasing protective factors may help prevent cancer. - The following risk factors may increase the risk of prostate cancer: - Age - Family history of prostate cancer - Race - Hormones - Vitamin E - Folic acid - Dairy and calcium - The following protective factors may decrease the risk of prostate cancer: - Folate - Finasteride and Dutasteride - The following have been proven not to affect the risk of prostate cancer, or their effects on prostate cancer risk are not known: - Selenium and vitamin E - Diet - Multivitamins - Lycopene - Cancer prevention clinical trials are used to study ways to prevent cancer. - New ways to prevent prostate cancer are being studied in clinical trials. Avoiding risk factors and increasing protective factors may help prevent cancer. Avoiding cancer risk factors may help prevent certain cancers. Risk factors include smoking, being overweight, and not getting enough exercise. Increasing protective factors such as quitting smoking and exercising may also help prevent some cancers. Talk to your doctor or other health care professional about how you might lower your risk of cancer. The following risk factors may increase the risk of prostate cancer: Age Prostate cancer is rare in men younger than 50 years of age. The chance of developing prostate cancer increases as men get older. Family history of prostate cancer A man whose father, brother, or son has had prostate cancer has a higher-than-average risk of prostate cancer. Race Prostate cancer occurs more often in African-American men than in white men. African-American men with prostate cancer are more likely to die from the disease than white men with prostate cancer. Hormones The prostate needs male hormones to work the way it should. The main male sex hormone is testosterone. Testosterone helps the body develop and maintain male sex characteristics. Testosterone is changed into dihydrotestosterone (DHT) by an enzyme in the body. DHT is important for normal prostate growth but can also cause the prostate to get bigger and may play a part in the development of prostate cancer. Vitamin E The Selenium and Vitamin E Cancer Prevention Trial (SELECT) found that vitamin E taken alone increased the risk of prostate cancer. The risk continued even after the men stopped taking vitamin E. Folic acid Folate is a kind of vitamin B that occurs naturally in some foods, such as green vegetables, beans and orange juice. Folic acid is a man-made form of folate that is found in vitamin supplements and fortified foods, such as whole-grain breads and cereals. A 10-year study showed that the risk of prostate cancer was increased in men who took 1 milligram (mg) supplements of folic acid. However, the risk of prostate cancer was lower in men who had enough folate in their diets. Dairy and calcium A diet high in dairy foods and calcium may cause a small increase in the risk of prostate cancer. The following protective factors may decrease the risk of prostate cancer: Folate Folate is a kind of vitamin B that occurs naturally in some foods, such as green vegetables, beans and orange juice. Folic acid is a man-made form of folate that is found in vitamin supplements and fortified foods, such as whole-grain breads and cereals. A 10-year study showed that the risk of prostate cancer was lower in men who had enough folate in their diets. However, the risk of prostate cancer was increased in men who took 1 milligram (mg) supplements of folic acid. Finasteride and Dutasteride Finasteride and dutasteride are drugs used to lower the amount of male sex hormones made by the body. These drugs block the enzyme that changes testosterone into dihydrotestosterone (DHT). Higher than normal levels of DHT may play a part in developing prostate cancer. Taking finasteride or dutasteride has been shown to lower the risk for prostate cancer, but it is not known if these drugs lower the risk of death from prostate cancer. The Prostate Cancer Prevention Trial (PCPT) studied whether the drug finasteride can prevent prostate cancer in healthy men 55 years of age and older. This prevention study showed there were fewer prostate cancers in the group of men that took finasteride compared with the group of men that did not. Also, the men who took finasteride who did have prostate cancer had more aggressive tumors. The number of deaths from prostate cancer was the same in both groups. Men who took finasteride reported more side effects compared with the group of men that did not, including erectile dysfunction, loss of desire for sex, and enlarged breasts. The Reduction by Dutasteride of Prostate Cancer Events Trial (REDUCE) studied whether the drug dutasteride can prevent prostate cancer in men aged 50 to 75 years at higher risk for the disease. This prevention study showed there were fewer prostate cancers in the group of men who took dutasteride compared with the group of men that did not. The number of less aggressive prostate cancers was lower, but the number of more aggressive prostate cancers was not. Men who took dutasteride reported more side effects than men who did not, including erectile dysfunction, loss of desire for sex, less semen, and gynecomastia (enlarged breasts). The following have been proven not to affect the risk of prostate cancer, or their effects on prostate cancer risk are not known: Selenium and vitamin E The Selenium and Vitamin E Cancer Prevention Trial (SELECT) studied whether taking vitamin E and selenium (a mineral) will prevent prostate cancer. The selenium and vitamin E were taken separately or together by healthy men 55 years of age and older (50 years of age and older for African-American men). The study showed that taking selenium alone or selenium and vitamin E together did not decrease the risk of prostate cancer. Diet It is not known if decreasing fat or increasing fruits and vegetables in the diet helps decrease the risk of prostate cancer or death from prostate cancer. In the PCPT trial, certain fatty acids increased the risk of high-grade prostate cancer while others decreased the risk of high-grade prostate cancer. Multivitamins Regular use of multivitamins has not been proven to increase the risk of early or localized prostate cancer. However, a large study showed an increased risk of advanced prostate cancer among men who took multivitamins more than seven times a week. Lycopene Some studies have shown that a diet high in lycopene may be linked to a decreased risk of prostate cancer, but other studies have not. It has not been proven that taking lycopene supplements decreases the risk of prostate cancer. Cancer prevention clinical trials are used to study ways to prevent cancer. Cancer prevention clinical trials are used to study ways to lower the risk of developing certain types of cancer. Some cancer prevention trials are conducted with healthy people who have not had cancer but who have an increased risk for cancer. Other prevention trials are conducted with people who have had cancer and are trying to prevent another cancer of the same type or to lower their chance of developing a new type of cancer. Other trials are done with healthy volunteers who are not known to have any risk factors for cancer. The purpose of some cancer prevention clinical trials is to find out whether actions people take can prevent cancer. These may include eating fruits and vegetables, exercising, quitting smoking, or taking certain medicines, vitamins, minerals, or food supplements. New ways to prevent prostate cancer are being studied in clinical trials. Clinical trials are taking place in many parts of the country. Information about clinical trials can be found in the Clinical Trials section of the NCI website. Check NCI's list of cancer clinical trials for prostate cancer prevention trials that are accepting patients.",Prostate Cancer,0000036_2,CancerGov,https://www.cancer.gov/types/prostate/patient/prostate-prevention-pdq,C0376358,T191,Disorders Who is at risk for Prostate Cancer? ?,0000036_2-4,susceptibility,"Avoiding risk factors and increasing protective factors may help prevent cancer. Avoiding cancer risk factors may help prevent certain cancers. Risk factors include smoking, being overweight, and not getting enough exercise. Increasing protective factors such as quitting smoking and exercising may also help prevent some cancers. Talk to your doctor or other health care professional about how you might lower your risk of cancer. The following risk factors may increase the risk of prostate cancer: Age Prostate cancer is rare in men younger than 50 years of age. The chance of developing prostate cancer increases as men get older. Family history of prostate cancer A man whose father, brother, or son has had prostate cancer has a higher-than-average risk of prostate cancer. Race Prostate cancer occurs more often in African-American men than in white men. African-American men with prostate cancer are more likely to die from the disease than white men with prostate cancer. Hormones The prostate needs male hormones to work the way it should. The main male sex hormone is testosterone. Testosterone helps the body develop and maintain male sex characteristics. Testosterone is changed into dihydrotestosterone (DHT) by an enzyme in the body. DHT is important for normal prostate growth but can also cause the prostate to get bigger and may play a part in the development of prostate cancer. Vitamin E The Selenium and Vitamin E Cancer Prevention Trial (SELECT) found that vitamin E taken alone increased the risk of prostate cancer. The risk continued even after the men stopped taking vitamin E. Folic acid Folate is a kind of vitamin B that occurs naturally in some foods, such as green vegetables, beans and orange juice. Folic acid is a man-made form of folate that is found in vitamin supplements and fortified foods, such as whole-grain breads and cereals. A 10-year study showed that the risk of prostate cancer was increased in men who took 1 milligram (mg) supplements of folic acid. However, the risk of prostate cancer was lower in men who had enough folate in their diets. Dairy and calcium A diet high in dairy foods and calcium may cause a small increase in the risk of prostate cancer. The following protective factors may decrease the risk of prostate cancer: Folate Folate is a kind of vitamin B that occurs naturally in some foods, such as green vegetables, beans and orange juice. Folic acid is a man-made form of folate that is found in vitamin supplements and fortified foods, such as whole-grain breads and cereals. A 10-year study showed that the risk of prostate cancer was lower in men who had enough folate in their diets. However, the risk of prostate cancer was increased in men who took 1 milligram (mg) supplements of folic acid. Finasteride and Dutasteride Finasteride and dutasteride are drugs used to lower the amount of male sex hormones made by the body. These drugs block the enzyme that changes testosterone into dihydrotestosterone (DHT). Higher than normal levels of DHT may play a part in developing prostate cancer. Taking finasteride or dutasteride has been shown to lower the risk for prostate cancer, but it is not known if these drugs lower the risk of death from prostate cancer. The Prostate Cancer Prevention Trial (PCPT) studied whether the drug finasteride can prevent prostate cancer in healthy men 55 years of age and older. This prevention study showed there were fewer prostate cancers in the group of men that took finasteride compared with the group of men that did not. Also, the men who took finasteride who did have prostate cancer had more aggressive tumors. The number of deaths from prostate cancer was the same in both groups. Men who took finasteride reported more side effects compared with the group of men that did not, including erectile dysfunction, loss of desire for sex, and enlarged breasts. The Reduction by Dutasteride of Prostate Cancer Events Trial (REDUCE) studied whether the drug dutasteride can prevent prostate cancer in men aged 50 to 75 years at higher risk for the disease. This prevention study showed there were fewer prostate cancers in the group of men who took dutasteride compared with the group of men that did not. The number of less aggressive prostate cancers was lower, but the number of more aggressive prostate cancers was not. Men who took dutasteride reported more side effects than men who did not, including erectile dysfunction, loss of desire for sex, less semen, and gynecomastia (enlarged breasts). The following have been proven not to affect the risk of prostate cancer, or their effects on prostate cancer risk are not known: Selenium and vitamin E The Selenium and Vitamin E Cancer Prevention Trial (SELECT) studied whether taking vitamin E and selenium (a mineral) will prevent prostate cancer. The selenium and vitamin E were taken separately or together by healthy men 55 years of age and older (50 years of age and older for African-American men). The study showed that taking selenium alone or selenium and vitamin E together did not decrease the risk of prostate cancer. Diet It is not known if decreasing fat or increasing fruits and vegetables in the diet helps decrease the risk of prostate cancer or death from prostate cancer. In the PCPT trial, certain fatty acids increased the risk of high-grade prostate cancer while others decreased the risk of high-grade prostate cancer. Multivitamins Regular use of multivitamins has not been proven to increase the risk of early or localized prostate cancer. However, a large study showed an increased risk of advanced prostate cancer among men who took multivitamins more than seven times a week. Lycopene Some studies have shown that a diet high in lycopene may be linked to a decreased risk of prostate cancer, but other studies have not. It has not been proven that taking lycopene supplements decreases the risk of prostate cancer.",Prostate Cancer,0000036_2,CancerGov,https://www.cancer.gov/types/prostate/patient/prostate-prevention-pdq,C0376358,T191,Disorders what research (or clinical trials) is being done for Prostate Cancer ?,0000036_2-5,research,"Cancer prevention clinical trials are used to study ways to prevent cancer. Cancer prevention clinical trials are used to study ways to lower the risk of developing certain types of cancer. Some cancer prevention trials are conducted with healthy people who have not had cancer but who have an increased risk for cancer. Other prevention trials are conducted with people who have had cancer and are trying to prevent another cancer of the same type or to lower their chance of developing a new type of cancer. Other trials are done with healthy volunteers who are not known to have any risk factors for cancer. The purpose of some cancer prevention clinical trials is to find out whether actions people take can prevent cancer. These may include eating fruits and vegetables, exercising, quitting smoking, or taking certain medicines, vitamins, minerals, or food supplements. New ways to prevent prostate cancer are being studied in clinical trials. Clinical trials are taking place in many parts of the country. Information about clinical trials can be found in the Clinical Trials section of the NCI website. Check NCI's list of cancer clinical trials for prostate cancer prevention trials that are accepting patients.",Prostate Cancer,0000036_2,CancerGov,https://www.cancer.gov/types/prostate/patient/prostate-prevention-pdq,C0376358,T191,Disorders What is (are) Prostate Cancer ?,0000036_3-1,information,"Key Points - Prostate cancer is a disease in which malignant (cancer) cells form in the tissues of the prostate. - Prostate cancer is the most common nonskin cancer among men in the United States. - Different factors increase or decrease the risk of developing prostate cancer. Prostate cancer is a disease in which malignant (cancer) cells form in the tissues of the prostate. The prostate is a gland in the male reproductive system located just below the bladder (the organ that collects and empties urine) and in front of the rectum (the lower part of the intestine). It is about the size of a walnut and surrounds part of the urethra (the tube that empties urine from the bladder). The prostate gland produces fluid that makes up part of semen. As men age, the prostate may get bigger. A bigger prostate may block the flow of urine from the bladder and cause problems with sexual function. This condition is called benign prostatic hyperplasia (BPH), and although it is not cancer, surgery may be needed to correct it. The symptoms of benign prostatic hyperplasia or of other problems in the prostate may be similar to symptoms of prostate cancer. See the following PDQ summaries for more information about prostate cancer: - Prostate Cancer Prevention - Prostate Cancer Treatment",Prostate Cancer,0000036_3,CancerGov,https://www.cancer.gov/types/prostate/patient/prostate-screening-pdq,C0376358,T191,Disorders Who is at risk for Prostate Cancer? ?,0000036_3-2,susceptibility,"Prostate cancer is the most common nonskin cancer among men in the United States. Prostate cancer is found mainly in older men. Although the number of men with prostate cancer is