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Wilms' Tumor (Childhood) General Information What is Wilms' tumor? Wilms' tumor is a disease in which cancer (malignant) cells are found in certain parts of the kidney. The kidneys are a "matched" pair of organs found on either side of the backbone. The kidneys are shaped like a kidney bean. Inside each kidney are tiny tubes that filter and clean the blood, taking out unneeded products, and making urine. The urine made by the kidneys passes through a tube called a ureter into the bladder where it is held until it is passed from the body. Wilms' tumor is curable in the majority of affected children. If your child has symptoms, your child's doctor will usually feel your child's abdomen for lumps and run blood and urine tests. The doctor may order a special x-ray called an intravenous pyelogram. During this test, a dye containing iodine is injected into your child's bloodstream. This allows your child's doctor to see the kidney more clearly on the x-ray. Your child's doctor may also do an ultrasound, which uses sound waves to make a picture, or a special x-ray called a computed tomographic scan to look for lumps in the kidney. A special scan called magnetic resonance imaging, which uses magnetic waves to make a picture, may also be done. Chest and bone x-rays may also be taken. If abnormal tissue is found, your child's doctor will need to cut out a small piece and look at it under the microscope to see if there are any cancer cells. This is called a biopsy. In Wilms' tumor, how the cancer cells look under a microscope (histology) is also very important. The cancer cells can be of favorable histology or unfavorable histology (which includes focal and diffuse anaplasia) Your child's chance of recovery (prognosis) and choice of treatment depend on the stage of your child's cancer (whether it is just in the kidney only or has spread to other places in the body), how the cancer cells look under a microscope (histology), tumor size, and your child's age and general health. What Are Other Childhood Kidney Tumors? Clear cell sarcoma of the kidney, rhabdoid tumor of the kidney, and neuroepithelial tumor of the kidney are childhood kidney tumors unrelated to Wilms' tumor. The treatment of these tumors is different from that of Wilms' tumor but because of their location near the kidneys, they have been treated on clinical trials developed by the National Wilms' Tumor Study Group. Stage Information Stages of Wilms' Tumor: Once Wilms' tumor has been found, more tests will be done to find out if cancer cells have spread from the kidney to other parts of the body. This is called staging. Your child's doctor needs to know the stage of the disease to plan treatment. The following stages are used for Wilms' tumor: Stage I Cancer is found only in the kidney and can be completely removed by surgery. Stage II Cancer has spread beyond the kidney, to fat or soft tissue or blood vessels. The cancer can be completely removed by surgery. Stage III Cancer has spread within the abdomen and cannot be completely removed by surgery. The cancer may have spread to the lymph nodes (small bean-shaped structures found throughout the body that produce and store infection-fighting cells) near the kidney, blood vessels, or the peritoneum (tissue that lines the abdomen and covers most organs in the abdomen). Stage IV Cancer has spread to other parts of the body, such as the lungs, liver, bone, and/or brain. Stage V Cancer cells are found in both kidneys when the disease is first diagnosed. Recurrent Recurrent disease means that the cancer has come back (recurred) after it has been treated. It may come back where it started or in another part of the body. Treatment Option Overview How Wilms' tumor and other childhood kidney tumors are treated: There are treatments for all patients with Wilms' tumor. Three kinds of treatment are used: -surgery (taking out the cancer in an operation) -chemotherapy (using drugs to kill cancer cells) -radiation therapy (using high-dose x-rays or other high-energy x-rays to kill cancer cells) Surgery is a common treatment for Wilms' tumor. Your doctor may take out the cancer using one of the following: Partial nephrectomy removes the cancer and part of the kidney around the cancer. This operation is usually used only in special cases, such as when the other kidney is damaged or has already been removed. Simple nephrectomy removes the whole kidney. The kidney on the other side of the body can take over filtering blood. Radical nephrectomy removes the whole kidney with the tissues around it. Some lymph nodes in the area may also be removed. Chemotherapy uses drugs to kill cancer cells. Chemotherapy may be taken by pill, or it may be put into the body by a needle in a vein or muscle. Chemotherapy is called a systemic treatment because the drugs enter the bloodstream, travel through the body, and can kill cancer cells throughout the body. Chemotherapy given after an operation to remove the tumor is called adjuvant therapy. When very high doses of chemotherapy are used to kill cancer cells, these high doses can destroy the blood-forming tissue in the bones (the bone marrow). If very high doses of chemotherapy are needed to treat the cancer, bone marrow may be taken from the bones before therapy and frozen until it is needed. Following chemotherapy, the bone marrow is given back through a needle in a vein. This is called autologous bone marrow reinfusion. Radiation therapy uses x-rays or other high-energy rays to kill cancer cells and shrink tumors. Radiation for Wilms' tumor usually comes from a machine outside the body (external radiation therapy). Radiation may be used before or after surgery and/or chemotherapy. After several years, some