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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.
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.
CONTENTS
- General Information About Wilms Tumor and Other Childhood Kidney Tumors
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Childhood kidney tumors are diseases in which malignant (cancer) cells form in the tissues of the kidney.
There are two kidneys, one on each side of the spine, above the waist. Tiny tubules in the kidneys filter and clean the blood. They take out waste products and make urine. The urine passes from each kidney through a long tube called a ureter into the bladder. The bladder holds the urine until it passes through the urethra and leaves the body.
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. There are many types of childhood kidney tumors.
Wilms Tumor
In Wilms tumor, one or more tumors may be found in one or both kidneys. Wilms tumor may spread to the lungs, liver, bone, brain, or nearby lymph nodes. In children and adolescents younger than 15 years old, most kidney cancers are Wilms tumors.
Renal Cell Cancer (RCC)
Renal cell cancer is rare in children and adolescents younger than 15 years old. It is much more common in adolescents between 15 and 19 years old. Children and adolescents are more likely to be diagnosed with a large renal cell tumor or cancer that has spread. Renal cell cancers may spread to the lungs, liver, bone, or lymph nodes. Renal cell cancer may also be called renal cell carcinoma.
Rhabdoid Tumor of the Kidney
Rhabdoid tumor of the kidney is a type of kidney cancer that occurs mostly in infants and young children. It is often advanced at the time of diagnosis. Rhabdoid tumor of the kidney grows and spreads quickly, often to the lungs or brain.
Children with a certain change in the SMARCB1 gene can also have tumors grow in the kidney, brain, or soft tissues. These children are checked regularly to see if a rhabdoid tumor has formed in the kidney or the brain:
Clear Cell Sarcoma of the Kidney
Clear cell sarcoma of the kidney is an uncommon kidney cancer that may spread to the bone, lungs, brain, liver, or soft tissue. It occurs most often before age 3 years. It may recur (come back) up to 14 years after treatment, often in the brain or lung.
Congenital Mesoblastic Nephroma
Congenital mesoblastic nephroma is a tumor of the kidney that is often diagnosed during the first year of life or before birth. It is the most common kidney tumor found in infants younger than 6 months old and is found more often in males than in females. It can usually be cured.
Ewing Sarcoma of the Kidney
Ewing sarcoma (previously called neuroepithelial tumor) of the kidney is rare and usually occurs in young adults. This cancer grows and spreads to other parts of the body quickly.
Primary Renal Myoepithelial Carcinoma
Primary renal myoepithelial carcinoma is a rare type of cancer that usually affects soft tissues, but sometimes forms in the internal organs (such as the kidney). This type of cancer grows and spreads quickly.
Cystic Partially Differentiated Nephroblastoma
Cystic partially differentiated nephroblastoma is a very rare type of Wilms tumor made up of cysts.
Multilocular Cystic Nephroma
Multilocular cystic nephromas are benign tumors made up of cysts and are most common in infants, young children, and adult women. These tumors can occur in one or both kidneys.
Children with this type of tumor also may have pleuropulmonary blastoma, so imaging tests that check the lungs for cysts or solid tumors are done. Since multilocular cystic nephroma may be an inherited condition, genetic counseling and genetic testing may be considered. See the PDQ summary about Childhood Pleuropulmonary Blastoma Treatment for more information.
Primary Renal Synovial Sarcoma
Primary renal synovial sarcoma is a cyst-like tumor of the kidney and is most common in young adults. These tumors grow and spread quickly.
Anaplastic Sarcoma of the Kidney
Anaplastic sarcoma of the kidney is a rare tumor that is most common in children or adolescents younger than 15 years of age. Anaplastic sarcoma of the kidney often spreads to the lungs, liver, or bones. Imaging tests that check the lungs for cysts or solid tumors may be done. Since anaplastic sarcoma may be an inherited condition, genetic counseling and genetic testing may be considered.
Nephroblastomatosis is not cancer but may become Wilms tumor.
Sometimes, after the kidneys form in the fetus, abnormal groups of kidney cells remain in one or both kidneys. In nephroblastomatosis (diffuse hyperplastic perilobar nephroblastomatosis), these abnormal groups of cells may grow in many places inside the kidney or make a thick layer around the kidney. When these groups of abnormal cells are found in a kidney after it was removed for Wilms tumor, the child has an increased risk of Wilms tumor in the other kidney. Frequent follow-up testing is important at least every 3 months, for at least 7 years after the child is diagnosed or treated.
