Wilms' Tumor and Other Childhood Kidney Tumors

Description

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 childâs 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.

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), 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.

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Stage Explanation

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. For Wilms' tumors, the stage is based on the amount of tumor that remains after the patient has had surgery to look at or remove the tumor. The following stages are used for Wilms' tumor:

Stage I

The tumor was completely removed by surgery and all of the following are true:

• Cancer was found only in the kidney and did not spread to blood vessels of the kidney.

• The outer layer of the kidney did not break open.

• The tumor did not break open.

• A biopsy of the tumor was not done.

• No cancer cells are found at the edges of the area where the tumor was removed.

Stage II

Cancer has spread deeper into other parts of the kidney and to nearby blood vessels, but was completely removed by surgery. No cancer cells are found at the edges of the area where the tumor was removed.

Stage III

Cancer remains in the abdomen after surgery to remove the tumor but no cancer remains in or on the blood vessels. Any 1 of the following may be true:

• Cancer has spread to lymph nodes in the abdomen or pelvis (the part of the body between the hips).

• Cancer has spread through the peritoneum (the layer of tissue that lines the abdominal cavity and covers most organs in the abdomen) or is found on the surface of the peritoneum.

• Cancer cells are found at the edges of the area where the tumor was removed.

• Cancer has spread to nearby tissues, where it cannot be completely removed by surgery.

• Cancer cells are found in the abdominal cavity (tumor cells spilled there before or during surgery).

• A biopsy of the tumor was done before surgery to remove it.

• The tumor was removed in more than 1 piece.

Stage IV

Cancer has spread through the blood to the lungs, liver, bone, or brain, or to lymph nodes outside of the abdomen and pelvis.

Stage V

Cancer cells are found in both kidneys when the disease is first diagnosed. The cancer in each kidney will be staged as I, II, III, or IV.

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.

Favorable and Anaplastic Histology

In Wilms' tumor, how the cancer cells look under a microscope (histology) is also very important for describing stage and determining treatment options. The cancer cells can be of favorable histology or anaplastic histology. Anaplastic means the cancer cells divide rapidly and look very different from normal cells. Anaplastic tumors may be focal (in one area) or diffuse (spread widely throughout an area).

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Treatment Option Overview

How Wilms' tumor and other childhood kidney tumors are treated

There are treatments for 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.

Some cancer treatments cause side effects that continue or appear years after cancer treatment has ended. These are called late effects. It is important that parents of children who are treated for cancer know about the possible late effects caused by certain treatments. After several years, some patients develop another form of cancer as a result of their treatment with chemotherapy and radiation. Refer to the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information. 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 unwanted side effects. 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. If you want more information, call the Cancer Information Service at 1-800-4- CANCER (1-800-422-6237); TTY at 1-800-332-8615.

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Stage I Wilms' Tumor

Treatment for stage I Wilms' tumor with either favorable or anaplastic histology will probably be surgery to remove the kidney and some of the lymph nodes near the kidney, followed by chemotherapy.

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Stage II Wilms' Tumor

Treatment for stage II Wilms' tumor depends on the histology of the cancer.

If your child has a favorable histology tumor, 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 anaplastic histology tumor, treatment will probably be surgery to remove the kidney followed by radiation therapy plus chemotherapy.

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Stage III Wilms' Tumor

Treatment of both favorable and anaplastic stage III Wilms' tumors 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.

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Stage IV Wilms' Tumor

Treatment of both favorable and anaplastic stage IV Wilms' tumors 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 also receive radiation therapy to the lungs.

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Stage V Wilms' Tumor

Your child's doctor will probably do a biopsy of the cancer by taking out a piece of the cancer in both kidneys and removing some of the lymph nodes around the kidney to see whether they contain cancer. Following the biopsy, chemotherapy will be given to shrink the cancer. A second surgery will remove as much of the cancer as possible, while leaving as much of the kidneys as possible. Surgery may be followed by more chemotherapy and/or radiation therapy.

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Inoperable Wilms' Tumors

Sometimes the cancer is inoperable (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 perform a biopsy only and then give chemotherapy with or without radiation therapy. Once the cancer has become smaller, surgery to remove part or all of the tumor may be done, followed by more chemotherapy and radiation therapy.

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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.

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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. Treatment is usually a clinical trial.

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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.

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Recurrent Wilms' Tumor and Other Childhood Kidney Tumors

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.

Clinical trials are evaluating new treatments, such as new chemotherapy drugs and combinations, and very high doses of chemotherapy followed by bone marrow reinfusion.

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Changes to This Summary (12/19/2005)

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

Changes were made to this summary to match those made to the health professional version and links to the NCI Dictionary of Cancer Terms were added.

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To Learn More

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About PDQ

PDQ is a comprehensive cancer database available on NCI's Web site.

PDQ is the National Cancer Institute's (NCI's) comprehensive cancer information database. Most of the information contained in PDQ is available online at NCI's Web site. PDQ is provided as a service of the NCI. The NCI is part of the National Institutes of Health, the federal government's focal point for biomedical research.

PDQ contains cancer information summaries.

The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries are available in two versions. The health professional versions provide detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions provide current and accurate cancer information.

The PDQ cancer information summaries are developed by cancer experts and reviewed regularly.

Editorial Boards made up of experts in oncology and related specialties are responsible for writing and maintaining the cancer information summaries. The summaries are reviewed regularly and changes are made as new information becomes available. The date on each summary ("Date Last Modified") indicates the time of the most recent change.

PDQ also contains information on clinical trials.

In the United States, about two-thirds of children with cancer are treated in a clinical trial at some point in their illness. A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about new treatments, the risks involved, and how well they do or do not work. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard."

Listings of clinical trials are included in PDQ and are available online at NCI's Web site. Descriptions of the trials are available in health professional and patient versions. For additional help in locating a childhood cancer clinical trial, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.

The PDQ database contains listings of groups specializing in clinical trials.

The Children's Oncology Group (COG) is the major group that organizes clinical trials for childhood cancers in the United States. Information about contacting COG is available on the NCI Web site or from the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.

The PDQ database contains listings of cancer health professionals and hospitals with cancer programs.

Because cancer in children and adolescents is rare, the majority of children with cancer are treated by health professionals specializing in childhood cancers, at hospitals or cancer centers with special facilities to treat them. The PDQ database contains listings of health professionals who specialize in childhood cancer and listings of hospitals with cancer programs. For help locating childhood cancer health professionals or a hospital with cancer programs, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.

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