large, most men diagnosed with this disease do not die from it. Prostate cancer causes more deaths in men than any other cancer except lung cancer and colorectal cancer. Prostate cancer occurs more often in African-American men than in white men. African-American men with prostate cancer are more likely to die from the disease than white men with prostate cancer.",Prostate Cancer,0000036_3,CancerGov,https://www.cancer.gov/types/prostate/patient/prostate-screening-pdq,C0376358,T191,Disorders Who is at risk for Prostate Cancer? ?,0000036_3-3,susceptibility,"Different factors increase or decrease the risk of developing prostate cancer. Anything that increases a person's chance of developing a disease is called a risk factor. Anything that decreases your chance of getting a disease is called a protective factor. For information about risk factors and protective factors for prostate cancer, see the PDQ summary on Prostate Cancer Prevention.",Prostate Cancer,0000036_3,CancerGov,https://www.cancer.gov/types/prostate/patient/prostate-screening-pdq,C0376358,T191,Disorders What is (are) Colon Cancer ?,0000037_1-1,information,"Key Points - Colon cancer is a disease in which malignant (cancer) cells form in the tissues of the colon. - Health history affects the risk of developing colon cancer. - Signs of colon cancer include blood in the stool or a change in bowel habits. - Tests that examine the colon and rectum are used to detect (find) and diagnose colon cancer. - Certain factors affect prognosis (chance of recovery) and treatment options. Colon cancer is a disease in which malignant (cancer) cells form in the tissues of the colon. The colon is part of the bodys digestive system. The digestive system removes and processes nutrients (vitamins, minerals, carbohydrates, fats, proteins, and water) from foods and helps pass waste material out of the body. The digestive system is made up of the esophagus, stomach, and the small and large intestines. The colon (large bowel) is the first part of the large intestine and is about 5 feet long. Together, the rectum and anal canal make up the last part of the large intestine and are about 6-8 inches long. The anal canal ends at the anus (the opening of the large intestine to the outside of the body). Gastrointestinal stromal tumors can occur in the colon. See the PDQ summary on Gastrointestinal Stromal Tumors Treatment for more information. See the PDQ summary about Unusual Cancers of Childhood Treatment for information about colorectal cancer in children.",Colon Cancer,0000037_1,CancerGov,https://www.cancer.gov/types/colorectal/patient/colon-treatment-pdq,C0346629,T191,Disorders Who is at risk for Colon Cancer? ?,0000037_1-2,susceptibility,"Health history affects the risk of developing colon cancer. Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesnt mean that you will not get cancer. Talk to your doctor if you think you may be at risk for colorectal cancer. Risk factors for colorectal cancer include the following: - Having a family history of colon or rectal cancer in a first-degree relative (parent, sibling, or child). - Having a personal history of cancer of the colon, rectum, or ovary. - Having a personal history of high-risk adenomas (colorectal polyps that are 1 centimeter or larger in size or that have cells that look abnormal under a microscope). - Having inherited changes in certain genes that increase the risk of familial adenomatous polyposis (FAP) or Lynch syndrome (hereditary nonpolyposis colorectal cancer). - Having a personal history of chronic ulcerative colitis or Crohn disease for 8 years or more. - Having three or more alcoholic drinks per day. - Smoking cigarettes. - Being black. - Being obese. Older age is a main risk factor for most cancers. The chance of getting cancer increases as you get older.",Colon Cancer,0000037_1,CancerGov,https://www.cancer.gov/types/colorectal/patient/colon-treatment-pdq,C0346629,T191,Disorders What are the symptoms of Colon Cancer ?,0000037_1-3,symptoms,"Signs of colon cancer include blood in the stool or a change in bowel habits. These and other signs and symptoms may be caused by colon cancer or by other conditions. Check with your doctor if you have any of the following: - A change in bowel habits. - Blood (either bright red or very dark) in the stool. - Diarrhea, constipation, or feeling that the bowel does not empty all the way. - Stools that are narrower than usual. - Frequent gas pains, bloating, fullness, or cramps. - Weight loss for no known reason. - Feeling very tired. - Vomiting.",Colon Cancer,0000037_1,CancerGov,https://www.cancer.gov/types/colorectal/patient/colon-treatment-pdq,C0346629,T191,Disorders How to diagnose Colon Cancer ?,0000037_1-4,exams and tests,"Tests that examine the colon and rectum are used to detect (find) and diagnose colon cancer. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Digital rectal exam : An exam of the rectum. The doctor or nurse inserts a lubricated, gloved finger into the rectum to feel for lumps or anything else that seems unusual. - Fecal occult blood test (FOBT): A test to check stool (solid waste) for blood that can only be seen with a microscope. A small sample of stool is placed on a special card or in a special container and returned to the doctor or laboratory for testing. Blood in the stool may be a sign of polyps, cancer, or other conditions. There are two types of FOBTs: - Guaiac FOBT : The sample of stool on the special card is tested with a chemical. If there is blood in the stool, the special card changes color. - Immunochemical FOBT : A liquid is added to the stool sample. This mixture is injected into a machine that contains antibodies that can detect blood in the stool. If there is blood in the stool, a line appears in a window in the machine. This test is also called fecal immunochemical test or FIT. - Barium enema : A series of x-rays of the lower gastrointestinal tract. A liquid that contains barium (a silver-white metallic compound) is put into the rectum. The barium coats the lower gastrointestinal tract and x-rays are taken. This procedure is also called a lower GI series. - Sigmoidoscopy : A procedure to look inside the rectum and sigmoid (lower) colon for polyps (small areas of bulging tissue), other abnormal areas, or cancer. A sigmoidoscope is inserted through the rectum into the sigmoid colon. A sigmoidoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove polyps or tissue samples, which are checked under a microscope for signs of cancer. - Colonoscopy : A procedure to look inside the rectum and colon for polyps, abnormal areas, or cancer. A colonoscope is inserted through the rectum into the colon. A colonoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove polyps or tissue samples, which are checked under a microscope for signs of cancer. - Virtual colonoscopy : A procedure that uses a series of x-rays called computed tomography to make a series of pictures of the colon. A computer puts the pictures together to create detailed images that may show polyps and anything else that seems unusual on the inside surface of the colon. This test is also called colonography or CT colonography. - Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer.",Colon Cancer,0000037_1,CancerGov,https://www.cancer.gov/types/colorectal/patient/colon-treatment-pdq,C0346629,T191,Disorders What is the outlook for Colon Cancer ?,0000037_1-5,outlook,"Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - The stage of the cancer (whether the cancer is in the inner lining of the colon only or has spread through the colon wall, or has spread to lymph nodes or other places in the body). - Whether the cancer has blocked or made a hole in the colon. - Whether there are any cancer cells left after surgery. - Whether the cancer has recurred. - The patients general health. The prognosis also depends on the blood levels of carcinoembryonic antigen (CEA) before treatment begins. CEA is a substance in the blood that may be increased when cancer is present.",Colon Cancer,0000037_1,CancerGov,https://www.cancer.gov/types/colorectal/patient/colon-treatment-pdq,C0346629,T191,Disorders What are the stages of Colon Cancer ?,0000037_1-6,stages,"Key Points - After colon cancer has been diagnosed, tests are done to find out if cancer cells have spread within the colon or to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - The following stages are used for colon cancer: - Stage 0 (Carcinoma in Situ) - Stage I - Stage II - Stage III - Stage IV After colon cancer has been diagnosed, tests are done to find out if cancer cells have spread within the colon or to other parts of the body. The process used to find out if cancer has spread within the colon or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following tests and procedures may be used in the staging process: - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the abdomen or chest, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the colon. A substance called gadolinium is injected into the patient through a vein. The gadolinium collects around the cancer cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI). - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. - Chest x-ray : An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. - Surgery : A procedure to remove the tumor and see how far it has spread through the colon. - Lymph node biopsy : The removal of all or part of a lymph node. A pathologist views the tissue under a microscope to look for cancer cells. - Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following: - The number of red blood cells, white blood cells, and platelets. - The amount of hemoglobin (the protein that carries oxygen) in the red blood cells. - The portion of the blood sample made up of red blood cells. - Carcinoembryonic antigen (CEA) assay : A test that measures the level of CEA in the blood. CEA is released into the bloodstream from both cancer cells and normal cells. When found in higher than normal amounts, it can be a sign of colon cancer or other conditions. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if colon cancer spreads to the lung, the cancer cells in the lung are actually colon cancer cells. The disease is metastatic colon cancer, not lung cancer. The following stages are used for colon cancer: Stage 0 (Carcinoma in Situ) In stage 0, abnormal cells are found in the mucosa (innermost layer) of the colon wall. These abnormal cells may become cancer and spread. Stage 0 is also called carcinoma in situ. Stage I In stage I, cancer has formed in the mucosa (innermost layer) of the colon wall and has spread to the submucosa (layer of tissue under the mucosa). Cancer may have spread to the muscle layer of the colon wall. Stage II Stage II colon cancer is divided into stage IIA, stage IIB, and stage IIC. - Stage IIA: Cancer has spread through the muscle layer of the colon wall to the serosa (outermost layer) of the colon wall. - Stage IIB: Cancer has spread through the serosa (outermost layer) of the colon wall but has not spread to nearby organs. - Stage IIC: Cancer has spread through the serosa (outermost layer) of the colon wall to nearby organs. Stage III Stage III colon cancer is divided into stage IIIA, stage IIIB, and stage IIIC. In stage IIIA: - Cancer has spread through the mucosa (innermost layer) of the colon wall to the submucosa (layer of tissue under the mucosa) and may have spread to the muscle layer of the colon wall. Cancer has spread to at least one but not more than 3 nearby lymph nodes or cancer cells have formed in tissues near the lymph nodes; or - Cancer has spread through the mucosa (innermost layer) of the colon wall to the submucosa (layer of tissue under the mucosa). Cancer has spread to at least 4 but not more than 6 nearby lymph nodes. In stage IIIB: - Cancer has spread through the muscle layer of the colon wall to the serosa (outermost layer) of the colon wall or has spread through the serosa but not to nearby organs. Cancer has spread to at least one but not more than 3 nearby lymph nodes or cancer cells have formed in tissues near the lymph nodes; or - Cancer has spread to the muscle layer of the colon wall or to the serosa (outermost layer) of the colon wall. Cancer has spread to at least 4 but not more than 6 nearby lymph nodes; or - Cancer has spread through the mucosa (innermost layer) of the colon wall to the submucosa (layer of tissue under the mucosa) and may have spread to the muscle layer of the colon wall. Cancer has spread to 7 or more nearby lymph nodes. In stage IIIC: - Cancer has spread through the serosa (outermost layer) of the colon wall but has not spread to nearby organs. Cancer has spread to at least 4 but not more than 6 nearby lymph nodes; or - Cancer has spread through the muscle layer of the colon wall to the serosa (outermost layer) of the colon wall or has spread through the serosa but has not spread to nearby organs. Cancer has spread to 7 or more nearby lymph nodes; or - Cancer has spread through the serosa (outermost layer) of the colon wall and has spread to nearby organs. Cancer has spread to one or more nearby lymph nodes or cancer cells have formed in tissues near the lymph nodes. Stage IV Stage IV colon cancer is divided into stage IVA and stage IVB. - Stage IVA: Cancer may have spread through the colon wall and may have spread to nearby organs or lymph nodes. Cancer has spread to one organ that is not near the colon, such as the liver, lung, or ovary, or to a distant lymph node. - Stage IVB: Cancer may have spread through the colon wall and may have spread to nearby organs or lymph nodes. Cancer has spread to more than one organ that is not near the colon or into the lining of the abdominal wall.",Colon Cancer,0000037_1,CancerGov,https://www.cancer.gov/types/colorectal/patient/colon-treatment-pdq,C0346629,T191,Disorders What are the treatments for Colon Cancer ?,0000037_1-7,treatment,"Key Points - There are different types of treatment for patients with colon cancer. - Six types of standard treatment are used: - Surgery - Radiofrequency ablation - Cryosurgery - Chemotherapy - Radiation therapy - Targeted therapy - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with colon cancer. Different types of treatment are available for patients with colon cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Six types of standard treatment are used: Surgery Surgery (removing the cancer in an operation) is the most common treatment for all stages of colon cancer. A doctor may remove the cancer using one of the following types of surgery: - Local excision: If the cancer is found at a very early stage, the doctor may remove it without cutting through the abdominal wall. Instead, the doctor may put a tube with a cutting tool through the rectum into the colon and cut the cancer out. This is called a local excision. If the cancer is found in a polyp (a small bulging area of tissue), the operation is called a polypectomy. - Resection of the colon with anastomosis: If the cancer is larger, the doctor will perform a partial colectomy (removing the cancer and a small amount of healthy tissue around it). The doctor may then perform an anastomosis (sewing the healthy parts of the colon together). The doctor will also usually remove lymph nodes near the colon and examine them under a microscope to see whether they contain cancer. - Resection of the colon with colostomy: If the doctor is not able to sew the 2 ends of the colon back together, a stoma (an opening) is made on the outside of the body for waste to pass through. This procedure is called a colostomy. A bag is placed around the stoma to collect the waste. Sometimes the colostomy is needed only until the lower colon has healed, and then it can be reversed. If the doctor needs to remove the entire lower colon, however, the colostomy may be permanent. Even if the doctor removes all the cancer that can be seen at the time of the operation, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiofrequency ablation Radiofrequency ablation is the use of a special probe with tiny electrodes that kill cancer cells. Sometimes the probe is inserted directly through the skin and only local anesthesia is needed. In other cases, the probe is inserted through an incision in the abdomen. This is done in the hospital with general anesthesia. Cryosurgery Cryosurgery is a treatment that uses an instrument to freeze and destroy abnormal tissue. This type of treatment is also called cryotherapy. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Chemoembolization of the hepatic artery may be used to treat cancer that has spread to the liver. This involves blocking the hepatic artery (the main artery that supplies blood to the liver) and injecting anticancer drugs between the blockage and the liver. The livers arteries then deliver the drugs throughout the liver. Only a small amount of the drug reaches other parts of the body. The blockage may be temporary or permanent, depending on what is used to block the artery. The liver continues to receive some blood from the hepatic portal vein, which carries blood from the stomach and intestine. The way the chemotherapy is given depends on the type and stage of the cancer being treated. See Drugs Approved for Colon and Rectal Cancer for more information. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External radiation therapy is used as palliative therapy to relieve symptoms and improve quality of life. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Types of targeted therapies used in the treatment of colon cancer include the following: - Monoclonal antibodies: Monoclonal antibodies are made in the laboratory from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. - Bevacizumab and ramucirumab are types of monoclonal antibodies that bind to a protein called vascular endothelial growth factor (VEGF). This may prevent the growth of new blood vessels that tumors need to grow. - Cetuximab and panitumumab are types of monoclonal antibodies that bind to a protein called epidermal growth factor receptor (EGFR) on the surface of some types of cancer cells. This may stop cancer cells from growing and dividing. - Angiogenesis inhibitors: Angiogenesis inhibitors stop the growth of new blood vessels that tumors need to grow. - Ziv-aflibercept is a vascular endothelial growth factor trap that blocks an enzyme needed for the growth of new blood vessels in tumors. - Regorafenib is used to treat colorectal cancer that has spread to other parts of the body and has not gotten better with other treatment. It blocks the action of certain proteins, including vascular endothelial growth factor. This may help keep cancer cells from growing and may kill them. It may also prevent the growth of new blood vessels that tumors need to grow. See Drugs Approved for Colon and Rectal Cancer for more information. New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Colon Cancer Stage 0 (Carcinoma in Situ) Treatment of stage 0 (carcinoma in situ) may include the following types of surgery: - Local excision or simple polypectomy. - Resection and anastomosis. This is done when the tumor is too large to remove by local excision. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage 0 colon cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage I Colon Cancer Treatment of stage I colon cancer usually includes the following: - Resection and anastomosis. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I colon cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage II Colon Cancer Treatment of stage II colon cancer may include the following: - Resection and anastomosis. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage II colon cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage III Colon Cancer Treatment of stage III colon cancer may include the following: - Resection and anastomosis which may be followed by chemotherapy. - Clinical trials of new chemotherapy regimens after surgery. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III colon cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage IV and Recurrent Colon Cancer Treatment of stage IV and recurrent colon cancer may include the following: - Local excision for tumors that have recurred. - Resection with or without anastomosis. - Surgery to remove parts of other organs, such as the liver, lungs, and ovaries, where the cancer may have recurred or spread. Treatment of cancer that has spread to the liver may also include the following: - Chemotherapy given before surgery to shrink the tumor, after surgery, or both before and after. - Radiofrequency ablation or cryosurgery, for patients who cannot have surgery. - Chemoembolization of the hepatic artery. - Radiation therapy or chemotherapy may be offered to some patients as palliative therapy to relieve symptoms and improve quality of life. - Chemotherapy and/or targeted therapy with a monoclonal antibody or an angiogenesis inhibitor. - Clinical trials of chemotherapy and/or targeted therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage IV colon cancer and recurrent colon cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Colon Cancer,0000037_1,CancerGov,https://www.cancer.gov/types/colorectal/patient/colon-treatment-pdq,C0346629,T191,Disorders what research (or clinical trials) is being done for Colon Cancer ?,0000037_1-8,research,"New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Colon Cancer,0000037_1,CancerGov,https://www.cancer.gov/types/colorectal/patient/colon-treatment-pdq,C0346629,T191,Disorders What is (are) Rectal Cancer ?,0000037_2-1,information,"Key Points - Rectal cancer is a disease in which malignant (cancer) cells form in the tissues of the rectum. - Health history affects the risk of developing rectal cancer. - Signs of rectal cancer include a change in bowel habits or blood in the stool. - Tests that examine the rectum and colon are used to detect (find) and diagnose rectal cancer. - Certain factors affect prognosis (chance of recovery) and treatment options. Rectal cancer is a disease in which malignant (cancer) cells form in the tissues of the rectum. The rectum is part of the bodys digestive system. The digestive system takes in nutrients (vitamins, minerals, carbohydrates, fats, proteins, and water) from foods and helps pass waste material out of the body. The digestive system is made up of the esophagus, stomach, and the small and large intestines. The colon (large bowel) is the first part of the large intestine and is about 5 feet long. Together, the rectum and anal canal make up the last part of the large intestine and are 6-8 inches long. The anal canal ends at the anus (the opening of the large intestine to the outside of the body). See the following PDQ summaries for more information about rectal cancer: - Unusual Cancers of Childhood Treatment (see Colorectal Cancer section) - Colorectal Cancer Prevention - Colorectal Cancer Screening - Gastrointestinal Stromal Tumors Treatment - Genetics of Colorectal Cancer",Rectal Cancer,0000037_2,CancerGov,https://www.cancer.gov/types/colorectal/patient/rectal-treatment-pdq,C0007113,T191,Disorders Who is at risk for Rectal Cancer? ?,0000037_2-2,susceptibility,"Health history affects the risk of developing rectal cancer. Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesnt mean that you will not get cancer. Talk to your doctor if you think you may be at risk for colorectal cancer. Risk factors for colorectal cancer include the following: - Having a family history of colon or rectal cancer in a first-degree relative (parent, sibling, or child). - Having a personal history of cancer of the colon, rectum, or ovary. - Having a personal history of high-risk adenomas (colorectal polyps that are 1 centimeter or larger in size or that have cells that look abnormal under a microscope). - Having inherited changes in certain genes that increase the risk of familial adenomatous polyposis (FAP) or Lynch syndrome (hereditary nonpolyposis colorectal cancer). - Having a personal history of chronic ulcerative colitis or Crohn disease for 8 years or more. - Having three or more alcoholic drinks per day. - Smoking cigarettes. - Being black. - Being obese. Older age is a main risk factor for most cancers. The chance of getting cancer increases as you get older.",Rectal Cancer,0000037_2,CancerGov,https://www.cancer.gov/types/colorectal/patient/rectal-treatment-pdq,C0007113,T191,Disorders What are the symptoms of Rectal Cancer ?,0000037_2-3,symptoms,"Signs of rectal cancer include a change in bowel habits or blood in the stool. These and other signs and symptoms may be caused by rectal cancer or by other conditions. Check with your doctor if you have any of the following: - Blood (either bright red or very dark) in the stool. - A change in bowel habits. - Diarrhea. - Constipation. - Feeling that the bowel does not empty completely. - Stools that are narrower or have a different shape than usual. - General abdominal discomfort (frequent gas pains, bloating, fullness, or cramps). - Change in appetite. - Weight loss for no known reason. - Feeling very tired.",Rectal Cancer,0000037_2,CancerGov,https://www.cancer.gov/types/colorectal/patient/rectal-treatment-pdq,C0007113,T191,Disorders How to diagnose Rectal Cancer ?,0000037_2-4,exams and tests,"Tests that examine the rectum and colon are used to detect (find) and diagnose rectal cancer. Tests used to diagnose rectal cancer include the following: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Digital rectal exam (DRE): An exam of the rectum. The doctor or nurse inserts a lubricated, gloved finger into the lower part of the rectum to feel for lumps or anything else that seems unusual. In women, the vagina may also be examined. - Colonoscopy : A procedure to look inside the rectum and colon for polyps (small pieces of bulging tissue), abnormal areas, or cancer. A colonoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove polyps or tissue samples, which are checked under a microscope for signs of cancer. - Biopsy : The removal of cells or tissues so they can be viewed under a microscope to check for signs of cancer. Tumor tissue that is removed during the biopsy may be checked to see if the patient is likely to have the gene mutation that causes HNPCC. This may help to plan treatment. The following tests may be used: - Reverse transcriptionpolymerase chain reaction (RTPCR) test: A laboratory test in which cells in a sample of tissue are studied using chemicals to look for certain changes in the structure or function of genes. - Immunohistochemistry : A test that uses antibodies to check for certain antigens in a sample of tissue. The antibody is usually linked to a radioactive substance or a dye that causes the tissue to light up under a microscope. This type of test may be used to tell the difference between different types of cancer. - Carcinoembryonic antigen (CEA) assay : A test that measures the level of CEA in the blood. CEA is released into the bloodstream from both cancer cells and normal cells. When found in higher than normal amounts, it can be a sign of rectal cancer or other conditions.",Rectal Cancer,0000037_2,CancerGov,https://www.cancer.gov/types/colorectal/patient/rectal-treatment-pdq,C0007113,T191,Disorders What is the outlook for Rectal Cancer ?,0000037_2-5,outlook,"Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - The stage of the cancer (whether it affects the inner lining of the rectum only, involves the whole rectum, or has spread to lymph nodes, nearby organs, or other places in the body). - Whether the tumor has spread into or through the bowel wall. - Where the cancer is found in the rectum. - Whether the bowel is blocked or has a hole in it. - Whether all of the tumor can be removed by surgery. - The patients general health. - Whether the cancer has just been diagnosed or has recurred (come back).",Rectal Cancer,0000037_2,CancerGov,https://www.cancer.gov/types/colorectal/patient/rectal-treatment-pdq,C0007113,T191,Disorders What are the stages of Rectal Cancer ?,0000037_2-6,stages,"Key Points - After rectal cancer has been diagnosed, tests are done to find out if cancer cells have spread within the rectum or to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - The following stages are used for rectal cancer: - Stage 0 (Carcinoma in Situ) - Stage I - Stage II - Stage III - Stage IV After rectal cancer has been diagnosed, tests are done to find out if cancer cells have spread within the rectum or to other parts of the body. The process used to find out whether cancer has spread within the rectum or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following tests and procedures may be used in the staging process: - Chest x-ray : An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. - Colonoscopy : A procedure to look inside the rectum and colon for polyps (small pieces of bulging tissue). abnormal areas, or cancer. A colonoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove polyps or tissue samples, which are checked under a microscope for signs of cancer. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the abdomen, pelvis, or chest, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. - Endorectal ultrasound : A procedure used to examine the rectum and nearby organs. An ultrasound transducer (probe) is inserted into the rectum and used to bounce high-energy sound waves (ultrasound) off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The doctor can identify tumors by looking at the sonogram. This procedure is also called transrectal ultrasound. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if rectal cancer spreads to the lung, the cancer cells in the lung are actually rectal cancer cells. The disease is metastatic rectal cancer, not lung cancer. The following stages are used for rectal cancer: Stage 0 (Carcinoma in Situ) In stage 0, abnormal cells are found in the mucosa (innermost layer) of the rectum wall. These abnormal cells may become cancer and spread. Stage 0 is also called carcinoma in situ. Stage I In stage I, cancer has formed in the mucosa (innermost layer) of the rectum wall and has spread to the submucosa (layer of tissue under the mucosa). Cancer may have spread to the muscle layer of the rectum wall. Stage II Stage II rectal cancer is divided into stage IIA, stage IIB, and stage IIC. - Stage IIA: Cancer has spread through the muscle layer of the rectum wall to the serosa (outermost layer) of the rectum wall. - Stage IIB: Cancer has spread through the serosa (outermost layer) of the rectum wall but has not spread to nearby organs. - Stage IIC: Cancer has spread through the serosa (outermost layer) of the rectum wall to nearby organs. Stage III Stage III rectal cancer is divided into stage IIIA, stage IIIB, and stage IIIC. In stage IIIA: - Cancer has spread through the mucosa (innermost layer) of the rectum wall to the submucosa (layer of tissue under the mucosa) and may have spread to the muscle layer of the rectum wall. Cancer has spread to at least one but not more than 3 nearby lymph nodes or cancer cells have formed in tissues near the lymph nodes; or - Cancer has spread through the mucosa (innermost layer) of the rectum wall to the submucosa (layer of tissue under the mucosa). Cancer has spread to at least 4 but not more than 6 nearby lymph nodes. In stage IIIB: - Cancer has spread through the muscle layer of the rectum wall to the serosa (outermost layer) of the rectum wall or has spread through the serosa but not to nearby organs. Cancer has spread to at least one but not more than 3 nearby lymph nodes or cancer cells have formed in tissues near the lymph nodes; or - Cancer has spread to the muscle layer of the rectum wall or to the serosa (outermost layer) of the rectum wall. Cancer has spread to at least 4 but not more than 6 nearby lymph nodes; or - Cancer has spread through the mucosa (innermost layer) of the rectum wall to the submucosa (layer of tissue under the mucosa) and may have spread to the muscle layer of the rectum wall. Cancer has spread to 7 or more nearby lymph nodes. In stage IIIC: - Cancer has spread through the serosa (outermost layer) of the rectum wall but has not spread to nearby organs. Cancer has spread to at least 4 but not more than 6 nearby lymph nodes; or - Cancer has spread through the muscle layer of the rectum wall to the serosa (outermost layer) of the rectum wall or has spread through the serosa but has not spread to nearby organs. Cancer has spread to 7 or more nearby lymph nodes; or - Cancer has spread through the serosa (outermost layer) of the rectum wall and has spread to nearby organs. Cancer has spread to one or more nearby lymph nodes or cancer cells have formed in tissues near the lymph nodes. Stage IV Stage IV rectal cancer is divided into stage IVA and stage IVB. - Stage IVA: Cancer may have spread through the rectum wall and may have spread to nearby organs or lymph nodes. Cancer has spread to one organ that is not near the rectum, such as the liver, lung, or ovary, or to a distant lymph node. - Stage IVB: Cancer may have spread through the rectum wall and may have spread to nearby organs or lymph nodes. Cancer has spread to more than one organ that is not near the rectum or into the lining of the abdominal wall.",Rectal Cancer,0000037_2,CancerGov,https://www.cancer.gov/types/colorectal/patient/rectal-treatment-pdq,C0007113,T191,Disorders What are the treatments for Rectal Cancer ?,0000037_2-7,treatment,"Key Points - There are different types of treatment for patients with rectal cancer. - Five types of standard treatment are used: - Surgery - Radiation therapy - Chemotherapy - Active surveillance - Targeted therapy - Other types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with rectal cancer. Different types of treatment are available for patients with rectal cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Five types of standard treatment are used: Surgery Surgery is the most common treatment for all stages of rectal cancer. The cancer is removed using one of the following types of surgery: - Polypectomy: If the cancer is found in a polyp (a small piece of bulging tissue), the polyp is often removed during a colonoscopy. - Local excision: If the cancer is found on the inside surface of the rectum and has not spread into the wall of the rectum, the cancer and a small amount of surrounding healthy tissue is removed. - Resection: If the cancer has spread into the wall of the rectum, the section of the rectum with cancer and nearby healthy tissue is removed. Sometimes the tissue between the rectum and the abdominal wall is also removed. The lymph nodes near the rectum are removed and checked under a microscope for signs of cancer. - Radiofrequency ablation: The use of a special probe with tiny electrodes that kill cancer cells. Sometimes the probe is inserted directly through the skin and only local anesthesia is needed. In other cases, the probe is inserted through an incision in the abdomen. This is done in the hospital with general anesthesia. - Cryosurgery: A treatment that uses an instrument to freeze and destroy abnormal tissue. This type of treatment is also called cryotherapy. - Pelvic exenteration: If the cancer has spread to other organs near the rectum, the lower colon, rectum, and bladder are removed. In women, the cervix, vagina, ovaries, and nearby lymph nodes may be removed. In men, the prostate may be removed. Artificial openings (stoma) are made for urine and stool to flow from the body to a collection bag. After the cancer is removed, the surgeon will either: - do an anastomosis (sew the healthy parts of the rectum together, sew the remaining rectum to the colon, or sew the colon to the anus); or - make a stoma (an opening) from the rectum to the outside of the body for waste to pass through. This procedure is done if the cancer is too close to the anus and is called a colostomy. A bag is placed around the stoma to collect the waste. Sometimes the colostomy is needed only until the rectum has healed, and then it can be reversed. If the entire rectum is removed, however, the colostomy may be permanent. Radiation therapy and/or chemotherapy may be given before surgery to shrink the tumor, make it easier to remove the cancer, and help with bowel control after surgery. Treatment given before surgery is called neoadjuvant therapy. Even if all the cancer that can be seen at the time of the operation is removed, some patients may be given radiation therapy and/or chemotherapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External radiation therapy is used to treat rectal cancer. Short-course preoperative radiation therapy is used in some types of rectal cancer. This treatment uses fewer and lower doses of radiation than standard treatment, followed by surgery several days after the last dose. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly in the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Chemoembolization of the hepatic artery is a type of regional chemotherapy that may be used to treat cancer that has spread to the liver. This is done by blocking the hepatic artery (the main artery that supplies blood to the liver) and injecting anticancer drugs between the blockage and the liver. The livers arteries then carry the drugs into the liver. Only a small amount of the drug reaches other parts of the body. The blockage may be temporary or permanent, depending on what is used to block the artery. The liver continues to receive some blood from the hepatic portal vein, which carries blood from the stomach and intestine. The way the chemotherapy is given depends on the type and stage of the cancer being treated. See Drugs Approved for Rectal Cancer for more information. Active surveillance Active surveillance is closely following a patient's condition without giving any treatment unless there are changes in test results. It is used to find early signs that the condition is getting worse. In active surveillance, patients are given certain exams and tests to check if the cancer is growing. When the cancer begins to grow, treatment is given to cure the cancer. Tests include the following: - Digital rectal exam. - MRI. - Endoscopy. - Sigmoidoscopy. - CT scan. - Carcinoembryonic antigen (CEA) assay. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Types of targeted therapies used in the treatment of rectal cancer include the following: - Monoclonal antibodies: Monoclonal antibody therapy is a type of targeted therapy being used for the treatment of rectal cancer. Monoclonal antibody therapy uses antibodies made in the laboratory from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. - Bevacizumab and ramucirumab are types of monoclonal antibodies that bind to a protein called vascular endothelial growth factor (VEGF). This may prevent the growth of new blood vessels that tumors need to grow. - Cetuximab and panitumumab are types of monoclonal antibodies that bind to a protein called epidermal growth factor receptor (EGFR) on the surface of some types of cancer cells. This may stop cancer cells from growing and dividing. - Angiogenesis inhibitors: Angiogenesis inhibitors stop the growth of new blood vessels that tumors need to grow. - Ziv-aflibercept is a vascular endothelial growth factor trap that blocks an enzyme needed for the growth of new blood vessels in tumors. - Regorafenib is used to treat colorectal cancer that has spread to other parts of the body and has not gotten better with other treatment. It blocks the action of certain proteins, including vascular endothelial growth factor. This may help keep cancer cells from growing and may kill them. It may also prevent the growth of new blood vessels that tumors need to grow. See Drugs Approved for Rectal Cancer for more information. Other types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. After treatment for rectal cancer, a blood test to measure amounts of carcinoembryonic antigen (a substance in the blood that may be increased when cancer is present) may be done to see if the cancer has come back. Treatment Options by Stage Stage 0 (Carcinoma in Situ) Treatment of stage 0 may include the following: - Simple polypectomy. - Local excision. - Resection (when the tumor is too large to remove by local excision). Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage 0 rectal cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage I Rectal Cancer Treatment of stage I rectal cancer may include the following: - Local excision. - Resection. - Resection with radiation therapy and chemotherapy after surgery. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I rectal cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stages II and III Rectal Cancer Treatment of stage II and stage III rectal cancer may include the following: - Surgery. - Chemotherapy combined with radiation therapy, followed by surgery. - Short-course radiation therapy followed by surgery and chemotherapy. - Resection followed by chemotherapy combined with radiation therapy. - Chemotherapy combined with radiation therapy, followed by active surveillance. Surgery may be done if the cancer recurs (comes back). - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage II rectal cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage IV and Recurrent Rectal Cancer Treatment of stage IV and recurrent rectal cancer may include the following: - Surgery with or without chemotherapy or radiation therapy. - Systemic chemotherapy with or without targeted therapy (a monoclonal antibody or angiogenesis inhibitor). - Chemotherapy to control the growth of the tumor. - Radiation therapy, chemotherapy, or a combination of both, as palliative therapy to relieve symptoms and improve the quality of life. - Placement of a stent to help keep the rectum open if it is partly blocked by the tumor, as palliative therapy to relieve symptoms and improve the quality of life. - A clinical trial of a new anticancer drug. Treatment of rectal cancer that has spread to other organs depends on where the cancer has spread. - Treatment for areas of cancer that have spread to the liver includes the following: - Surgery to remove the tumor. Chemotherapy may be given before surgery, to shrink the tumor. - Cryosurgery or radiofrequency ablation. - Chemoembolization and/or systemic chemotherapy. - A clinical trial of chemoembolization combined with radiation therapy to the tumors in the liver. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage IV rectal cancer and recurrent rectal cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Rectal Cancer,0000037_2,CancerGov,https://www.cancer.gov/types/colorectal/patient/rectal-treatment-pdq,C0007113,T191,Disorders what research (or clinical trials) is being done for Rectal Cancer ?,0000037_2-8,research,"Other types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Rectal Cancer,0000037_2,CancerGov,https://www.cancer.gov/types/colorectal/patient/rectal-treatment-pdq,C0007113,T191,Disorders What is (are) Colorectal Cancer ?,0000037_3-1,information,"Key Points - Colorectal cancer is a disease in which malignant (cancer) cells form in the tissues of the colon or the rectum. - Colorectal cancer is the second leading cause of death from cancer in the United States. Colorectal cancer is a disease in which malignant (cancer) cells form in the tissues of the colon or the rectum. The colon is part of the body's digestive system. The digestive system removes and processes nutrients (vitamins, minerals, carbohydrates, fats, proteins, and water) from foods and helps pass waste material out of the body. The digestive system is made up of the mouth, throat, esophagus, stomach, and the small and large intestines. The colon (large bowel) is the first part of the large intestine and is about 5 feet long. Together, the rectum and anal canal make up the last part of the large intestine and are 6 to 8 inches long. The anal canal ends at the anus (the opening of the large intestine to the outside of the body). Cancer that begins in the colon is called colon cancer, and cancer that begins in the rectum is called rectal cancer. Cancer that affects either of these organs may also be called colorectal cancer. See the following PDQ summaries for more information about colorectal cancer: - Colorectal Cancer Screening - Colon Cancer Treatment - Rectal Cancer Treatment - Genetics of Colorectal Cancer Colorectal cancer is the second leading cause of death from cancer in the United States. The number of new colorectal cancer cases and the number of deaths from colorectal cancer are both decreasing a little bit each year. However, in adults younger than 50 years, the number of new colorectal cancer cases has slowly increased since 1998. The number of new colorectal cancers and deaths from colorectal cancer are higher in African Americans than in other races. Finding and treating colorectal cancer early may prevent death from colorectal cancer. Screening tests may be used to help find colorectal cancer.",Colorectal Cancer,0000037_3,CancerGov,https://www.cancer.gov/types/colorectal/patient/colorectal-prevention-pdq,C1527249,T191,Disorders How to prevent Colorectal Cancer ?,0000037_3-2,prevention,"Key Points - Avoiding risk factors and increasing protective factors may help prevent cancer. - The following risk factors increase the risk of colorectal cancer: - Age - Family history of colorectal cancer - Personal history - Inherited risk - Alcohol - Cigarette smoking - Obesity - The following protective factors decrease the risk of colorectal cancer: - Physical activity - Aspirin - Combination hormone replacement therapy - Polyp removal - It is not clear if the following affect the risk of colorectal cancer: - Nonsteroidal anti-inflammatory drugs (NSAIDs) other than aspirin - Calcium - Diet - The following factors do not affect the risk of colorectal cancer: - Hormone replacement therapy with estrogen only - Statins - Cancer prevention clinical trials are used to study ways to prevent cancer. - New ways to prevent colorectal cancer are being studied in clinical trials. Avoiding risk factors and increasing protective factors may help prevent cancer. Avoiding cancer risk factors may help prevent certain cancers. Risk factors include smoking, being overweight, and not getting enough exercise. Increasing protective factors such as quitting smoking and exercising may also help prevent some cancers. Talk to your doctor or other health care professional about how you might lower your risk of cancer. The following risk factors increase the risk of colorectal cancer: Age The risk of colorectal cancer increases after age 50. Most cases of colorectal cancer are diagnosed after age 50. Family history of colorectal cancer Having a parent, brother, sister, or child with colorectal cancer doubles a person's risk of colorectal cancer. Personal history Having a personal history of the following conditions increases the risk of colorectal cancer: - Previous colorectal cancer. - High-risk adenomas (colorectal polyps that are 1 centimeter or larger in size or that have cells that look abnormal under a microscope). - Ovarian cancer. - Inflammatory bowel disease (such as ulcerative colitis or Crohn disease). Inherited risk The risk of colorectal cancer is increased when certain gene changes linked to familial adenomatous polyposis (FAP) or hereditary nonpolyposis colon cancer (HNPCC or Lynch Syndrome) are inherited. Alcohol Drinking 3 or more alcoholic beverages per day increases the risk of colorectal cancer. Drinking alcohol is also linked to the risk of forming large colorectal adenomas (benign tumors). Cigarette smoking Cigarette smoking is linked to an increased risk of colorectal cancer and death from colorectal cancer. Smoking cigarettes is also linked to an increased risk of forming colorectal adenomas. Cigarette smokers who have had surgery to remove colorectal adenomas are at an increased risk for the adenomas to recur (come back). Obesity Obesity is linked to an increased risk of colorectal cancer and death from colorectal cancer. The following protective factors decrease the risk of colorectal cancer: Physical activity A lifestyle that includes regular physical activity is linked to a decreased risk of colorectal cancer. Aspirin Studies have shown that taking aspirin lowers the risk of colorectal cancer and the risk of death from colorectal cancer. The decrease in risk begins 10 to 20 years after patients start taking aspirin. The possible harms of aspirin use (100 mg or less) daily or every other day include an increased risk of stroke and bleeding in the stomach and intestines. These risks may be greater among the elderly, men, and those with conditions linked to a higher than normal risk of bleeding. Combination hormone replacement therapy Studies have shown that combination hormone replacement therapy (HRT) that includes both estrogen and progestin lowers the risk of invasive colorectal cancer in postmenopausal women. However, in women who take combination HRT and do develop colorectal cancer, the cancer is more likely to be advanced when it is diagnosed and the risk of dying from colorectal cancer is not decreased. The possible harms of combination HRT include an increased risk of having: - Breast cancer. - Heart disease. - Blood clots. Polyp removal Most colorectal polyps are adenomas, which may develop into cancer. Removing colorectal polyps that are larger than 1 centimeter (pea-sized) may lower the risk of colorectal cancer. It is not known if removing smaller polyps lowers the risk of colorectal cancer. The possible harms of polyp removal during colonoscopy or sigmoidoscopy include a tear in the wall of the colon and bleeding. It is not clear if the following affect the risk of colorectal cancer: Nonsteroidal anti-inflammatory drugs (NSAIDs) other than aspirin It is not known if the use of nonsteroidal anti-inflammatory drugs or NSAIDs (such as sulindac, celecoxib, naproxen, and ibuprofen) lowers the risk of colorectal cancer. Studies have shown that taking the nonsteroidal anti-inflammatory drug celecoxib reduces the risk of colorectal adenomas (benign tumors) coming back after they have been removed. It is not clear if this results in a lower risk of colorectal cancer. Taking sulindac or celecoxib has been shown to reduce the number and size of polyps that form in the colon and rectum of people with familial adenomatous polyposis (FAP). It is not clear if this results in a lower risk of colorectal cancer. The possible harms of NSAIDs include: - Kidney problems. - Bleeding in the stomach, intestines, or brain. - Heart problems such as heart attack and congestive heart failure. Calcium It is not known if taking calcium supplements lowers the risk of colorectal cancer. Diet It is not known if a diet low in fat and meat and high in fiber, fruits, and vegetables lowers the risk of colorectal cancer. Some studies have shown that a diet high in fat, proteins, calories, and meat increases the risk of colorectal cancer, but other studies have not. The following factors do not affect the risk of colorectal cancer: Hormone replacement therapy with estrogen only Hormone replacement therapy with estrogen only does not lower the risk of having invasive colorectal cancer or the risk of dying from colorectal cancer. Statins Studies have shown that taking statins (drugs that lower cholesterol) does not increase or decrease the risk of colorectal cancer. Cancer prevention clinical trials are used to study ways to prevent