Stage I Wilms Tumor
Stage II Wilms Tumor
Stage III Wilms Tumor
Stage IV Wilms Tumor
Stage V Wilms Tumor
Inoperable Tumors
Clear Cell Sarcoma of the Kidney
Rhabdoid Tumor of the Kidney
Neuroepithelial Tumor of the Kidney
Mesoblastic Nephroma
Renal Cell Cancer
Diffuse Hyperplastic Perilobar Nephroblastomatosis
Recurrent Wilms Tumor and Other Childhood Kidney Tumors

A link to a list of current clinical trials is included for each treatment section. For some types or stages of cancer, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you.

Treatment of stage I Wilms tumor with favorable histology may include the following:

• Nephrectomy with lymph node removal followed by combination chemotherapy.
• A clinical trial of nephrectomy with lymph node removal, followed by observation or new combinations of chemotherapy.

Treatment of stage I anaplastic Wilms tumor may include the following:

• Nephrectomy with lymph node removal followed by combination chemotherapy.
• A clinical trial of nephrectomy with lymph node removal, followed by combination chemotherapy and radiation therapy.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage I Wilms tumor ¹.

Treatment of stage II Wilms tumor with favorable histology may include the following:

• Nephrectomy with removal of lymph nodes, followed by combination chemotherapy.
• A clinical trial of nephrectomy with removal of lymph nodes, followed by new combinations of chemotherapy.

Treatment of stage II anaplastic Wilms tumor may include the following:

• Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy.
• A clinical trial of nephrectomy with removal of lymph nodes, followed by new combinations of chemotherapy and radiation therapy.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage II Wilms tumor ².

Treatment of stage III Wilms tumor with favorable histology may include the following:

• Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy.
• A clinical trial of nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and new combinations of chemotherapy.

Treatment of stage III anaplastic Wilms tumor may include the following:

• Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy.
• A clinical trial of nephrectomy with removal of lymph nodes, followed by new combinations of chemotherapy and radiation therapy.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage III Wilms tumor ³.

Treatment of stage IV Wilms tumor with favorable histology may include the following:

• Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy. If cancer has spread to the lungs, patients will also receive radiation therapy to the lungs.
• A clinical trial of chemotherapy for cancer that has spread only to the lungs. If tumors remain after chemotherapy, radiation therapy may be given.
• A clinical trial of chemotherapy and radiation therapy to places where cancer has spread.

Treatment of stage IV anaplastic Wilms tumor may include nephrectomy with removal of lymph nodes, followed by new combinations of chemotherapy and radiation therapy.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage IV Wilms tumor ⁴.

Treatment of stage V Wilms tumor may be different for each patient. Treatment is usually chemotherapy to shrink the tumor, followed by surgery 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.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage V Wilms tumor ⁵.

Sometimes the tumor is inoperable (cannot be removed by surgery) because it is too close to important organs or blood vessels or because it is too large to remove. In this case, chemotherapy may be given to reduce the size of the tumor so it may be removed in surgery. If the tumor does not shrink enough after chemotherapy, radiation therapy may be given to shrink it further so that surgery may be done. This may be followed by more chemotherapy and/or more radiation therapy.

There is no standard treatment for clear cell sarcoma of the kidney. Treatment is usually within a clinical trial and may include nephrectomy with lymph node removal, with or without radiation therapy to the abdomen, and new combinations of chemotherapy.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with clear cell sarcoma of the kidney ⁶.

There is no standard treatment for rhabdoid tumor of the kidney. Treatment may be within a clinical trial and may include new combinations of chemotherapy and radiation therapy.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with rhabdoid tumor of the kidney ⁷.

There is no standard treatment for neuroepithelial tumor of the kidney. Treatment is usually within a clinical trial. It may be treated in the same way that Ewing family of tumors or primitive neuroectodermal tumors are treated. See the PDQ summary on Ewing Family of Tumors Treatment ⁸ for more information.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with peripheral primitive neuroectodermal tumor of the kidney ⁹.

Treatment for mesoblastic nephroma is usually surgery with or without chemotherapy.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with congenital mesoblastic nephroma ¹⁰.

