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What is (are) Adult Acute Lymphoblastic Leukemia ? | 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. | information | CancerGov | Adult Acute Lymphoblastic Leukemia |
What are the symptoms of Adult Acute Lymphoblastic Leukemia ? | 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. | symptoms | CancerGov | Adult Acute Lymphoblastic Leukemia |
How to diagnose Adult Acute Lymphoblastic Leukemia ? | 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. | exams and tests | CancerGov | Adult Acute Lymphoblastic Leukemia |
What is the outlook for Adult Acute Lymphoblastic Leukemia ? | 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). | outlook | CancerGov | Adult Acute Lymphoblastic Leukemia |
Who is at risk for Adult Acute Lymphoblastic Leukemia? ? | 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. | susceptibility | CancerGov | Adult Acute Lymphoblastic Leukemia |
What are the stages of Adult Acute Lymphoblastic Leukemia ? | 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. | stages | CancerGov | Adult Acute Lymphoblastic Leukemia |
What are the treatments for Adult Acute Lymphoblastic Leukemia ? | 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. | treatment | CancerGov | Adult Acute Lymphoblastic Leukemia |
What is (are) Adult Acute Myeloid Leukemia ? | 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. | information | CancerGov | Adult Acute Myeloid Leukemia |
Who is at risk for Adult Acute Myeloid Leukemia? ? | 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. | susceptibility | CancerGov | Adult Acute Myeloid Leukemia |
What are the symptoms of Adult Acute Myeloid Leukemia ? | 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. | symptoms | CancerGov | Adult Acute Myeloid Leukemia |
How to diagnose Adult Acute Myeloid Leukemia ? | 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). | exams and tests | CancerGov | Adult Acute Myeloid Leukemia |
What is the outlook for Adult Acute Myeloid Leukemia ? | 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. | outlook | CancerGov | Adult Acute Myeloid Leukemia |
What are the stages of Adult Acute Myeloid Leukemia ? | 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. | stages | CancerGov | Adult Acute Myeloid Leukemia |
What are the treatments for Adult Acute Myeloid Leukemia ? | 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. | treatment | CancerGov | Adult Acute Myeloid Leukemia |
Who is at risk for Chronic Lymphocytic Leukemia? ? | 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. | susceptibility | CancerGov | Chronic Lymphocytic Leukemia |
What are the symptoms of Chronic Lymphocytic Leukemia ? | 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. | symptoms | CancerGov | Chronic Lymphocytic Leukemia |
What is the outlook for Chronic Lymphocytic Leukemia ? | 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. | outlook | CancerGov | Chronic Lymphocytic Leukemia |
what research (or clinical trials) is being done for Chronic Lymphocytic Leukemia ? | 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. | research | CancerGov | Chronic Lymphocytic Leukemia |
What are the treatments for Chronic Lymphocytic Leukemia ? | 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. | treatment | CancerGov | Chronic Lymphocytic Leukemia |
What is (are) Chronic Myelogenous Leukemia ? | 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 | information | CancerGov | Chronic Myelogenous Leukemia |
What are the symptoms of Chronic Myelogenous Leukemia ? | 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. | symptoms | CancerGov | Chronic Myelogenous Leukemia |
What are the genetic changes related to Chronic Myelogenous Leukemia ? | 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. | genetic changes | CancerGov | Chronic Myelogenous Leukemia |
How to diagnose Chronic Myelogenous Leukemia ? | 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. | exams and tests | CancerGov | Chronic Myelogenous Leukemia |
What is the outlook for Chronic Myelogenous Leukemia ? | 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. | outlook | CancerGov | Chronic Myelogenous Leukemia |
What are the stages of Chronic Myelogenous Leukemia ? | 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. | stages | CancerGov | Chronic Myelogenous Leukemia |
what research (or clinical trials) is being done for Chronic Myelogenous Leukemia ? | 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. | research | CancerGov | Chronic Myelogenous Leukemia |
What are the treatments for Chronic Myelogenous Leukemia ? | 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. | treatment | CancerGov | Chronic Myelogenous Leukemia |
What is (are) Hairy Cell Leukemia ? | 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. | information | CancerGov | Hairy Cell Leukemia |
What are the symptoms of Hairy Cell Leukemia ? | 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. | symptoms | CancerGov | Hairy Cell Leukemia |
How to diagnose Hairy Cell Leukemia ? | 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. | exams and tests | CancerGov | Hairy Cell Leukemia |
What is the outlook for Hairy Cell Leukemia ? | 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. | outlook | CancerGov | Hairy Cell Leukemia |
What are the stages of Hairy Cell Leukemia ? | 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. | stages | CancerGov | Hairy Cell Leukemia |
what research (or clinical trials) is being done for Hairy Cell Leukemia ? | 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. | research | CancerGov | Hairy Cell Leukemia |
What is (are) Childhood Acute Lymphoblastic Leukemia ? | 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 | information | CancerGov | Childhood Acute Lymphoblastic Leukemia |