patients develop another form of cancer as a result of their treatment with chemotherapy and radiation. Clinical trials are ongoing to determine if lower doses of chemotherapy and radiation can be used. Treatment of Wilms' tumor by stage: Treatments for Wilms' tumor depend on the stage of your child's disease, the histology (cell type), and your child's age and general health. Your child may receive treatment that is considered standard based on its effectiveness in a number of patients in past studies, or you may choose to have your child take part in a clinical trial. Not all patients are cured with standard therapy and some standard treatments may have more side effects than are desired. For these reasons, clinical trials are designed to test new treatments and to find better ways to treat cancer patients. Clinical trials are ongoing in most parts of the country for most stages of Wilms' tumor. Stage I Wilms' Tumor If your child has Stage I Wilms's tumor or anaplastic cancer, your child's treatment will probably be surgery to remove the kidney and some of the lymph nodes near the kidney followed by chemotherapy. Stage II Wilms' Tumor Your child's treatment depends on the histology of the cancer. If your child has a favorable histology tumor, your child's treatment will probably be surgery to remove the kidney and some of the lymph nodes near the kidney followed by chemotherapy. If your child has an unfavorable histology tumor (anaplasia), your child's treatment will probably be surgery to remove the kidney followed by radiation therapy plus chemotherapy. Stage III Wilms' Tumor Whether your child has a favorable or unfavorable histology tumor (anaplasia), his or her treatment will probably be surgery to remove the kidney and some of the lymph nodes near the kidney followed by radiation therapy to the abdomen and chemotherapy. Sometimes the cancer cannot be removed during surgery because it is too close to important organs or blood vessels or because it is too large to remove. In this case, the doctor may only perform a biopsy and then give chemotherapy with or without radiation therapy. Once the cancer has become smaller, surgery can be performed, followed by additional chemotherapy and radiation therapy. Stage IV Wilms' Tumor Whether your child has a favorable or unfavorable histology tumor (anaplasia), his or her treatment will probably be surgery to remove the kidney and some of the lymph nodes near the kidney followed by radiation therapy to the abdomen and chemotherapy. Patients whose cancer has spread to the lungs will receive radiation therapy to the lungs. Stage V Wilms' Tumor Your child's doctor will probably take out a piece of the cancer in both kidneys and remove some of the lymph nodes around the kidney to see whether they contain cancer. following surgery, chemotherapy will be given to shrink the cancer. A second surgery will remove as much of the cancer while leaving as much of the kidneys as possible. Surgery may be followed by more chemotherapy and/or radiation therapy. Clear Cell Sarcoma of the Kidney Clear cell sarcoma of the kidney is a primary kidney tumor. It is not a type of Wilms' tumor. This tumor can spread to the lung, bone, brain, and soft tissue. If your child has clear cell sarcoma of the kidney, he or she may be treated with surgery to remove the kidney followed by radiation therapy to the abdomen and lung (if cancer has spread to the lung) followed by chemotherapy. Rhabdoid Tumor of the Kidney Rhabdoid tumor of the kidney is a type of cancer that grows and spreads quickly. At diagnosis, children are usually younger than 1 year and may have fever, blood in the urine, and advanced cancer. This tumor type tends to spread to the lungs and the brain. If your child has rhabdoid tumor of the kidney, he or she will probably undergo surgery to remove the kidney followed by chemotherapy. Neuroepithelial Tumor of the Kidney Neuroepithelial tumors of the kidney are tumors that grow and spread quickly. At diagnosis, these tumors have often spread to the outer layer of the kidney, the veins of the kidney, and to other parts of the body. If your child has neuroepithelial tumors of the kidney, treatment on an Ewing's/primitive neuroectodermal tumors (PNET) clinical trial should be considered. Recurrent Wilms' Tumor If your child's cancer comes back (recurs), treatment depends on the treatment he or she received before, how much time has passed since the first cancer was treated, the histology of the cancer, and where the cancer came back. Your child's treatment may be surgery to remove the cancer plus radiation therapy and chemotherapy. Clinical trials are evaluating new treatments, such as new chemotherapy drugs and combinations, and very high doses of chemotherapy followed by bone marrow reinfusion. The information on this page was obtained from the National Cancer Institute. The National Cancer Institute provides accurate, up-to-date information on many types of cancer, information on clinical trials, resources for people dealing with cancer, and information for researchers and health professionals. The National Cancer Institute is in no way affiliated with the Mary Stolfa Cancer Foundation. The information on this web site is provided for general information only. It is not intended as medical advice, and should not be relied upon as a substitute for consultations with qualified health professionals who are familiar with your individual medical needs. The MSCF disclaims all obligations and liabilities for damages arising from the use or attempted use of the information, including but not limited to direct, indirect, special, and consequential damages, attorneys' and experts' fees and court costs. 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