Treatment of renal cell cancer is usually nephrectomy with removal of lymph nodes. If cancer has spread, treatment may include biologic therapy or surgery to remove the primary tumor. See the PDQ summary on Renal Cell Cancer Treatment ¹¹ for more information.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with childhood renal cell carcinoma ¹².

Treatment of diffuse hyperplastic perilobar nephroblastomatosis (DHPLN) may include nephrectomy with lymph node removal followed by combination chemotherapy. A partial nephrectomy may be done to keep as much kidney function as possible.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with diffuse hyperplastic perilobar nephroblastomatosis ¹³.

Treatment of recurrent Wilms tumor may be within a clinical trial of combination chemotherapy, surgery, and radiation therapy, with or without stem cell transplant, using the child's own blood stem cells.

Treatment of recurrent clear cell sarcoma, rhabdoid tumor, and neuroepithelial tumor of the kidney is usually within a clinical trial.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent Wilms tumor and other childhood kidney tumors ¹⁴.

Glossary Terms

The area of the body that contains the pancreas, stomach, intestines, liver, gallbladder, and other organs.

A term used to describe cancer cells that divide rapidly and have little or no resemblance to normal cells.

Treatment to boost or restore the ability of the immune system to fight cancer, infections, and other diseases. Also used to lessen certain side effects that may be caused by some cancer treatments. Agents used in biological therapy include monoclonal antibodies, growth factors, and vaccines. These agents may also have a direct antitumor effect. Also called immunotherapy, biotherapy, biological response modifier therapy, and BRM therapy.

A tissue with red blood cells, white blood cells, platelets, and other substances suspended in fluid called plasma. Blood takes oxygen and nutrients to the tissues, and carries away wastes.

A tube through which the blood circulates in the body. Blood vessels include a network of arteries, arterioles, capillaries, venules, and veins.

A term for diseases in which abnormal cells divide without control. Cancer cells can invade nearby tissues and can spread to other parts of the body through the blood and lymph systems. There are several main types of cancer. Carcinoma is cancer that begins in the skin or in tissues that line or cover internal organs. Sarcoma is cancer that begins in bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue. Leukemia is cancer that starts in blood-forming tissue such as the bone marrow, and causes large numbers of abnormal blood cells to be produced and enter the blood. Lymphoma and multiple myeloma are cancers that begin in the cells of the immune system. Central nervous system cancers are cancers that begin in the tissues of the brain and spinal cord.

Treatment with drugs that kill cancer cells.

A rare type of kidney cancer, in which the inside of the cells look clear when viewed under a microscope. Clear cell sarcoma can spread from the kidney to other organs, most commonly the bone, but also including the lungs, brain, and soft tissues of the body.

A type of research study that tests how well new medical approaches work in people. These studies test new methods of screening, prevention, diagnosis, or treatment of a disease. Also called a clinical study.

Treatment using more than one anticancer drug.

Widely spread; not localized or confined.

A group of cancers that includes Ewing tumor of bone (ETB or Ewing sarcoma of bone), extraosseous Ewing (EOE) tumors, primitive neuroectodermal tumors (PNET or peripheral neuroepithelioma), and Askin tumors (PNET of the chest wall). These tumors all come from the same type of stem cell. Also called EFTs.

The study of tissues and cells under a microscope.

An abnormal increase in the number of cells in an organ or tissue.

Describes a condition that cannot be treated by surgery.

One of a pair of organs in the abdomen. Kidneys remove waste from the blood (as urine), produce erythropoietin (a substance that stimulates red blood cell production), and play a role in blood pressure regulation.

One of a pair of organs in the chest that supplies the body with oxygen, and removes carbon dioxide from the body.

A rounded mass of lymphatic tissue that is surrounded by a capsule of connective tissue. Lymph nodes filter lymph (lymphatic fluid), and they store lymphocytes (white blood cells). They are located along lymphatic vessels. Also called lymph gland.

Surgery to remove a kidney or part of a kidney. In a partial nephrectomy, part of one kidney or a tumor is removed, but not an entire kidney. In a simple nephrectomy, one kidney is removed. In a radical nephrectomy, an entire kidney, nearby adrenal gland and lymph nodes, and other surrounding tissue are removed. In a bilateral nephrectomy, both kidneys are removed.

Having to do with tissue made up of sensory cells, such as tissue found in the ear, nose, and tongue.

