Topic: Wilms Tumor and Other Childhood Kidney Tumors
Question: What are the treatments for Wilms Tumor and Other Childhood Kidney Tumors ?
Answer: Key Points
                    - There are different types of treatment for patients with Wilms tumor and other childhood kidney tumors.    - Children with Wilms tumor or other childhood kidney tumors should have their treatment planned by a team of health care providers who are experts in treating cancer in children.    - Some cancer treatments cause side effects months or years after treatment has ended.    - Five types of standard treatment are used:         - Surgery     - Radiation therapy     - Chemotherapy     - Biologic therapy     - High-dose chemotherapy with stem cell rescue        - New types of treatment are being tested in clinical trials.         - Targeted therapy        - Patients may want to think about taking part in a clinical trial.    - Patients can enter clinical trials before, during, or after starting their cancer treatment.    - Follow-up tests may be needed.
                
                
                    There are different types of treatment for patients with Wilms tumor and other childhood kidney tumors.
                    Different types of treatment are available for children with Wilms and other childhood kidney tumors. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.   Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment.
                
                
                    Children with Wilms tumor or other childhood kidney tumors should have their treatment planned by a team of health care providers who are experts in treating cancer in children.
                    Your child's treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other pediatric health care providers who are experts in treating children with Wilms tumor or other childhood kidney tumors and who specialize in certain areas of medicine. These may include the following specialists:         -  Pediatrician.    -  Pediatric surgeon or urologist.    -  Radiation oncologist.    -  Rehabilitation specialist.    -  Pediatric nurse specialist.    -  Social worker.
                
                
                    Some cancer treatments cause side effects months or years after treatment has ended.
                    Side effects from cancer treatment that begin during or after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include the following:          - Physical problems, such as heart problems, kidney problems, or problems during pregnancy.    - Changes in mood, feelings, thinking, learning, or memory.    -  Second cancers (new types of cancer), such as cancer of the gastrointestinal tract or breast cancer.        Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. (See the PDQ summary about Late Effects of Treatment for Childhood Cancer for more information).     Clinical trials are being done to find out if lower doses of chemotherapy and radiation can be used to lessen the late effects of treatment without changing how well the treatment works.
                
                
                    Five types of standard treatment are used:
                    Surgery    Two types of surgery are used to treat kidney tumors:            -  Nephrectomy: Wilms tumor and other childhood kidney tumors are usually treated with nephrectomy (surgery to remove the whole kidney). Nearby lymph nodes may also be removed and checked for signs of cancer. Sometimes a kidney transplant (surgery to remove the kidney and replace it with a kidney from a donor) is done when the cancer is in both kidneys and the kidneys are not working well.     -  Partial nephrectomy: If cancer is found in both kidneys or is likely to spread to both kidneys, surgery may include a partial nephrectomy (removal of the cancer in the kidney and a small amount of normal tissue around it). Partial nephrectomy is done to keep as much of the kidney working as possible. A partial nephrectomy is also called renal-sparing surgery.           Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk of the cancer coming back, is called adjuvant therapy. Sometimes, a second-look surgery is done to see if cancer remains after chemotherapy or radiation therapy.       Radiation therapy    Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy:            -  External radiation therapy uses a machine outside the body to send radiation toward the cancer.     -  Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer.           The way the radiation therapy is given depends on the type and stage of the cancer being treated and whether a biopsy was done before surgery to remove the tumor. External radiation therapy is used to treat Wilms tumor and other childhood kidney tumors.       Chemotherapy    Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is treatment using two or more anticancer drugs.    The way the chemotherapy is given depends on the type and stage of the cancer being treated. Systemic chemotherapy is used to treat Wilms tumor and other childhood kidney tumors.    Sometimes the tumor cannot be removed by surgery for one of the following reasons:            - The tumor is too close to important organs or blood vessels.     - The tumor is too large to remove.     - The cancer is in both kidneys.     - There is a blood clot in the vessels near the liver.     - The patient has trouble breathing because cancer has spread to the lungs.            In this case, a biopsy is done first. Then chemotherapy is given to reduce the size of the tumor before surgery, in order to save as much healthy tissue as possible and lessen problems after surgery. This is called neoadjuvant chemotherapy. Radiation therapy is given after surgery.    See Drugs Approved for Wilms Tumor and Other Childhood Kidney Cancers for more information.       Biologic therapy     Biologic therapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy.     Interferon and interleukin-2 (IL-2) are types of biologic therapy used to treat childhood renal cell cancer. Interferon affects the division of cancer cells and can slow tumor growth. IL-2 boosts the growth and activity of many immune cells, especially lymphocytes (a type of white blood cell). Lymphocytes can attack and kill cancer cells.       High-dose chemotherapy with stem cell rescue     High-dose chemotherapy with stem cell rescue is a method of giving high doses of chemotherapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These re-infused stem cells grow into (and restore) the body's blood cells.    High-dose chemotherapy with stem cell rescue may be used to treat recurrent Wilms tumor.
                
                
                    Follow-up tests may be needed.
                    Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.   Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.
                
		        
		            Treatment Options for Wilms Tumor
		            
		                
		                    Stage I Wilms Tumor
		                    Treatment of stage I Wilms tumor with favorable histology may include:         -  Nephrectomy with removal of lymph nodes, followed by combination chemotherapy.    - A clinical trial of nephrectomy only.        Treatment of stage I anaplastic Wilms tumor may include:         - Nephrectomy with removal of lymph nodes followed by combination chemotherapy and radiation therapy to the flank area (either side of the body between the ribs and hipbone).        Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I Wilms tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website.
		                