How to diagnose Childhood Acute Lymphoblastic Leukemia ? | 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. | exams and tests | CancerGov | Childhood Acute Lymphoblastic Leukemia |
What is the outlook for Childhood Acute Lymphoblastic Leukemia ? | 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. | outlook | CancerGov | Childhood Acute Lymphoblastic Leukemia |
what research (or clinical trials) is being done for Childhood Acute Lymphoblastic Leukemia ? | 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. | research | CancerGov | Childhood Acute Lymphoblastic Leukemia |
Who is at risk for Childhood Acute Lymphoblastic Leukemia? ? | 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. | susceptibility | CancerGov | Childhood Acute Lymphoblastic Leukemia |
What are the treatments for Childhood Acute Lymphoblastic Leukemia ? | 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. | treatment | CancerGov | Childhood Acute Lymphoblastic Leukemia |
What is (are) Childhood Acute Myeloid Leukemia and Other Myeloid Malignancies ? | 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. | information | CancerGov | Childhood Acute Myeloid Leukemia and Other Myeloid Malignancies |
Who is at risk for Childhood Acute Myeloid Leukemia and Other Myeloid Malignancies? ? | 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. | susceptibility | CancerGov | Childhood Acute Myeloid Leukemia and Other Myeloid Malignancies |
What are the symptoms of Childhood Acute Myeloid Leukemia and Other Myeloid Malignancies ? | 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. | symptoms | CancerGov | Childhood Acute Myeloid Leukemia and Other Myeloid Malignancies |
What is the outlook for Childhood Acute Myeloid Leukemia and Other Myeloid Malignancies ? | 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. | outlook | CancerGov | Childhood Acute Myeloid Leukemia and Other Myeloid Malignancies |
What are the stages of Childhood Acute Myeloid Leukemia and Other Myeloid Malignancies ? | 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. | stages | CancerGov | Childhood Acute Myeloid Leukemia and Other Myeloid Malignancies |
what research (or clinical trials) is being done for Childhood Acute Myeloid Leukemia and Other Myeloid Malignancies ? | 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. | research | CancerGov | Childhood Acute Myeloid Leukemia and Other Myeloid Malignancies |
What is (are) Adult Soft Tissue Sarcoma ? | 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 | information | CancerGov | Adult Soft Tissue Sarcoma |
Who is at risk for Adult Soft Tissue Sarcoma? ? | 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. | susceptibility | CancerGov | Adult Soft Tissue Sarcoma |
How to diagnose Adult Soft Tissue Sarcoma ? | 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. | exams and tests | CancerGov | Adult Soft Tissue Sarcoma |
What is the outlook for Adult Soft Tissue Sarcoma ? | 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). | outlook | CancerGov | Adult Soft Tissue Sarcoma |
What are the stages of Adult Soft Tissue Sarcoma ? | 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. | stages | CancerGov | Adult Soft Tissue Sarcoma |
what research (or clinical trials) is being done for Adult Soft Tissue 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. | research | CancerGov | Adult Soft Tissue Sarcoma |
What are the treatments for Adult Soft Tissue Sarcoma ? | 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. | treatment | CancerGov | Adult Soft Tissue Sarcoma |
What is (are) Gastrointestinal Stromal Tumors ? | 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. | information | CancerGov | Gastrointestinal Stromal Tumors |
Who is at risk for Gastrointestinal Stromal Tumors? ? | 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. | susceptibility | CancerGov | Gastrointestinal Stromal Tumors |
What are the symptoms of Gastrointestinal Stromal Tumors ? | 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. | symptoms | CancerGov | Gastrointestinal Stromal Tumors |
What are the stages of Gastrointestinal Stromal Tumors ? | 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. | stages | CancerGov | Gastrointestinal Stromal Tumors |
What are the treatments for Gastrointestinal Stromal Tumors ? | 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. | treatment | CancerGov | Gastrointestinal Stromal Tumors |
How to diagnose 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. | exams and tests | CancerGov | Kaposi Sarcoma |
what research (or clinical trials) is being done for Kaposi 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. 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. | research | CancerGov | Kaposi Sarcoma |
What are the symptoms of Childhood Rhabdomyosarcoma ? | 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. | symptoms | CancerGov | Childhood Rhabdomyosarcoma |
How to diagnose Childhood Rhabdomyosarcoma ? | 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. | exams and tests | CancerGov | Childhood Rhabdomyosarcoma |
What are the stages of Childhood Rhabdomyosarcoma ? | 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. | stages | CancerGov | Childhood Rhabdomyosarcoma |
what research (or clinical trials) is being done for Childhood Rhabdomyosarcoma ? | 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. | research | CancerGov | Childhood Rhabdomyosarcoma |
What are the treatments for Childhood Rhabdomyosarcoma ? | 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. | treatment | CancerGov | Childhood Rhabdomyosarcoma |
What is (are) Childhood Soft Tissue Sarcoma ? | 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). | information | CancerGov | Childhood Soft Tissue Sarcoma |
Who is at risk for Childhood Soft Tissue Sarcoma? ? | 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. | susceptibility | CancerGov | Childhood Soft Tissue Sarcoma |
