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Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ)

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General Information About Wilms Tumor and Other Childhood Kidney Tumors
Stages of Wilms Tumor and Other Childhood Kidney Tumors
Recurrent Wilms Tumor and Other Childhood Kidney Tumors
Treatment Option Overview
Treatment Options for Wilms Tumor and Other Childhood Kidney Tumors
To Learn More About Wilms Tumor and Other Childhood Kidney Tumors
Changes to This Summary (05/22/2014)
About This PDQ Summary
Get More Information From NCI

General Information About Wilms Tumor and Other Childhood Kidney Tumors

Wilms tumor and other childhood kidney tumors are diseases in which malignant (cancer) cells form in the tissues of the kidney.

Wilms tumor

Wilms tumor and other kidney tumors are diseases in which malignant (cancer) cells are found in the kidney. In Wilms tumor, one or more tumors may be found in one or both kidneys. There are two kidneys, one on each side of the backbone, above the waist. Tiny tubules in the kidneys filter and clean the blood, taking out waste products and making urine. The urine passes from each kidney through a long tube called a ureter into the bladder. The bladder holds the urine until it is passed from the body.

Anatomy of the female urinary system; drawing shows a front view of the right and left kidneys, the ureters,  urethra, and bladder filled with urine. The inside of the left kidney shows the renal pelvis. An inset shows the renal tubules and urine. The spine, adrenal glands, and uterus are also shown.Anatomy of the female urinary system showing the kidneys, adrenal glands, ureters, bladder, and urethra. Urine is made in the renal tubules and collects in the renal pelvis of each kidney. The urine flows from the kidneys through the ureters to the bladder. The urine is stored in the bladder until it leaves the body through the urethra.

Wilms tumor may spread to the lungs, liver, or nearby lymph nodes.

Nephroblastomatosis is a condition in which abnormal tissue grows on the outer part of one or both kidneys. Children with this condition are at risk for developing a type of Wilms tumor that grows quickly. Frequent follow-up testing is important for at least 7 years after the child is treated.

Other kidney tumors

Other childhood kidney tumors, which are diagnosed and treated in different ways, include:

Having certain genetic syndromes or birth defects can increase the risk of Wilms tumor.

Anything that increases the 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 to your child's doctor if you think your child may be at risk.

Wilms tumor may be part of a genetic syndrome that affects growth or development. A genetic syndrome is a set of symptoms or conditions that occur together and is usually caused by abnormal genes. Certain birth defects can also increase a child's risk for developing Wilms tumor. The following genetic syndromes and birth defects have been linked to Wilms tumor:

Children with these genetic syndromes and birth defects should be screened for Wilms tumor every three months until at least age 8. An ultrasound test of the abdomen may be used for screening. Children with Beckwith-Wiedemann syndrome or hemihypertrophy are also screened for liver and adrenal tumors that are linked to these genetic syndromes. A test to check the alpha-fetoprotein level in the blood and an ultrasound of the abdomen are done until age 4 years. An ultrasound of the kidneys is done after age 4 years.

Some children develop Wilms tumor in both kidneys. These often appear when Wilms tumor is first diagnosed, but a Wilms tumor may also occur in the second kidney after the child is successfully treated for Wilms tumor in one kidney. A second tumor is much more likely to develop in the other kidney when a child's first Wilms tumor is diagnosed before age 12 months or when embryonic cells remain in the kidney.

Children at risk for developing a second Wilms tumor in the other kidney should be screened for Wilms tumor every three months for two to six years. An ultrasound test of the abdomen may be used for screening.

Having certain conditions may be associated with renal cell carcinoma.

Renal cell carcinoma may be related to the following conditions:

Signs of Wilms tumor and other childhood kidney tumors include a lump in the abdomen and blood in the urine.

These and other signs and symptoms may be caused by kidney tumors or by other conditions. Check with a doctor if your child has any of the following:

Tests that examine the kidney and the blood are used to detect (find) Wilms tumor and other childhood kidney tumors.