Having certain genetic syndromes, other conditions, or environmental exposures 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 signs and symptoms or conditions that occur together and is caused by certain changes in the genes. Certain conditions or environmental exposures can also increase a child's risk of developing Wilms tumor. The following have been linked to Wilms tumor:
Tests are used to screen for Wilms tumor.
Screening tests are done in children with an increased risk of Wilms tumor. These tests may help find cancer early and decrease the chance of dying from cancer.
In general, children with an increased risk of Wilms tumor should be screened for Wilms tumor every 3 months until they are at least 8 years old. An ultrasound test of the abdomen is usually used for screening. Small Wilms tumors may be found and removed before symptoms occur.
Children with Beckwith-Wiedemann syndrome or hemihyperplasia are also screened for liver and adrenal tumors that are linked to these genetic syndromes. A test to check the alpha-fetoprotein (AFP) level in the blood and an ultrasound of the abdomen are done until the child is 4 years old. An ultrasound of the kidneys is done between the ages of 4 and 7 years old. A physical exam by a specialist (geneticist or pediatric oncologist) is done two times each year. In children with certain gene changes, a different schedule for ultrasound of the abdomen may be used.
Children with aniridia and a certain gene change are screened for Wilms tumor every 3 months until they are 8 years old. An ultrasound test of the abdomen is used for screening.
Some children develop Wilms tumor in both kidneys. These often appear when Wilms tumor is first diagnosed, but Wilms tumor may also occur in the second kidney after the child is successfully treated for Wilms tumor in one kidney. Children with an increased risk of a second Wilms tumor in the other kidney should be screened for Wilms tumor every 3 months for up to 8 years. An ultrasound test of the abdomen may be used for screening.
Having certain conditions may increase the risk of renal cell cancer.
Renal cell cancer may be related to the following conditions:
Prior chemotherapy or radiation therapy for a childhood cancer, such as neuroblastoma, soft tissue sarcoma, leukemia, or Wilms tumor may also increase the risk of renal cell cancer. See the Second Cancers section in the PDQ summary about Late Effects of Treatment for Childhood Cancer for more information.
Treatment for Wilms tumor and other childhood kidney tumors may include genetic counseling.
Genetic counseling (a discussion with a trained professional about genetic diseases and whether genetic testing is needed) may be done if the child has one of the following syndromes or conditions:
Signs of Wilms tumor and other childhood kidney tumors include a lump in the abdomen and blood in the urine.
Sometimes childhood kidney tumors do not cause signs and symptoms and the parent finds a mass in the abdomen by chance or the mass is found during a well-child health check-up. These and other signs and symptoms may be caused by kidney tumors or by other conditions. Check with your child's doctor if your child has any of the following:
Wilms tumor that has spread to the lungs or liver may cause the following signs and symptoms:
Tests that examine the kidney and the blood are used to diagnose Wilms tumor and other childhood kidney tumors.
The following tests and procedures may be used:
Certain factors affect prognosis (chance of recovery) and treatment options.
The prognosis and treatment options for Wilms tumor depend on the following:
The prognosis for renal cell cancer depends on the following:
The prognosis for rhabdoid tumor of the kidney depends on the following:
The prognosis for clear cell sarcoma of the kidney depends on the following:
- Stages of Wilms Tumor
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Wilms 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. The results of the tests and procedures done to diagnose and stage Wilms tumor are used to help make decisions about treatment.
There is no staging for the other types of childhood kidney tumors. The treatment of these tumors depends on the tumor type.
The following 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 anaplastic tumors. Tumor cells that are anaplastic divide quickly and under a microscope 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 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. Cancer has not spread to lymph nodes. 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:
Sometimes Wilms tumor and other childhood kidney tumors come back after treatment.
Childhood Wilms tumor may recur (come back) in the lungs, abdomen, liver, or other places in the body.
Childhood clear cell sarcoma of the kidney may recur in the brain, lungs, or other places in the body.
Childhood congenital mesoblastic nephroma may recur in the kidneys or in other places in the body.
- Treatment Option Overview
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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:
Six types of treatment are used:
Surgery
Two types of surgery are used to treat kidney tumors:
After 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.
Sometimes the tumor cannot be removed by surgery for one of the following reasons:
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.
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. External radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer.
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). Combination chemotherapy is treatment using two or more anticancer drugs.
Systemic chemotherapy is used to treat Wilms tumor and other childhood kidney tumors.
Sometimes 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.
See Drugs Approved for Wilms Tumor and Other Childhood Kidney Cancers for more information.
Immunotherapy
Immunotherapy 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 cancer treatment is a type of biologic therapy.