Closely monitoring a patient's condition but withholding treatment until symptoms appear or change. Also called watchful waiting.

A part of the body that performs a specific function. For example, the heart is an organ.

Surgery to remove part of one kidney or a kidney tumor, but not an entire kidney.

PDQ is an online database developed and maintained by the National Cancer Institute. Designed to make the most current, credible, and accurate cancer information available to health professionals and the public, PDQ contains peer-reviewed summaries on cancer treatment, screening, prevention, genetics, complementary and alternative medicine, and supportive care; a registry of cancer clinical trials from around the world; and directories of physicians, professionals who provide genetics services, and organizations that provide cancer care. Most of this information, and more specific information about PDQ, can be found on the NCI's Web site at http://www.cancer.gov/cancertopics/pdq. Also called Physician Data Query.

The original tumor.

One of a group of cancers that develop from the same type of early cells, and share certain biochemical and genetic features. Some primitive neuroectodermal tumors develop in the brain and central nervous system (CNS-PNET), and others develop in sites outside of the brain such as the limbs, pelvis, and chest wall (peripheral PNET). Also called PNET.

The use of high-energy radiation from x-rays, gamma rays, neutrons, protons, and other sources to kill cancer cells and shrink tumors. Radiation may come from a machine outside the body (external-beam radiation therapy), or it may come from radioactive material placed in the body near cancer cells (internal radiation therapy). Systemic radiation therapy uses a radioactive substance, such as a radiolabeled monoclonal antibody, that travels in the blood to tissues throughout the body. Also called radiotherapy and irradiation.

Cancer that has recurred (come back), usually after a period of time during which the cancer could not be detected. The cancer may come back to the same place as the original (primary) tumor or to another place in the body. Also called recurrence.

The most common type of kidney cancer. It begins in the lining of the renal tubules in the kidney. The renal tubules filter the blood and produce urine. Also called hypernephroma.

A malignant tumor of either the central nervous system (CNS) or the kidney. Malignant rhabdoid tumors of the CNS often have an abnormality of chromosome 22. These tumors usually occur in children younger than 2 years.

The extent of a cancer in the body. Staging is usually based on the size of the tumor, whether lymph nodes contain cancer, and whether the cancer has spread from the original site to other parts of the body.

The tumor was completely removed by surgery and all of the following are true: (1) cancer was found only in the kidney and did not spread to blood vessels of the kidney; (2) the outer layer of the kidney did not break open; (3) the tumor did not break open; (4) a biopsy of the tumor was not done; and (5) no cancer cells were found at the edges of the area where the tumor was removed.

Cancer spread out of the kidney to nearby soft tissue or to blood vessels of the kidney and was completely removed by surgery. No cancer cells were found at the edges of the area where the cancer was removed.

Cancer remains in the abdomen after surgery and at least one of the following is true: (1) cancer has spread to lymph nodes in the abdomen or pelvis (the part of the body between the hips); (2) cancer has spread to or through the surface of the peritoneum (the layer of tissue that lines the abdominal cavity and covers most organs in the abdomen); (3) a biopsy of the tumor was done during surgery to remove it; (4) the tumor was removed in more than one piece.

Cancer has spread through the blood to organs such as the lungs, liver, bone, or brain, or to lymph nodes outside of the abdomen and pelvis (the part of the body between the hips).

Cancer cells are found in both kidneys when the disease is first diagnosed. Each kidney is staged separately as I, II, III, or IV.

In medicine, treatment that experts agree is appropriate, accepted, and widely used. Health care providers are obligated to provide patients with standard therapy. Also called standard of care or best practice.

A cell from which other types of cells develop. For example, blood cells develop from blood-forming stem cells.

A method of replacing immature blood-forming cells that were destroyed by cancer treatment. The stem cells are given to the person after treatment to help the bone marrow recover and continue producing healthy blood cells.

A procedure to remove or repair a part of the body or to find out whether disease is present. An operation.

An abnormal mass of tissue that results when cells divide more than they should or do not die when they should. Tumors may be benign (not cancerous), or malignant (cancerous). Also called neoplasm.

A disease in which malignant (cancer) cells are found in the kidney, and may spread to the lungs, liver, or nearby lymph nodes. Wilms tumor usually occurs in children younger than 5 years old.