The following tests and procedures may be used:

Wilms tumor and other childhood kidney tumors are usually diagnosed and removed in surgery.

Once a kidney tumor is found, surgery is done to find out whether or not the tumor is cancer. If the tumor is only in the kidney, the surgeon will remove the whole kidney (nephrectomy). If there are tumors in both kidneys or if the tumor has spread outside the kidney, a piece of the tumor will be removed. In any case, a sample of tissue from the tumor is sent to a pathologist, who looks at it under a microscope to check for signs of cancer.

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis (chance of recovery) and treatment options depend on the following:

Wilms tumor is rare in adults.

Wilms tumor in adults is harder to cure than in children. When a Wilms tumor is found, tests should include checking lymph nodes for cancer.

Stages of Wilms Tumor and Other Childhood Kidney Tumors

Wilms tumors and other childhood kidney tumors are staged during surgery and with imaging tests.

The process used to find out if cancer has spread outside of the kidney 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.

For Wilms tumor, the stage is determined during the initial surgery and with the results from imaging tests. The following imaging tests may be done to see if cancer has spread to other places in the body:

There are three ways that cancer spreads in the body.

Cancer can spread through tissue, the lymph system, and the blood:

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.

The metastatic tumor is the same type of cancer as the primary tumor. For example, if Wilms tumor spreads to the lung, the cancer cells in the lung are actually Wilms tumor cells. The disease is metastatic Wilms tumor, not lung cancer.

In addition to the stages, Wilms tumors are described by their histology.

The histology (how the cells look under a microscope) of the tumor affects the prognosis and the treatment of Wilms tumor. The histology may be favorable or anaplastic (unfavorable). Tumors with a favorable histology have a better prognosis and respond better to chemotherapy than those with anaplastic histology. Tumor cells that are anaplastic divide rapidly and do not look like the type of cells they came from. Anaplastic tumors are harder to treat with chemotherapy than other Wilms tumors at the same stage.

The following stages are used for both favorable histology and anaplastic Wilms tumors:

Stage I

In stage I, the tumor was completely removed by surgery and all of the following are true:

Stage II

In stage II, the tumor was completely removed by surgery and no cancer cells were found at the edges of the area where the cancer was removed. Before the tumor was removed, one of the following was true:

Stage III

In stage III, cancer remains in the abdomen after surgery and at least one of the following is true:

Stage IV

In stage IV, 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.

Stage V and those at high risk of developing Wilms tumor

In stage V, cancer cells are found in both kidneys when the disease is first diagnosed.

Recurrent Wilms Tumor and Other Childhood Kidney Tumors

Recurrent cancer is cancer that has recurred (come back) after it has been treated.

Treatment Option Overview

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:

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:

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 ongoing to find out if lower doses of chemotherapy and radiation can be used.

Four types of standard treatment are used:

Surgery

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.

If cancer is found in 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 the kidney working.

Chemotherapy may be given before surgery to make the tumor smaller so less kidney tissue needs to be removed and there are fewer problems after surgery. This is called neoadjuvant chemotherapy.

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 cancer will come 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.

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 using two or more anticancer drugs.

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.

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 Web site.

Watchful waiting

Watchful waiting is closely monitoring a patient’s condition without giving any treatment until signs or symptoms appear or change.

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 re-infused stem cells grow into (and restore) the body's blood cells.

Targeted therapy

Targeted therapy is a treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Wilms tumor may be treated with a targeted therapy drug that stops cells from dividing and prevents the growth of new blood vessels that tumors need to grow.

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. This is sometimes called re-staging.

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 and Other Childhood Kidney Tumors

Stage I Wilms Tumor

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

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

Check for U.S. clinical trials from NCI's list of 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 Web site.

Stage II Wilms Tumor

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

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

Check for U.S. clinical trials from NCI's list of 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 Web site.