		                
		                    Stage II Wilms Tumor
		                    Treatment of stage II Wilms tumor with favorable histology may include:         -  Nephrectomy with removal of lymph nodes, followed by combination chemotherapy.        Treatment of stage II anaplastic Wilms tumor may include:         - Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy.        Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage II Wilms tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website.
		                
		                
		                    Stage III Wilms Tumor
		                    Treatment of stage III Wilms tumor with favorable histology may include:         -  Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy.        Treatment of stage III anaplastic Wilms tumor may include:         - Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy.    - Combination chemotherapy followed by nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen.        Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III Wilms tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website.
		                
		                
		                    Stage IV Wilms Tumor
		                    Treatment of stage IV Wilms tumor with favorable histology may include:         -  Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy. If cancer has spread to other parts of the body, patients will also receive radiation therapy to those areas.         Treatment of stage IV anaplastic Wilms tumor may include:          - Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy. If cancer has spread to other parts of the body, patients will also receive radiation therapy to those areas.    - Combination chemotherapy given before nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen. If cancer has spread to other parts of the body, patients will also receive radiation therapy to those areas.        Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage IV Wilms tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website.
		                
		                
		                    Stage V Wilms Tumor and patients at high risk of developing bilateral Wilms tumor
		                    Treatment of stage V Wilms tumor may be different for each patient and may include:         -  Combination chemotherapy to shrink the tumor, followed by repeat imaging at 4 to 8 weeks to decide on further therapy (partial nephrectomy, biopsy, continued chemotherapy, and/or radiation therapy).    - A biopsy of the kidneys is followed by combination chemotherapy to shrink the tumor. A second surgery is done to remove as much of the cancer as possible. This may be followed by more chemotherapy and/or radiation therapy if cancer remains after surgery.        If a kidney transplant is needed because of kidney problems, it is delayed until 1 to 2 years after treatment is completed and there are no signs of cancer.   Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage V Wilms tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website.
		                
				        
				            Treatment Options for Other Childhood Kidney Tumors
				            
				                
				                    Renal Cell Cancer (RCC)
				                    Treatment of renal cell cancer usually includes:         -  Surgery, which may be:                -  nephrectomy with removal of lymph nodes; or      -  partial nephrectomy with removal of lymph nodes.             -  Biologic therapy (interferon and interleukin-2) for cancer that has spread to other parts of the body.        See the PDQ summary about Renal Cell Cancer Treatment for more information.   Check the list of NCI-supported cancer clinical trials that are now accepting patients with renal cell carcinoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website.
				                
				                
				                    Rhabdoid Tumor of the Kidney
				                    There is no standard treatment for rhabdoid tumor of the kidney. Treatment may include:         - A combination of surgery, chemotherapy, and/or radiation therapy.    - A clinical trial of targeted therapy (tazemetostat).        Check the list of NCI-supported cancer clinical trials that are now accepting patients with rhabdoid tumor of the kidney. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website.
				                
				                
				                    Clear Cell Sarcoma of the Kidney
				                    Treatment of clear cell sarcoma of the kidney may include:         -  Nephrectomy with removal of lymph nodes followed by combination chemotherapy and radiation therapy to the abdomen.    - A clinical trial of a new treatment.        Check the list of NCI-supported cancer clinical trials that are now accepting patients with clear cell sarcoma of the kidney. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website.
				                
				                
				                    Congenital Mesoblastic Nephroma
				                    Treatment for congenital mesoblastic nephroma usually includes:          -  Surgery that may be followed by chemotherapy.    - A clinical trial of targeted therapy (LOXO-101 or entrectinib).        Check the list of NCI-supported cancer clinical trials that are now accepting patients with congenital mesoblastic nephroma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website.
				                
				                
				                    Ewing Sarcoma of the Kidney
				                    There is no standard treatment for Ewing sarcoma of the kidney. Treatment may include:         - A combination of surgery, chemotherapy, and radiation therapy.        It may also be treated in the same way that Ewing sarcoma is treated. See the PDQ summary about Ewing Sarcoma Treatment for more information.   Check the list of NCI-supported cancer clinical trials that are now accepting patients with Ewing sarcoma/peripheral primitive neuroectodermal tumor (PNET). For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website.
				                
				                
				                    Primary Renal Myoepithelial Carcinoma
				                    There is no standard treatment for primary renal myoepithelial carcinoma. Treatment may include:         - A combination of surgery, chemotherapy, and radiation therapy.
				                
				                
				                    Cystic Partially Differentiated Nephroblastoma
				                    Treatment of cystic partially differentiated nephroblastoma may include:         -  Surgery that may be followed by chemotherapy.
				                
				                
				                    Multilocular Cystic Nephroma
				                    Treatment of multilocular cystic nephroma usually includes:         -  Surgery.
				                
				                
				                    Primary Renal Synovial Sarcoma
				                    Treatment of primary renal synovial sarcoma usually includes:         -  Chemotherapy.
				                
				                
				                    Anaplastic Sarcoma of the Kidney
				                    There is no standard treatment for anaplastic sarcoma of the kidney. Treatment is usually the same treatment given for anaplastic Wilms tumor.
				                
				                
				                    Nephroblastomatosis (Diffuse Hyperplastic Perilobar Nephroblastomatosis)
				                    The treatment of nephroblastomatosis depends on the following:         - Whether the child has abnormal groups of cells in one or both kidneys.    - Whether the child has Wilms tumor in one kidney and groups of abnormal cells in the other kidney.        Treatment of nephroblastomatosis may include:         -  Chemotherapy followed by nephrectomy. Sometimes a partial nephrectomy may be done to keep as much kidney function as possible.
(Source: CancerGov)