What are the symptoms 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. 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. | symptoms | CancerGov | Childhood Soft Tissue Sarcoma |
What is the outlook for Childhood Soft Tissue Sarcoma ? | 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). | outlook | CancerGov | Childhood Soft Tissue Sarcoma |
What are the stages of Childhood Soft Tissue Sarcoma ? | 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. | stages | CancerGov | Childhood Soft Tissue Sarcoma |
what research (or clinical trials) is being done for Childhood Soft Tissue 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. 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. | research | CancerGov | Childhood Soft Tissue Sarcoma |
What is (are) Childhood Vascular Tumors ? | 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. | information | CancerGov | Childhood Vascular Tumors |
How to diagnose Childhood Vascular Tumors ? | 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. | exams and tests | CancerGov | Childhood Vascular Tumors |
What are the treatments for Childhood Vascular Tumors ? | 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. | treatment | CancerGov | Childhood Vascular Tumors |
what research (or clinical trials) is being done for Childhood Vascular Tumors ? | 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. | research | CancerGov | Childhood Vascular Tumors |
What is (are) Adult Hodgkin Lymphoma ? | 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. | information | CancerGov | Adult Hodgkin Lymphoma |
Who is at risk for Adult Hodgkin Lymphoma? ? | 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. | susceptibility | CancerGov | Adult Hodgkin Lymphoma |
What are the symptoms of Adult Hodgkin Lymphoma ? | 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. | symptoms | CancerGov | Adult Hodgkin Lymphoma |
How to diagnose Adult Hodgkin Lymphoma ? | 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. | exams and tests | CancerGov | Adult Hodgkin Lymphoma |
what research (or clinical trials) is being done for Adult 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. 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. | research | CancerGov | Adult Hodgkin Lymphoma |
What are the stages of Adult Hodgkin Lymphoma ? | 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). | stages | CancerGov | Adult Hodgkin Lymphoma |
What are the treatments for Adult Hodgkin Lymphoma ? | 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. | treatment | CancerGov | Adult Hodgkin Lymphoma |
What is (are) Adult Non-Hodgkin Lymphoma ? | 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. | information | CancerGov | Adult Non-Hodgkin Lymphoma |
Who is at risk for Adult Non-Hodgkin Lymphoma? ? | 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. | susceptibility | CancerGov | Adult Non-Hodgkin Lymphoma |
How to diagnose Adult Non-Hodgkin Lymphoma ? | 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. | exams and tests | CancerGov | Adult Non-Hodgkin Lymphoma |
What is the outlook for Adult Non-Hodgkin Lymphoma ? | 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. | outlook | CancerGov | Adult Non-Hodgkin Lymphoma |
What are the stages of Adult Non-Hodgkin Lymphoma ? | 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. | stages | CancerGov | Adult Non-Hodgkin Lymphoma |
What are the treatments for Adult Non-Hodgkin Lymphoma ? | 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. | treatment | CancerGov | Adult Non-Hodgkin Lymphoma |
What is (are) AIDS-Related Lymphoma ? | 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 | information | CancerGov | AIDS-Related Lymphoma |
What are the symptoms of AIDS-Related Lymphoma ? | 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. | symptoms | CancerGov | AIDS-Related Lymphoma |
How to diagnose AIDS-Related Lymphoma ? | 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. | exams and tests | CancerGov | AIDS-Related Lymphoma |
What is the outlook for AIDS-Related Lymphoma ? | 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. | outlook | CancerGov | AIDS-Related Lymphoma |
What are the stages of AIDS-Related Lymphoma ? | 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. | stages | CancerGov | AIDS-Related Lymphoma |
What are the treatments for AIDS-Related Lymphoma ? | 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. | treatment | CancerGov | AIDS-Related Lymphoma |
What is (are) Mycosis Fungoides and the Szary Syndrome ? | 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. | information | CancerGov | Mycosis Fungoides and the Szary Syndrome |
What are the symptoms of Mycosis Fungoides and the Szary Syndrome ? | 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. | symptoms | CancerGov | Mycosis Fungoides and the Szary Syndrome |
How to diagnose Mycosis Fungoides and the Szary Syndrome ? | 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. | exams and tests | CancerGov | Mycosis Fungoides and the Szary Syndrome |
what research (or clinical trials) is being done for Mycosis Fungoides and the Szary Syndrome ? | 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. | research | CancerGov | Mycosis Fungoides and the Szary Syndrome |
What are the stages of Mycosis Fungoides and the Szary Syndrome ? | 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. | stages | CancerGov | Mycosis Fungoides and the Szary Syndrome |
What are the treatments for Mycosis Fungoides and the Szary Syndrome ? | 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. | treatment | CancerGov | Mycosis Fungoides and the Szary Syndrome |
Who is at risk for Primary CNS Lymphoma? ? | 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. | susceptibility | CancerGov | Primary CNS Lymphoma |