cancer. Cancer prevention clinical trials are used to study ways to lower the risk of developing certain types of cancer. Some cancer prevention trials are conducted with healthy people who have not had cancer but who have an increased risk for cancer. Other prevention trials are conducted with people who have had cancer and are trying to prevent another cancer of the same type or to lower their chance of developing a new type of cancer. Other trials are done with healthy volunteers who are not known to have any risk factors for cancer. The purpose of some cancer prevention clinical trials is to find out whether actions people take can prevent cancer. These may include exercising more or quitting smoking or taking certain medicines, vitamins, minerals, or food supplements. New ways to prevent colorectal cancer are being studied in clinical trials. Clinical trials are taking place in many parts of the country. Information about clinical trials can be found in the Clinical Trials section of the NCI website. Check NCI's list of cancer clinical trials for colon cancer prevention trials or rectal cancer prevention trials that are now accepting patients.",Colorectal Cancer,0000037_3,CancerGov,https://www.cancer.gov/types/colorectal/patient/colorectal-prevention-pdq,C1527249,T191,Disorders Who is at risk for Colorectal Cancer? ?,0000037_3-3,susceptibility,"Key Points - Avoiding risk factors and increasing protective factors may help prevent cancer. - The following risk factors increase the risk of colorectal cancer: - Age - Family history of colorectal cancer - Personal history - Inherited risk - Alcohol - Cigarette smoking - Obesity - The following protective factors decrease the risk of colorectal cancer: - Physical activity - Aspirin - Combination hormone replacement therapy - Polyp removal - It is not clear if the following affect the risk of colorectal cancer: - Nonsteroidal anti-inflammatory drugs (NSAIDs) other than aspirin - Calcium - Diet - The following factors do not affect the risk of colorectal cancer: - Hormone replacement therapy with estrogen only - Statins - Cancer prevention clinical trials are used to study ways to prevent cancer. - New ways to prevent colorectal cancer are being studied in clinical trials. Avoiding risk factors and increasing protective factors may help prevent cancer. Avoiding cancer risk factors may help prevent certain cancers. Risk factors include smoking, being overweight, and not getting enough exercise. Increasing protective factors such as quitting smoking and exercising may also help prevent some cancers. Talk to your doctor or other health care professional about how you might lower your risk of cancer. The following risk factors increase the risk of colorectal cancer: Age The risk of colorectal cancer increases after age 50. Most cases of colorectal cancer are diagnosed after age 50. Family history of colorectal cancer Having a parent, brother, sister, or child with colorectal cancer doubles a person's risk of colorectal cancer. Personal history Having a personal history of the following conditions increases the risk of colorectal cancer: - Previous colorectal cancer. - High-risk adenomas (colorectal polyps that are 1 centimeter or larger in size or that have cells that look abnormal under a microscope). - Ovarian cancer. - Inflammatory bowel disease (such as ulcerative colitis or Crohn disease). Inherited risk The risk of colorectal cancer is increased when certain gene changes linked to familial adenomatous polyposis (FAP) or hereditary nonpolyposis colon cancer (HNPCC or Lynch Syndrome) are inherited. Alcohol Drinking 3 or more alcoholic beverages per day increases the risk of colorectal cancer. Drinking alcohol is also linked to the risk of forming large colorectal adenomas (benign tumors). Cigarette smoking Cigarette smoking is linked to an increased risk of colorectal cancer and death from colorectal cancer. Smoking cigarettes is also linked to an increased risk of forming colorectal adenomas. Cigarette smokers who have had surgery to remove colorectal adenomas are at an increased risk for the adenomas to recur (come back). Obesity Obesity is linked to an increased risk of colorectal cancer and death from colorectal cancer. It is not clear if the following affect the risk of colorectal cancer: Nonsteroidal anti-inflammatory drugs (NSAIDs) other than aspirin It is not known if the use of nonsteroidal anti-inflammatory drugs or NSAIDs (such as sulindac, celecoxib, naproxen, and ibuprofen) lowers the risk of colorectal cancer. Studies have shown that taking the nonsteroidal anti-inflammatory drug celecoxib reduces the risk of colorectal adenomas (benign tumors) coming back after they have been removed. It is not clear if this results in a lower risk of colorectal cancer. Taking sulindac or celecoxib has been shown to reduce the number and size of polyps that form in the colon and rectum of people with familial adenomatous polyposis (FAP). It is not clear if this results in a lower risk of colorectal cancer. The possible harms of NSAIDs include: - Kidney problems. - Bleeding in the stomach, intestines, or brain. - Heart problems such as heart attack and congestive heart failure. Calcium It is not known if taking calcium supplements lowers the risk of colorectal cancer. Diet It is not known if a diet low in fat and meat and high in fiber, fruits, and vegetables lowers the risk of colorectal cancer. Some studies have shown that a diet high in fat, proteins, calories, and meat increases the risk of colorectal cancer, but other studies have not. The following factors do not affect the risk of colorectal cancer: Hormone replacement therapy with estrogen only Hormone replacement therapy with estrogen only does not lower the risk of having invasive colorectal cancer or the risk of dying from colorectal cancer. Statins Studies have shown that taking statins (drugs that lower cholesterol) does not increase or decrease the risk of colorectal cancer.",Colorectal Cancer,0000037_3,CancerGov,https://www.cancer.gov/types/colorectal/patient/colorectal-prevention-pdq,C1527249,T191,Disorders what research (or clinical trials) is being done for Colorectal Cancer ?,0000037_3-4,research,"Cancer prevention clinical trials are used to study ways to prevent cancer. Cancer prevention clinical trials are used to study ways to lower the risk of developing certain types of cancer. Some cancer prevention trials are conducted with healthy people who have not had cancer but who have an increased risk for cancer. Other prevention trials are conducted with people who have had cancer and are trying to prevent another cancer of the same type or to lower their chance of developing a new type of cancer. Other trials are done with healthy volunteers who are not known to have any risk factors for cancer. The purpose of some cancer prevention clinical trials is to find out whether actions people take can prevent cancer. These may include exercising more or quitting smoking or taking certain medicines, vitamins, minerals, or food supplements. New ways to prevent colorectal cancer are being studied in clinical trials. Clinical trials are taking place in many parts of the country. Information about clinical trials can be found in the Clinical Trials section of the NCI website. Check NCI's list of cancer clinical trials for colon cancer prevention trials or rectal cancer prevention trials that are now accepting patients.",Colorectal Cancer,0000037_3,CancerGov,https://www.cancer.gov/types/colorectal/patient/colorectal-prevention-pdq,C1527249,T191,Disorders What is (are) Colorectal Cancer ?,0000037_4-1,information,"Key Points - Colorectal cancer is a disease in which malignant (cancer) cells form in the tissues of the colon or the rectum. - Colorectal cancer is the second leading cause of death from cancer in the United States. - Different factors increase or decrease the risk of getting colorectal cancer. Colorectal cancer is a disease in which malignant (cancer) cells form in the tissues of the colon or the rectum. The colon and rectum are parts of the body's digestive system. The digestive system removes and processes nutrients (vitamins, minerals, carbohydrates, fats, proteins, and water) from foods and helps pass waste material out of the body. The digestive system is made up of the mouth, throat, esophagus, stomach, and the small and large intestines. The colon (large bowel) is the first part of the large intestine and is about 5 feet long. Together, the rectum and anal canal make up the last part of the large intestine and are 6-8 inches long. The anal canal ends at the anus (the opening of the large intestine to the outside of the body). Cancer that begins in the colon is called colon cancer, and cancer that begins in the rectum is called rectal cancer. Cancer that begins in either of these organs may also be called colorectal cancer. See the following PDQ summaries for more information about colorectal cancer: - Colorectal Cancer Prevention - Colon Cancer Treatment - Rectal Cancer Treatment - Genetics of Colorectal Cancer Colorectal cancer is the second leading cause of death from cancer in the United States. The number of new colorectal cancer cases and the number of deaths from colorectal cancer are decreasing a little bit each year. But in adults younger than 50 years, there has been a small increase in the number of new cases each year since 1998. Colorectal cancer is found more often in men than in women.",Colorectal Cancer,0000037_4,CancerGov,https://www.cancer.gov/types/colorectal/patient/colorectal-screening-pdq,C1527249,T191,Disorders Who is at risk for Colorectal Cancer? ?,0000037_4-2,susceptibility,"Different factors increase or decrease the risk of getting colorectal cancer. Anything that increases your chance of getting a disease is called a risk factor. Anything that decreases your chance of getting a disease is called a protective factor. For information about risk factors and protective factors for colorectal cancer, see the PDQ summary on Colorectal Cancer Prevention.",Colorectal Cancer,0000037_4,CancerGov,https://www.cancer.gov/types/colorectal/patient/colorectal-screening-pdq,C1527249,T191,Disorders What is (are) Small Intestine Cancer ?,0000039_1-1,information,"Key Points - Small intestine cancer is a rare disease in which malignant (cancer) cells form in the tissues of the small intestine. - There are five types of small intestine cancer. - Diet and health history can affect the risk of developing small intestine cancer. - Signs and symptoms of small intestine cancer include unexplained weight loss and abdominal pain. - Tests that examine the small intestine are used to detect (find), diagnose, and stage small intestine cancer. - Certain factors affect prognosis (chance of recovery) and treatment options. Small intestine cancer is a rare disease in which malignant (cancer) cells form in the tissues of the small intestine. The small intestine is part of the bodys digestive system, which also includes the esophagus, stomach, and large intestine. The digestive system removes and processes nutrients (vitamins, minerals, carbohydrates, fats, proteins, and water) from foods and helps pass waste material out of the body. The small intestine is a long tube that connects the stomach to the large intestine. It folds many times to fit inside the abdomen. There are five types of small intestine cancer. The types of cancer found in the small intestine are adenocarcinoma, sarcoma, carcinoid tumors, gastrointestinal stromal tumor, and lymphoma. This summary discusses adenocarcinoma and leiomyosarcoma (a type of sarcoma). Adenocarcinoma starts in glandular cells in the lining of the small intestine and is the most common type of small intestine cancer. Most of these tumors occur in the part of the small intestine near the stomach. They may grow and block the intestine. Leiomyosarcoma starts in the smooth muscle cells of the small intestine. Most of these tumors occur in the part of the small intestine near the large intestine. See the following PDQ summaries for more information on small intestine cancer: - Adult Soft Tissue Sarcoma Treatment - Childhood Soft Tissue Sarcoma Treatment - Adult Non-Hodgkin Lymphoma Treatment - Childhood Non-Hodgkin Lymphoma Treatment - Gastrointestinal Carcinoid Tumors Treatment - Gastrointestinal Stromal Tumors Treatment",Small Intestine Cancer,0000039_1,CancerGov,https://www.cancer.gov/types/small-intestine/patient/small-intestine-treatment-pdq,C0238196,T191,Disorders Who is at risk for Small Intestine Cancer? ?,0000039_1-2,susceptibility,Diet and health history can affect the risk of developing small intestine cancer. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for small intestine cancer include the following: - Eating a high-fat diet. - Having Crohn disease. - Having celiac disease. - Having familial adenomatous polyposis (FAP).,Small Intestine Cancer,0000039_1,CancerGov,https://www.cancer.gov/types/small-intestine/patient/small-intestine-treatment-pdq,C0238196,T191,Disorders What are the symptoms of Small Intestine Cancer ?,0000039_1-3,symptoms,Signs and symptoms of small intestine cancer include unexplained weight loss and abdominal pain. These and other signs and symptoms may be caused by small intestine cancer or by other conditions. Check with your doctor if you have any of the following: - Pain or cramps in the middle of the abdomen. - Weight loss with no known reason. - A lump in the abdomen. - Blood in the stool.,Small Intestine Cancer,0000039_1,CancerGov,https://www.cancer.gov/types/small-intestine/patient/small-intestine-treatment-pdq,C0238196,T191,Disorders How to diagnose Small Intestine Cancer ?,0000039_1-4,exams and tests,"Tests that examine the small intestine are used to detect (find), diagnose, and stage small intestine cancer.Procedures that make pictures of the small intestine and the area around it help diagnose small intestine cancer and show how far the cancer has spread. The process used to find out if cancer cells have spread within and around the small intestine is called staging. In order to plan treatment, it is important to know the type of small intestine cancer and whether the tumor can be removed by surgery. Tests and procedures to detect, diagnose, and stage small intestine cancer are usually done at the same time. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. - Liver function tests : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by the liver. A higher than normal amount of a substance can be a sign of liver disease that may be caused by small intestine cancer. - Endoscopy : A procedure to look at organs and tissues inside the body to check for abnormal areas. There are different types of endoscopy: - Upper endoscopy : A procedure to look at the inside of the esophagus, stomach, and duodenum (first part of the small intestine, near the stomach). An endoscope is inserted through the mouth and into the esophagus, stomach, and duodenum. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer. - Capsule endoscopy : A procedure to look at the inside of the small intestine. A capsule that is about the size of a large pill and contains a light and a tiny wireless camera is swallowed by the patient. The capsule travels through the digestive tract, including the small intestine, and sends many pictures of the inside of the digestive tract to a recorder that is worn around the waist or over the shoulder. The pictures are sent from the recorder to a computer and viewed by the doctor who checks for signs of cancer. The capsule passes out of the body during a bowel movement. - Double balloon endoscopy : A procedure to look at the inside of the small intestine. A special instrument made up of two tubes (one inside the other) is inserted through the mouth or rectum and into the small intestine. The inside tube (an endoscope with a light and lens for viewing) is moved through part of the small intestine and a balloon at the end of it is inflated to keep the endoscope in place. Next, the outer tube is moved through the small intestine to reach the end of the endoscope, and a balloon at the end of the outer tube is inflated to keep it in place. Then, the balloon at the end of the endoscope is deflated and the endoscope is moved through the next part of the small intestine. These steps are repeated many times as the tubes move through the small intestine. The doctor is able to see the inside of the small intestine through the endoscope and use a tool to remove samples of abnormal tissue. The tissue samples are checked under a microscope for signs of cancer. This procedure may be done if the results of a capsule endoscopy are abnormal. This procedure is also called double balloon enteroscopy. - Laparotomy : A surgical procedure in which an incision (cut) is made in the wall of the abdomen to check the inside of the abdomen for signs of disease. The size of the incision depends on the reason the laparotomy is being done. Sometimes organs or lymph nodes are removed or tissue samples are taken and checked under a microscope for signs of disease. - Biopsy : The removal of cells or tissues so they can be viewed under a microscope to check for signs of cancer. This may be done during an endoscopy or laparotomy. The sample is checked by a pathologist to see if it contains cancer cells. - Upper GI series with small bowel follow-through: A series of x-rays of the esophagus, stomach, and small bowel. The patient drinks a liquid that contains barium (a silver-white metallic compound). The liquid coats the esophagus, stomach, and small bowel. X-rays are taken at different times as the barium travels through the upper GI tract and small bowel. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI).",Small Intestine Cancer,0000039_1,CancerGov,https://www.cancer.gov/types/small-intestine/patient/small-intestine-treatment-pdq,C0238196,T191,Disorders What is the outlook for Small Intestine Cancer ?,0000039_1-5,outlook,"Certain factors affect prognosis (chance of recovery) and treatment options.The prognosis (chance of recovery) and treatment options depend on the following: - The type of small intestine cancer. - Whether the cancer is in the inner lining of the small intestine only or has spread into or beyond the wall of the small intestine. - Whether the cancer has spread to other places in the body, such as the lymph nodes, liver, or peritoneum (tissue that lines the wall of the abdomen and covers most of the organs in the abdomen). - Whether the cancer can be completely removed by surgery. - Whether the cancer is newly diagnosed or has recurred.",Small Intestine Cancer,0000039_1,CancerGov,https://www.cancer.gov/types/small-intestine/patient/small-intestine-treatment-pdq,C0238196,T191,Disorders What are the stages of Small Intestine Cancer ?,0000039_1-6,stages,"Key Points - Tests and procedures to stage small intestine cancer are usually done at the same time as diagnosis. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - Small intestine cancer is grouped according to whether or not the tumor can be completely removed by surgery. Tests and procedures to stage small intestine cancer are usually done at the same time as diagnosis. Staging is used to find out how far the cancer has spread, but treatment decisions are not based on stage. See the General Information section for a description of tests and procedures used to detect, diagnose, and stage small intestine cancer. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if small intestine cancer spreads to the liver, the cancer cells in the liver are actually small intestine cancer cells. The disease is metastatic small intestine cancer, not liver cancer. Small intestine cancer is grouped according to whether or not the tumor can be completely removed by surgery. Treatment depends on whether the tumor can be removed by surgery and if the cancer is being treated as a primary tumor or is metastatic cancer.",Small Intestine Cancer,0000039_1,CancerGov,https://www.cancer.gov/types/small-intestine/patient/small-intestine-treatment-pdq,C0238196,T191,Disorders What are the treatments for Small Intestine Cancer ?,0000039_1-7,treatment,"Key Points - There are different types of treatment for patients with small intestine cancer. - Three types of standard treatment are used: - Surgery - Radiation therapy - Chemotherapy - New types of treatment are being tested in clinical trials. - Biologic therapy - Radiation therapy with radiosensitizers - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with small intestine cancer. Different types of treatments are available for patients with small intestine cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Three types of standard treatment are used: Surgery Surgery is the most common treatment of small intestine cancer. One of the following types of surgery may be done: - Resection: Surgery to remove part or all of an organ that contains cancer. The resection may include the small intestine and nearby organs (if the cancer has spread). The doctor may remove the section of the small intestine that contains cancer and perform an anastomosis (joining the cut ends of the intestine together). The doctor will usually remove lymph nodes near the small intestine and examine them under a microscope to see whether they contain cancer. - Bypass: Surgery to allow food in the small intestine to go around (bypass) a tumor that is blocking the intestine but cannot be removed. Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type of the cancer being treated. External radiation therapy is used to treat small intestine cancer. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Biologic therapy Biologic therapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy. Radiation therapy with radiosensitizers Radiosensitizers are drugs that make tumor cells more sensitive to radiation therapy. Combining radiation therapy with radiosensitizers may kill more tumor cells. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Small Intestine Cancer Small Intestine Adenocarcinoma When possible, treatment of small intestine adenocarcinoma will be surgery to remove the tumor and some of the normal tissue around it. Treatment of small intestine adenocarcinoma that cannot be removed by surgery may include the following: - Surgery to bypass the tumor. - Radiation therapy as palliative therapy to relieve symptoms and improve the patient's quality of life. - A clinical trial of radiation therapy with radiosensitizers, with or without chemotherapy. - A clinical trial of new anticancer drugs. - A clinical trial of biologic therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with small intestine adenocarcinoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Small Intestine Leiomyosarcoma When possible, treatment of small intestine leiomyosarcoma will be surgery to remove the tumor and some of the normal tissue around it. Treatment of small intestine leiomyosarcoma that cannot be removed by surgery may include the following: - Surgery (to bypass the tumor) and radiation therapy. - Surgery, radiation therapy, or chemotherapy as palliative therapy to relieve symptoms and improve the patient's quality of life. - A clinical trial of new anticancer drugs. - A clinical trial of biologic therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with small intestine leiomyosarcoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Recurrent Small Intestine Cancer Treatment of recurrent small intestine cancer that has spread to other parts of the body is usually a clinical trial of new anticancer drugs or biologic therapy. Treatment of locally recurrent small intestine cancer may include the following: - Surgery. - Radiation therapy or chemotherapy as palliative therapy to relieve symptoms and improve the patient's quality of life. - A clinical trial of radiation therapy with radiosensitizers, with or without chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent small intestine cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Small Intestine Cancer,0000039_1,CancerGov,https://www.cancer.gov/types/small-intestine/patient/small-intestine-treatment-pdq,C0238196,T191,Disorders What is (are) Thymoma and Thymic Carcinoma ?,0000040_1-1,information,"Key Points - Thymoma and thymic carcinoma are diseases in which malignant (cancer) cells form on the outside surface of the thymus. - Thymoma is linked with myasthenia gravis and other autoimmune diseases. - Signs and symptoms of thymoma and thymic carcinoma include a cough and chest pain. - Tests that examine the thymus are used to detect (find) thymoma or thymic carcinoma. - Thymoma and thymic carcinoma are usually diagnosed, staged, and treated during surgery. - Certain factors affect prognosis (chance of recovery) and treatment options. Thymoma and thymic carcinoma are diseases in which malignant (cancer) cells form on the outside surface of the thymus. The thymus, a small organ that lies in the upper chest under the breastbone, is part of the lymph system. It makes white blood cells, called lymphocytes, that protect the body against infections. There are different types of tumors of the thymus. Thymomas and thymic carcinomas are rare tumors of the cells that are on the outside surface of the thymus. The tumor cells in a thymoma look similar to the normal cells of the thymus, grow slowly, and rarely spread beyond the thymus. On the other hand, the tumor cells in a thymic carcinoma look very different from the normal cells of the thymus, grow more quickly, and have usually spread to other parts of the body when the cancer is found. Thymic carcinoma is more difficult to treat than thymoma. For information on thymoma and thymic carcinoma in children, see the PDQ summary on Unusual Cancers of Childhood Treatment. Thymoma is linked with myasthenia gravis and other autoimmune diseases. People with thymoma often have autoimmune diseases as well. These diseases cause the immune system to attack healthy tissue and organs. They include: - Myasthenia gravis. - Acquired pure red cell aplasia. - Hypogammaglobulinemia. - Polymyositis. - Lupus erythematosus. - Rheumatoid arthritis. - Thyroiditis. - Sjgren syndrome.",Thymoma and Thymic Carcinoma,0000040_1,CancerGov,https://www.cancer.gov/types/thymoma/patient/thymoma-treatment-pdq,C1322286,T191,Disorders What are the symptoms of Thymoma and Thymic Carcinoma ?,0000040_1-2,symptoms,"Signs and symptoms of thymoma and thymic carcinoma include a cough and chest pain. Thymoma and thymic carcinoma may not cause early signs or symptoms. The cancer may be found during a routine chest x-ray. Signs and symptoms may be caused by thymoma, thymic carcinoma, or other conditions. Check with your doctor if you have any of the following: - A cough that doesn't go away. - Chest pain. - Trouble breathing.",Thymoma and Thymic Carcinoma,0000040_1,CancerGov,https://www.cancer.gov/types/thymoma/patient/thymoma-treatment-pdq,C1322286,T191,Disorders How to diagnose Thymoma and Thymic Carcinoma ?,0000040_1-3,exams and tests,"Tests that examine the thymus are used to detect (find) thymoma or thymic carcinoma. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Chest x-ray: An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the chest, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body, such as the chest. This procedure is also called nuclear magnetic resonance imaging (NMRI). - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. Thymoma and thymic carcinoma are usually diagnosed, staged, and treated during surgery. A biopsy of the tumor is done to diagnose the disease. The biopsy may be done before or during surgery (a mediastinoscopy or mediastinotomy), using a thin needle to remove a sample of cells. This is called a fine-needle aspiration (FNA) biopsy. Sometimes a wide needle is used to remove a sample of cells and this is called a core biopsy. A pathologist will view the sample under a microscope to check for cancer. If thymoma or thymic carcinoma is diagnosed, the pathologist will determine the type of cancer cell in the tumor. There may be more than one type of cancer cell in a thymoma. The surgeon will decide if all or part of the tumor can be removed by surgery. In some cases, lymph nodes and other tissues may be removed as well.",Thymoma and Thymic Carcinoma,0000040_1,CancerGov,https://www.cancer.gov/types/thymoma/patient/thymoma-treatment-pdq,C1322286,T191,Disorders What is the outlook for Thymoma and Thymic Carcinoma ?,0000040_1-4,outlook,Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - The stage of the cancer. - The type of cancer cell. - Whether the tumor can be removed completely by surgery. - The patient's general health. - Whether the cancer has just been diagnosed or has recurred (come back).,Thymoma and Thymic Carcinoma,0000040_1,CancerGov,https://www.cancer.gov/types/thymoma/patient/thymoma-treatment-pdq,C1322286,T191,Disorders What are the stages of Thymoma and Thymic Carcinoma ?,0000040_1-5,stages,"Key Points - Tests done to detect thymoma or thymic carcinoma are also used to stage the disease. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - The following stages are used for thymoma: - Stage I - Stage II - Stage III - Stage IV - Thymic carcinomas have usually spread to other parts of the body when diagnosed. Tests done to detect thymoma or thymic carcinoma are also used to stage the disease. Staging is the process used to find out if cancer has spread from the thymus to other parts of the body. The findings made during surgery and the results of tests and procedures are used to determine the stage of the disease. It is important to know the stage in order to plan treatment. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if thymic carcinoma spreads to the bone, the cancer cells in the bone are actually thymic carcinoma cells. The disease is metastatic thymic carcinoma, not bone cancer. The following stages are used for thymoma: Stage I In stage I, cancer is found only within the thymus. All cancer cells are inside the capsule (sac) that surrounds the thymus. Stage II In stage II, cancer has spread through the capsule and into the fat around the thymus or into the lining of the chest cavity. Stage III In stage III, cancer has spread to nearby organs in the chest, including the lung, the sac around the heart, or large blood vessels that carry blood to the heart. Stage IV Stage IV is divided into stage IVA and stage IVB, depending on where the cancer has spread. - In stage IVA, cancer has spread widely around the lungs and heart. - In stage IVB, cancer has spread to the blood or lymph system. Thymic carcinomas have usually spread to other parts of the body when diagnosed. The staging system used for thymomas is sometimes used for thymic carcinomas.",Thymoma and Thymic Carcinoma,0000040_1,CancerGov,https://www.cancer.gov/types/thymoma/patient/thymoma-treatment-pdq,C1322286,T191,Disorders what research (or clinical trials) is being done for Thymoma and Thymic Carcinoma ?,0000040_1-6,research,"New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Thymoma and Thymic Carcinoma,0000040_1,CancerGov,https://www.cancer.gov/types/thymoma/patient/thymoma-treatment-pdq,C1322286,T191,Disorders What are the treatments for Thymoma and Thymic Carcinoma ?,0000040_1-7,treatment,"Key Points - There are different types of treatment for patients with thymoma and thymic carcinoma. - Four types of standard treatment are used: - Surgery - Radiation therapy - Chemotherapy - Hormone therapy - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with thymoma and thymic carcinoma. Different types of treatments are available for patients with thymoma and thymic carcinoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Four types of standard treatment are used: Surgery Surgery to remove the tumor is the most common treatment of thymoma. Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External radiation therapy is used to treat thymoma and thymic carcinoma. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. Chemotherapy may be used to shrink the tumor before surgery or radiation therapy. This is called neoadjuvant chemotherapy. Hormone therapy Hormone therapy is a cancer treatment that removes hormones or blocks their action and stops cancer cells from growing. Hormones are substances produced by glands in the body and circulated in the bloodstream. Some hormones can cause certain cancers to grow. If tests show that the cancer cells have places where hormones can attach (receptors), drugs, surgery, or radiation therapy is used to reduce the production of hormones or block them from working. Hormone therapy with drugs called corticosteroids may be used to treat thymoma or thymic carcinoma. New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Thymoma and Thymic Carcinoma Stage I and Stage II Thymoma Treatment of stage I thymoma is surgery. Treatment of stage II thymoma is surgery followed by radiation therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I thymoma and stage II thymoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage III and Stage IV Thymoma Treatment of stage III and stage IV thymoma that may be completely removed by surgery includes the following: - Surgery with or without radiation therapy. - Neoadjuvant chemotherapy followed by surgery with or without radiation therapy. - A clinical trial of anticancer drugs in new combinations or doses. - A clinical trial of new ways of giving radiation therapy. Treatment of stage III and stage IV thymoma that cannot be completely removed by surgery includes the following: - Neoadjuvant chemotherapy followed by surgery and/or radiation therapy. - Radiation therapy. - Chemotherapy. - A clinical trial of anticancer drugs in new combinations or doses. - A clinical trial of new ways of giving radiation therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III thymoma and stage IV thymoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Thymic Carcinoma Treatment of thymic carcinoma that may be completely removed by surgery includes the following: - Surgery with or without radiation therapy. - A clinical trial of anticancer drugs in new combinations or doses. - A clinical trial of new ways of giving radiation therapy. Treatment of thymic carcinoma that cannot be completely removed by surgery includes the following: - Radiation therapy. - Chemotherapy with or without surgery to remove part of the tumor and/or radiation therapy. - Chemotherapy with radiation therapy. - Chemotherapy. - A clinical trial of anticancer drugs in new combinations or doses. - A clinical trial of new ways of giving radiation therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with thymic carcinoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Recurrent Thymoma and Thymic Carcinoma Treatment of recurrent thymoma and thymic carcinoma may include the following: - Surgery with or without radiation therapy. - Radiation therapy. - Hormone therapy. - Chemotherapy. - A clinical trial of anticancer drugs in new combinations or doses. - A clinical trial of new ways of giving radiation therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent thymoma and thymic carcinoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Thymoma and Thymic Carcinoma,0000040_1,CancerGov,https://www.cancer.gov/types/thymoma/patient/thymoma-treatment-pdq,C1322286,T191,Disorders What is (are) Urethral Cancer ?,0000041_1-1,information,"Key Points - Urethral cancer is a disease in which malignant (cancer) cells form in the tissues of the urethra. - There are different types of urethral cancer that begin in cells that line the urethra. - A history of bladder cancer can affect the risk of urethral cancer. - Signs of urethral cancer include bleeding or trouble with urination. - Tests that examine the urethra and bladder are used to detect (find) and diagnose urethral cancer. - Certain factors affect prognosis (chance of recovery) and treatment options. Urethral cancer is a disease in which malignant (cancer) cells form in the tissues of the urethra. The urethra is the tube that carries urine from the bladder to outside the body. In women, the urethra is about 1 inches long and is just above the vagina. In men, the urethra is about 8 inches long, and goes through the prostate gland and the penis to the outside of the body. In men, the urethra also carries semen. Urethral cancer is a rare cancer that occurs more often in men than in women. There are different types of urethral cancer that begin in cells that line the urethra. These cancers are named for the types of cells that become malignant (cancer): - Squamous cell carcinoma is the most common type of urethral cancer. It forms in cells in the part of the urethra near the bladder in women, and in the lining of the urethra in the penis in men. - Transitional cell carcinoma forms in the area near the urethral opening in women, and in the part of the urethra that goes through the prostate gland in men. - Adenocarcinoma forms in the glands that are around the urethra in both men and women. Urethral cancer can metastasize (spread) quickly to tissues around the urethra and is often found in nearby lymph nodes by the time it is diagnosed. A history of bladder cancer can affect the risk of urethral cancer. Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesnt mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for urethral cancer include the following: - Having a history of bladder cancer. - Having conditions that cause chronic inflammation in the urethra, including: - Sexually transmitted diseases (STDs), including human papillomavirus (HPV), especially HPV type 16. - Frequent urinary tract infections (UTIs).",Urethral Cancer,0000041_1,CancerGov,https://www.cancer.gov/types/urethral/patient/urethral-treatment-pdq,C0700101,T191,Disorders What are the symptoms of Urethral Cancer ?,0000041_1-2,symptoms,"Signs of urethral cancer include bleeding or trouble with urination. These and other signs and symptoms may be caused by urethral cancer or by other conditions. There may be no signs or symptoms in the early stages. Check with your doctor if you have any of the following: - Trouble starting the flow of urine. - Weak or interrupted (""stop-and-go"") flow of urine. - Frequent urination, especially at night. - Incontinence. - Discharge from the urethra. - Bleeding from the urethra or blood in the urine. - A lump or thickness in the perineum or penis. - A painless lump or swelling in the groin.",Urethral Cancer,0000041_1,CancerGov,https://www.cancer.gov/types/urethral/patient/urethral-treatment-pdq,C0700101,T191,Disorders How to diagnose Urethral Cancer ?,0000041_1-3,exams and tests,"Tests that examine the urethra and bladder are used to detect (find) and diagnose urethral cancer. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient's health habits and past illnesses and treatments will also be taken. - Pelvic exam : An exam of the vagina, cervix, uterus, fallopian tubes, ovaries, and rectum. A speculum is inserted into the vagina and the doctor or nurse looks at the vagina and cervix for signs of disease. The doctor or nurse also inserts one or two lubricated, gloved fingers of one hand into the vagina and places the other hand over the lower abdomen to feel the size, shape, and position of the uterus and ovaries. The doctor or nurse also inserts a lubricated, gloved finger into the rectum to feel for lumps or abnormal areas. - Digital rectal exam : An exam of the rectum. The doctor or nurse inserts a lubricated, gloved finger into the lower part of the rectum to feel for lumps or anything else that seems unusual. - Urine cytology : A laboratory test in which a sample of urine is checked under a microscope for abnormal cells. - Urinalysis : A test to check the color of urine and its contents, such as sugar, protein, blood, and white blood cells. If white blood cells (a sign of infection) are found, a urine culture is usually done to find out what type of infection it is. - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. - Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following: - The number of red blood cells, white blood cells, and platelets. - The amount of hemoglobin (the protein that carries oxygen) in the red blood cells. - The portion of the blood sample made up of red blood cells. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the pelvis and abdomen, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - Ureteroscopy : A procedure to look inside the ureter and renal pelvis to check for abnormal areas. A ureteroscope is a thin, tube-like instrument with a light and a lens for viewing. The ureteroscope is inserted through the urethra into the bladder, ureter, and renal pelvis. A tool may be inserted through the ureteroscope to take tissue samples to be checked under a microscope for signs of disease. - Biopsy: The removal of cell or tissue samples from the urethra, bladder, and, sometimes, the prostate gland. The samples are viewed under a microscope by a pathologist to check for signs of cancer.",Urethral Cancer,0000041_1,CancerGov,https://www.cancer.gov/types/urethral/patient/urethral-treatment-pdq,C0700101,T191,Disorders What is the outlook for Urethral Cancer ?,0000041_1-4,outlook,"Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - Where the cancer formed in the urethra. - Whether the cancer has spread through the mucosa lining the urethra to nearby tissue, to lymph nodes, or to other parts of the body. - Whether the patient is a male or female. - The patient's general health. - Whether the cancer has just been diagnosed or has recurred (come back).",Urethral Cancer,0000041_1,CancerGov,https://www.cancer.gov/types/urethral/patient/urethral-treatment-pdq,C0700101,T191,Disorders What are the stages of Urethral Cancer ?,0000041_1-5,stages,"Key Points - After urethral cancer has been diagnosed, tests are done to find out if cancer cells have spread within the urethra or to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - Urethral cancer is staged and treated based on the part of the urethra that is affected. - Distal urethral cancer - Proximal urethral cancer - Bladder and/or prostate cancer may occur at the same time as urethral cancer. After urethral cancer has been diagnosed, tests are done to find out if cancer cells have spread within the urethra or to other parts of the body. The process used to find out if cancer has spread within the urethra or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following procedures may be used in the staging process: - Chest x-ray : An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. - CT scan (CAT scan) of the pelvis and abdomen : A procedure that makes a series of detailed pictures of the pelvis and abdomen, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of the urethra, nearby lymph nodes, and other soft tissue and bones in the pelvis. A substance called gadolinium is injected into the patient through a vein. The gadolinium collects around the cancer cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Urethrography: A series of x-rays of the urethra. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. A dye is injected through the urethra into the bladder. The dye coats the bladder and urethra and x-rays are taken to see if the urethra is blocked and if cancer has spread to nearby tissue. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if urethral cancer spreads to the lung, the cancer cells in the lung are actually urethral cancer cells. The disease is metastatic urethral cancer, not lung cancer. Urethral cancer is staged and treated based on the part of the urethra that is affected. Urethral cancer is staged and treated based on the part of the urethra that is affected and how deeply the tumor has spread into tissue around the urethra. Urethral cancer can be described as distal or proximal. Distal urethral cancer In distal urethral cancer, the cancer usually has not spread deeply into the tissue. In women, the part of the urethra that is closest to the outside of the body (about inch) is affected. In men, the part of the urethra that is in the penis is affected. Proximal urethral cancer Proximal urethral cancer affects the part of the urethra that is not the distal urethra. In women and men, proximal urethral cancer usually has spread deeply into tissue. Bladder and/or prostate cancer may occur at the same time as urethral cancer. In men, cancer that forms in the proximal urethra (the part of the urethra that passes through the prostate to the bladder) may occur at the same time as cancer of the bladder and/or prostate. Sometimes this occurs at diagnosis and sometimes it occurs later.",Urethral Cancer,0000041_1,CancerGov,https://www.cancer.gov/types/urethral/patient/urethral-treatment-pdq,C0700101,T191,Disorders What are the treatments for Urethral Cancer ?,0000041_1-6,treatment,"Key Points - There are different types of treatment for patients with urethral cancer. - Four types of standard treatment are used: - Surgery - Radiation therapy - Chemotherapy - Active surveillance - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with urethral cancer. Different types of treatments are available for patients with urethral cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Four types of standard treatment are used: Surgery Surgery to remove the cancer is the most common treatment for cancer of the urethra. One of the following types of surgery may be done: - Open excision: Removal of the cancer by surgery. - Transurethral resection (TUR): Surgery to remove the cancer using a special tool inserted into the urethra. - Electroresection with fulguration: Surgery to remove the cancer by electric current. A lighted tool with a small wire loop on the end is used to remove the cancer or to burn the tumor away with high-energy electricity. - Laser surgery: A surgical procedure that uses a laser beam (a narrow beam of intense light) as a knife to make bloodless cuts in tissue or to remove or destroy tissue. - Lymph node dissection: Lymph nodes in the pelvis and groin may be removed. - Cystourethrectomy: Surgery to remove the bladder and the urethra. - Cystoprostatectomy: Surgery to remove the bladder and the prostate. - Anterior exenteration: Surgery to remove the urethra, the bladder, and the vagina. Plastic surgery may be done to rebuild the vagina. - Partial penectomy: Surgery to remove the part of the penis surrounding the urethra where cancer has spread. Plastic surgery may be done to rebuild the penis. - Radical penectomy: Surgery to remove the entire penis. Plastic surgery may be done to rebuild the penis. If the urethra is removed, the surgeon will make a new way for the urine to pass from the body. This is called urinary diversion. If the bladder is removed, the surgeon will make a new way for urine to be stored and passed from the body. The surgeon may use part of the small intestine to make a tube that passes urine through an opening (stoma). This is called an ostomy or urostomy. If a patient has an ostomy, a disposable bag to collect urine is worn under clothing. The surgeon may also use part of the small intestine to make a new storage pouch (continent reservoir) inside the body where the urine can collect. A tube (catheter) is then used to drain the urine through a stoma. Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type of cancer and where the cancer formed in the urethra. External and internal radiation therapy are used to treat urethral cancer. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type of cancer and where the cancer formed in the urethra. Active surveillance Active surveillance is following a patient's condition without giving any treatment unless there are changes in test results. It is used to find early signs that the condition is getting worse. In active surveillance, patients are given certain exams and tests, including biopsies, on a regular schedule. New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Urethral Cancer Distal Urethral Cancer Treatment of abnormal cells in the mucosa (inside lining of the urethra that have not become cancer, may include surgery to remove the tumor (open excision or transurethral resection), electroresection with fulguration, or laser surgery. Treatment of distal urethral cancer is different for men and women. For women, treatment may include the following: - Surgery to remove the tumor (transurethral resection), electroresection and fulguration, or laser surgery for tumors that have not spread deeply into tissue. - Brachytherapy and/or external radiation therapy for tumors that have not spread deeply into tissue. - Surgery to remove the tumor (anterior exenteration) for tumors that have spread deeply into tissue. Sometimes nearby lymph nodes are also removed (lymph node dissection). Radiation therapy may be given before surgery. For men, treatment may include the following: - Surgery to remove the tumor (transurethral resection), electroresection and fulguration, or laser surgery for tumors that have not spread deeply into tissue. - Surgery to remove part of the penis (partial penectomy) for tumors that are near the tip of the penis. Sometimes nearby lymph nodes are also removed (lymph node dissection). - Surgery to remove part of the urethra for tumors that are in the distal urethra but not at the tip of the penis and have not spread deeply into tissue. Sometimes nearby lymph nodes are also removed (lymph node dissection). - Surgery to remove the penis (radical penectomy) for tumors that have spread deeply into tissue. Sometimes nearby lymph nodes are also removed (lymph node dissection). - Radiation therapy with or without chemotherapy. - Chemotherapy given together with radiation therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with distal urethral cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Proximal Urethral Cancer Treatment of proximal urethral cancer or urethral cancer that affects the entire urethra is different for men and women. For women, treatment may include the following: - Radiation therapy and/or surgery (open excision, transurethral resection) for tumors that are of an inch or smaller. - Radiation therapy followed by surgery (anterior exenteration with lymph node dissection and urinary diversion). For men, treatment may include the following: - Radiation therapy or radiation therapy and chemotherapy, followed by surgery (cystoprostatectomy, penectomy, lymph node dissection, and urinary diversion). Check the list of NCI-supported cancer clinical trials that are now accepting patients with proximal urethral cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Urethral Cancer that Forms with Invasive Bladder Cancer Treatment of urethral cancer that forms at the same time as invasive bladder cancer may include the following: - Surgery (cystourethrectomy in women, or urethrectomy and cystoprostatectomy in men). If the urethra is not removed during surgery to remove the bladder, treatment may include the following: - Active surveillance. Samples of cells are taken from inside the urethra and checked under a microscope for signs of cancer. Check the list of NCI-supported cancer clinical trials that are now accepting patients with urethral cancer associated with invasive bladder cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Metastatic or Recurrent Urethral Cancer Treatment of urethral cancer that has metastasized (spread to other parts of the body) is usually chemotherapy. Treatment of recurrent urethral cancer may include one or more of the following: - Surgery to remove the tumor. Sometimes nearby lymph nodes are also removed (lymph node dissection). - Radiation therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent urethral cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Urethral Cancer,0000041_1,CancerGov,https://www.cancer.gov/types/urethral/patient/urethral-treatment-pdq,C0700101,T191,Disorders what research (or clinical trials) is being done for Urethral Cancer ?,0000041_1-7,research,"New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Urethral Cancer,0000041_1,CancerGov,https://www.cancer.gov/types/urethral/patient/urethral-treatment-pdq,C0700101,T191,Disorders What is (are) Vulvar Cancer ?,0000043_1-1,information,"Key Points - Vulvar cancer is a rare disease in which malignant (cancer) cells form in the tissues of the vulva. - Having vulvar intraepithelial neoplasia or HPV infection can affect the risk of vulvar cancer. - Signs of vulvar cancer include bleeding or itching. - Tests that examine the vulva are used to detect (find) and diagnose vulvar cancer. - Certain factors affect prognosis (chance of recovery) and treatment options. Vulvar cancer is a rare disease in which malignant (cancer) cells form in the tissues of the vulva. Vulvar cancer forms in a woman's external genitalia. The vulva includes: - Inner and outer lips of the vagina. - Clitoris (sensitive tissue between the lips). - Opening of the vagina and its glands. - Mons pubis (the rounded area in front of the pubic bones that becomes covered with hair at puberty). - Perineum (the area between the vulva and the anus). Vulvar cancer most often affects the outer vaginal lips. Less often, cancer affects the inner vaginal lips, clitoris, or vaginal glands. Vulvar cancer usually forms slowly over a number of years. Abnormal cells can grow on the surface of the vulvar skin for a long time. This condition is called vulvar intraepithelial neoplasia (VIN). Because it is possible for VIN to become vulvar cancer, it is very important to get treatment. Having vulvar intraepithelial neoplasia or HPV infection can affect the risk of vulvar cancer. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for vulvar cancer include the following: - Having vulvar intraepithelial neoplasia (VIN). - Having human papillomavirus (HPV) infection. - Having a history of genital warts. Other possible risk factors include the following: - Having many sexual partners. - Having first sexual intercourse at a young age. - Having a history of abnormal Pap tests (Pap smears). Recurrent Vulvar Cancer Recurrent vulvar cancer is cancer that has recurred (come back) after it has been treated. The cancer may come back in the vulva or in other parts of the body.",Vulvar Cancer,0000043_1,CancerGov,https://www.cancer.gov/types/vulvar/patient/vulvar-treatment-pdq,C0375071,T191,Disorders What are the symptoms of Vulvar Cancer ?,0000043_1-2,symptoms,"Signs of vulvar cancer include bleeding or itching. Vulvar cancer often does not cause early signs or symptoms. Signs and symptoms may be caused by vulvar cancer or by other conditions. Check with your doctor if you have any of the following: - A lump or growth on the vulva. - Changes in the vulvar skin, such as color changes or growths that look like a wart or ulcer. - Itching in the vulvar area, that does not go away. - Bleeding not related to menstruation (periods). - Tenderness in the vulvar area.",Vulvar Cancer,0000043_1,CancerGov,https://www.cancer.gov/types/vulvar/patient/vulvar-treatment-pdq,C0375071,T191,Disorders How to diagnose Vulvar Cancer ?,0000043_1-3,exams and tests,"Tests that examine the vulva are used to detect (find) and diagnose vulvar cancer. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking the vulva for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Biopsy : The removal of samples of cells or tissues from the vulva so they can be viewed under a microscope by a pathologist to check for signs of cancer.",Vulvar Cancer,0000043_1,CancerGov,https://www.cancer.gov/types/vulvar/patient/vulvar-treatment-pdq,C0375071,T191,Disorders What is the outlook for Vulvar Cancer ?,0000043_1-4,outlook,Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - The stage of the cancer. - The patient's age and general health. - Whether the cancer has just been diagnosed or has recurred (come back).,Vulvar Cancer,0000043_1,CancerGov,https://www.cancer.gov/types/vulvar/patient/vulvar-treatment-pdq,C0375071,T191,Disorders What are the stages of Vulvar Cancer ?,0000043_1-5,stages,"Key Points - After vulvar cancer has been diagnosed, tests are done to find out if cancer cells have spread within the vulva or to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - In vulvar intraepithelial neoplasia (VIN), abnormal cells are found on the surface of the vulvar skin. - The following stages are used for vulvar cancer: - Stage I - Stage II - Stage III - Stage IV After vulvar cancer has been diagnosed, tests are done to find out if cancer cells have spread within the vulva or to other parts of the body. The process used to find out if cancer has spread within the vulva or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following tests and procedures may be used in the staging process: - Pelvic exam : An exam of the vagina, cervix, uterus, fallopian tubes, ovaries, and rectum. A speculum is inserted into the vagina and the doctor or nurse looks at the vagina and cervix for signs of disease. A Pap test of the cervix is usually done. The doctor or nurse also inserts one or two lubricated, gloved fingers of one hand into the vagina and places the other hand over the lower abdomen to feel the size, shape, and position of the uterus and ovaries. The doctor or nurse also inserts a lubricated, gloved finger into the rectum to feel for lumps or abnormal areas. - Colposcopy : A procedure in which a colposcope (a lighted, magnifying instrument) is used to check the vagina and cervix for abnormal areas. Tissue samples may be taken using a curette (spoon-shaped instrument) or a brush and checked under a microscope for signs of disease. - Cystoscopy : A procedure to look inside the bladder and urethra to check for abnormal areas. A cystoscope is inserted through the urethra into the bladder. A cystoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer. - Proctoscopy : A procedure to look inside the rectum and anus to check for abnormal areas. A proctoscope is inserted into the anus and rectum. A proctoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer. - X-rays : An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. To stage vulvar cancer, x-rays may be taken of the organs and bones inside the chest, and the pelvic bones. - Intravenous pyelogram (IVP): A series of x-rays of the kidneys, ureters, and bladder to find out if cancer has spread to these organs. A contrast dye is injected into a vein. As the contrast dye moves through the kidneys, ureters and bladder, x-rays are taken to see if there are any blockages. This procedure is also called intravenous urography. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. - Sentinel lymph node biopsy : The removal of the sentinel lymph node during surgery. The sentinel lymph node is the first lymph node to receive lymphatic drainage from a tumor. It is the first lymph node the cancer is likely to spread to from the tumor. A radioactive substance and/or blue dye is injected near the tumor. The substance or dye flows through the lymph ducts to the lymph nodes. The first lymph node to receive the substance or dye is removed. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are not found, it may not be necessary to remove more lymph nodes. Sentinel lymph node biopsy may be done during surgery to remove the tumor for early-stage vulvar cancer. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if vulvar cancer spreads to the lung, the cancer cells in the lung are actually vulvar cancer cells. The disease is metastatic vulvar cancer, not lung cancer. In vulvar intraepithelial neoplasia (VIN), abnormal cells are found on the surface of the vulvar skin. These abnormal cells are not cancer. Vulvar intraepithelial neoplasia (VIN) may become cancer and spread into nearby tissue. VIN is sometimes called stage 0 or carcinoma in situ. The following stages are used for vulvar cancer: Stage I In stage I, cancer has formed. The tumor is found only in the vulva or perineum (area between the rectum and the vagina). Stage I is divided into stages IA and IB. - In stage IA, the tumor is 2 centimeters or smaller and has spread 1 millimeter or less into the tissue of the vulva. Cancer has not spread to the lymph nodes. - In stage IB, the tumor is larger than 2 centimeters or has spread more than 1 millimeter into the tissue of the vulva. Cancer has not spread to the lymph nodes. Stage II In stage II, the tumor is any size and has spread into the lower part of the urethra, the lower part of the vagina, or the anus. Cancer has not spread to the lymph nodes. Stage III In stage III, the tumor is any size and may have spread into the lower part of the urethra, the lower part of the vagina, or the anus. Cancer has spread to one or more nearby lymph nodes. Stage III is divided into stages IIIA, IIIB, and IIIC. - In stage IIIA, cancer is found in 1 or 2 lymph nodes that are smaller than 5 millimeters or in one lymph node that is 5 millimeters or larger. - In stage IIIB, cancer is found in 2 or more lymph nodes that are 5 millimeters or larger, or in 3 or more lymph nodes that are smaller than 5 millimeters. - In stage IIIC, cancer is found in lymph nodes and has spread to the outside surface of the lymph nodes. Stage IV In stage IV, the tumor has spread into the upper part of the urethra, the upper part of the vagina, or to other parts of the body. Stage IV is divided into stages IVA and IVB. - In stage IVA: - cancer has spread into the lining of the upper urethra, the upper vagina, the bladder, or the rectum, or has attached to the pelvic bone; or - cancer has spread to nearby lymph nodes and the lymph nodes are not moveable or have formed an ulcer. - In stage IVB, cancer has spread to lymph nodes in the pelvis or to other parts of the body.",Vulvar Cancer,0000043_1,CancerGov,https://www.cancer.gov/types/vulvar/patient/vulvar-treatment-pdq,C0375071,T191,Disorders what research (or clinical trials) is being done for Vulvar Cancer ?,0000043_1-6,research,"New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.",Vulvar Cancer,0000043_1,CancerGov,https://www.cancer.gov/types/vulvar/patient/vulvar-treatment-pdq,C0375071,T191,Disorders What are the treatments for Vulvar Cancer ?,0000043_1-7,treatment,"Key Points - There are different types of treatment for patients with vulvar cancer. - Four types of standard treatment are used: - Surgery - Radiation therapy - Chemotherapy - Biologic therapy - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with vulvar cancer. Different types of treatments are available for patients with vulvar cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Four types of standard treatment are used: Surgery Surgery is the most common treatment for vulvar cancer. The goal of surgery is to remove all the cancer without any loss of the woman's sexual function. One of the following types of surgery may be done: - Laser surgery: A surgical procedure that uses a laser beam (a narrow beam of intense light) as a knife to make bloodless cuts in tissue or to remove a surface lesion such as a tumor. - Wide local excision: A surgical procedure to remove the cancer and some of the normal tissue around the cancer. - Radical local excision: A surgical procedure to remove the cancer and a large amount of normal tissue around it. Nearby lymph nodes in the groin may also be removed. - Ultrasound surgical aspiration (USA): A surgical procedure to break the tumor up into small pieces using very fine vibrations. The small pieces of tumor are washed away and removed by suction. This procedure causes less damage to nearby tissue. - Vulvectomy: A surgical procedure to remove part or all of the vulva: - Skinning vulvectomy: The top layer of vulvar skin where the cancer is found is removed. Skin grafts from other parts of the body may be needed to cover the area where the skin was removed. - Modified radical vulvectomy: Surgery to remove part of the vulva. Nearby lymph nodes may also be removed. - Radical vulvectomy: Surgery to remove the entire vulva. Nearby lymph nodes are also removed. - Pelvic exenteration: A surgical procedure to remove the lower colon, rectum, and bladder. The cervix, vagina, ovaries, and nearby lymph nodes are also removed. Artificial openings (stoma) are made for urine and stool to flow from the body into a collection bag. Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may have chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External and internal radiation therapy are used to treat vulvar cancer, and external radiation therapy may also be used as palliative therapy to relieve symptoms and improve quality of life. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, a body cavity such as the abdomen, or onto the skin, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. Topical chemotherapy for vulvar cancer may be applied to the skin in a cream or lotion. See Drugs Approved to Treat Vulvar Cancer for more information. Biologic therapy Biologic therapy is a treatment that uses the patients immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the bodys natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy. Imiquimod is a biologic therapy that may be used to treat vulvar lesions and is applied to the skin in a cream. New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. It is important to have regular follow-up exams to check for recurrent vulvar cancer. Treatment Options by Stage Vulvar Intraepithelial Neoplasia (VIN): Treatment of vulvar intraepithelial neoplasia (VIN) may include the following: - Removal of single lesions or wide local excision. - Laser surgery. - Ultrasound surgical aspiration. - Skinning vulvectomy with or without a skin graft. - Biologic therapy with topical imiquimod. - Stage I Vulvar Cancer: Treatment of stage I vulvar cancer may include the following: - Wide local excision for lesions that are less than 1 millimeter deep.. - Radical local excision and removal of nearby lymph nodes. - Radical local excision and sentinel lymph node biopsy. If cancer is found in the sentinel lymph node, nearby lymph nodes are also removed. - Radiation therapy for patients who cannot have surgery. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I vulvar cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. - Stage II Vulvar Cancer: Treatment of stage II vulvar cancer may include the following: - Radical local excision and removal of nearby lymph nodes. - Modified radical vulvectomy or radical vulvectomy for large tumors. Nearby lymph nodes may be removed. Radiation therapy may be given after surgery. - Radical local excision and sentinel lymph node biopsy. If cancer is found in the sentinel lymph node, nearby lymph nodes are also removed. - Radiation therapy for patients who cannot have surgery. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage II vulvar cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. - Stage III Vulvar Cancer: Treatment of stage III vulvar cancer may include the following: - Modified radical vulvectomy or radical vulvectomy. Nearby lymph nodes may be removed. Radiation therapy may be given after surgery. - Radiation therapy or chemotherapy and radiation therapy followed by surgery. - Radiation therapy with or without chemotherapy for patients who cannot have surgery. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III vulvar cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. - Stage IV Vulvar Cancer: Treatment of stage IVA vulvar cancer may include the following: - Radical vulvectomy and pelvic exenteration. - Radical vulvectomy followed by radiation therapy. - Radiation therapy or chemotherapy and radiation therapy followed by surgery. - Radiation therapy with or without chemotherapy for patients who cannot have surgery. There is no standard treatment for stage IVB vulvar cancer. Treatment may include a clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage IVB vulvar cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Treatment Options for Recurrent Vulvar Cancer Treatment of recurrent vulvar cancer may include the following: - Wide local excision with or without radiation therapy to treat cancer that has come back in the same area. - Radical vulvectomy and pelvic exenteration to treat cancer that has come back in the same area. - Chemotherapy and radiation therapy with or without surgery. - Radiation therapy followed by surgery or chemotherapy. - Radiation therapy as palliative treatment to relieve symptoms and improve quality of life. - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent vulvar cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.",Vulvar Cancer,0000043_1,CancerGov,https://www.cancer.gov/types/vulvar/patient/vulvar-treatment-pdq,C0375071,T191,Disorders