Interferon and interleukin-2 (IL-2) are types of immunotherapy used to treat childhood renal cell cancer. Interferon may slow tumor growth and may help kill the cancer cells. 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 doses of chemotherapy are given to kill cancer cells. Healthy cells, including blood-forming cells, are also destroyed by the cancer treatment. Stem cell rescue is a treatment to replace the blood-forming cells. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient and are frozen and stored. After the patient completes chemotherapy, the stored stem cells are thawed and given back to the patient through an infusion. These stem cells grow into (and restore) the body's blood cells.
High-dose chemotherapy with stem cell rescue may be used to treat rhabdoid tumor of the kidney or recurrent Wilms tumor.
Targeted therapy
Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. Targeted therapy used to treat childhood kidney tumors may include the following:
Other targeted therapies are being studied for the treatment of childhood kidney tumors that have recurred (come back).
New types of treatment are being tested in clinical trials.
Information about clinical trials is available from the NCI website.
Treatment for Wilms tumor and other childhood kidney tumors may cause side effects.
For information about side effects that begin during treatment for cancer, see our Side Effects page.
Side effects from cancer treatment that begin 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 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.
Monitoring for late effects involving the kidneys in patients with Wilms tumor and related conditions includes the following:
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. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.
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
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For information about the treatments listed below, see the Treatment Option Overview section.
Stage I Wilms Tumor
Treatment of stage I Wilms tumor with favorable histology may include:
Treatment of stage I anaplastic Wilms tumor may include:
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
Stage II Wilms Tumor
Treatment of stage II Wilms tumor with favorable histology may include:
Treatment of stage II anaplastic Wilms tumor may include:
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
Stage III Wilms Tumor
Treatment of stage III Wilms tumor with favorable histology may include:
Treatment of stage III anaplastic Wilms tumor may include:
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
Stage IV Wilms Tumor
Treatment of stage IV Wilms tumor with favorable histology may include:
Treatment of stage IV anaplastic Wilms tumor may include:
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
Stage V Wilms Tumor and patients at high risk of developing bilateral Wilms tumor
Treatment of stage V Wilms tumor (bilateral) may be different for each patient and may include:
If a kidney transplant is needed because of kidney problems, it is usually delayed until 1 to 2 years after treatment is completed and there are no signs of cancer.
(See the Treatment of Recurrent Childhood Kidney Tumors section of this summary for information about recurrent disease.)
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
- Treatment Options for Other Childhood Kidney Tumors
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For information about the treatments listed below, see the Treatment Option Overview section.
Renal Cell Cancer (RCC)
Treatment of renal cell cancer may include:
See the PDQ summary about Renal Cell Cancer Treatment for more information.
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
Rhabdoid Tumor of the Kidney
There is no standard treatment for rhabdoid tumor of the kidney. Treatment may include:
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
Clear Cell Sarcoma of the Kidney
Treatment of clear cell sarcoma of the kidney may include:
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
Congenital Mesoblastic Nephroma
Treatment of stages I, II, and certain patients with stage III congenital mesoblastic nephroma may include:
Treatment for certain patients with stage III congenital mesoblastic nephroma may include:
A clinical trial of targeted therapy with a tyrosine kinase inhibitor (larotrectinib) for cancer that has a certain gene change and has not been treated.
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
Ewing Sarcoma of the Kidney
There is no standard treatment for Ewing sarcoma of the kidney. Treatment may include:
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.
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
Primary Renal Myoepithelial Carcinoma
There is no standard treatment for primary renal myoepithelial carcinoma. Treatment may include:
Cystic Partially Differentiated Nephroblastoma
Treatment of cystic partially differentiated nephroblastoma may include:
Multilocular Cystic Nephroma
Treatment of multilocular cystic nephroma usually includes:
Primary Renal Synovial Sarcoma
Treatment of primary renal synovial sarcoma usually includes:
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:
Treatment of nephroblastomatosis may include:
- Treatment of Recurrent Childhood Kidney Tumors
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For information about the treatments listed below, see the Treatment Option Overview section.
Treatment of recurrent Wilms tumor may include:
Treatment of recurrent rhabdoid tumor of the kidney may include:
Treatment of recurrent clear cell sarcoma of the kidney may include:
Treatment of recurrent congenital mesoblastic nephroma may include:
Treatment of other recurrent childhood kidney tumors is usually within a clinical trial.
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
- To Learn More About Wilms Tumor and Other Childhood Kidney Tumors
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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:
- About This PDQ Summary
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About PDQ
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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 ("Updated") 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 can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
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The best way to cite this PDQ summary is:
PDQ® Pediatric Treatment Editorial Board. PDQ Wilms Tumor and Other Childhood Kidney Tumors Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/kidney/patient/wilms-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389390]
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