Stage III Wilms Tumor

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

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

Check for U.S. clinical trials from NCI's list of 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 Web site.

Stage IV Wilms Tumor

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

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

Check for U.S. clinical trials from NCI's list of 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 Web site.

Stage V Wilms Tumor and those at high risk of developing Wilms tumor

Treatment of stage V Wilms tumor may be different for each patient and may include:

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 or symptoms of cancer.

Check for U.S. clinical trials from NCI's list of 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 Web site.

Inoperable Tumors

Sometimes the tumor is inoperable (cannot be removed by surgery) because it is too close to important organs or blood vessels, too large to remove, or there is cancer in both kidneys. In this case, chemotherapy may be given to reduce the size of the tumor so as much tumor as possible can be removed in surgery. Radiation therapy is given after surgery.

Wilms Tumor in Adults

There is no standard treatment for adults with Wilms tumor. Pediatric surgeons and oncologists are often part of the team that plans treatment because the treatments used for children may also be used for adults.

Clear Cell Sarcoma of the Kidney

Treatment of clear cell sarcoma of the kidney may include the following:

Check for U.S. clinical trials from NCI's list of 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 Web site.

Rhabdoid Tumor of the Kidney

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

Check for U.S. clinical trials from NCI's list of 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 Web site.

Neuroepithelial Tumor of the Kidney

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 about Ewing Sarcoma Family of Tumors Treatment for more information.

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with peripheral primitive neuroectodermal 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 Web site.

Congenital Mesoblastic Nephroma

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

Check for U.S. clinical trials from NCI's list of 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 Web site.

Renal Cell Cancer

Treatment of renal cell cancer is usually nephrectomy with removal of lymph nodes. In some cases, treatment may be a partial nephrectomy with removal of lymph nodes. If cancer has spread, treatment may include biologic therapy. See the PDQ summary about Renal Cell Cancer Treatment for more information.

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with childhood 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 Web site.

Nephroblastomatosis

Treatment of nephroblastomatosis may include the following:

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with diffuse hyperplastic perilobar nephroblastomatosis. 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 Web site.

Recurrent Wilms Tumor and Other Childhood Kidney Tumors

Treatment of recurrent Wilms tumor may include the following:

Treatment of recurrent clear cell sarcoma of the kidney may include chemotherapy. Treatment of clear cell sarcoma of the kidney that has recurred in the brain may include chemotherapy, surgery, and radiation therapy.

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

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with recurrent Wilms tumor and other childhood kidney tumors. 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 Web site.

To Learn More About Wilms Tumor and Other Childhood Kidney Tumors

For more information from the National Cancer Institute about Wilms tumor and other childhood kidney tumors, see the following:

For more childhood cancer information and other general cancer resources, see the following:

Changes to This Summary (05/22/2014)

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.

Editorial changes were made to this summary.

About This PDQ Summary

About PDQ

Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. 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 come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish.

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Purpose of This Summary

This PDQ cancer information summary has current information about the treatment of Wilms tumor and other childhood kidney tumors. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.

Reviewers and Updates

Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Date Last Modified") is the date of the most recent change.

The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.

Clinical Trial Information

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 the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." 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.

Clinical trials are listed in PDQ and can be found online at NCI's Web site. Many cancer doctors who take part in clinical trials are also listed in PDQ. For more information, call the Cancer Information Service 1-800-4-CANCER (1-800-422-6237).

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The best way to cite this PDQ summary is:

National Cancer Institute: PDQ Wilms Tumor and Other Childhood Kidney Tumors Treatment. Bethesda, MD: National Cancer Institute. Date last modified <MM/DD/YYYY>. Available at: http://cancer.gov/cancertopics/pdq/treatment/wilms/Patient. Accessed <MM/DD/YYYY>.

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Physicians version: CDR0000062789
Date first published: 2006-05-05 Date last modified: 2014-05-22

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