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Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®)

Patient Version
Last Modified: 10/24/2014

General Information About Wilms Tumor and Other Childhood Kidney Tumors



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 backbone, 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.

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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.


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. Most childhood kidney cancers are Wilms tumors, but in children 15 to 19 years old, renal cell cancer is more common.

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. Renal cell cancers may spread to the lungs, liver, 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 gene change are checked regularly to see if a rhabdoid tumor has formed in the kidney or has spread to the brain:

  • Children younger than one year old have an ultrasound of the abdomen every two to three months and an ultrasound of the brain every month.
  • Children one to four years old have an ultrasound of the abdomen and an MRI of the brain and spine every three months.

Clear Cell Sarcoma of the Kidney

Clear cell sarcoma of the kidney is a type of kidney tumor that may spread to the lung, bone, brain, or soft tissue. When it recurs (comes back) after treatment, it often recurs in the brain.

Congenital Mesoblastic Nephroma

Congenital mesoblastic nephroma is a tumor of the kidney that is often diagnosed during the first year of life. It can usually be cured.

Ewing Sarcoma (Neuroepithelial Tumor) of the Kidney

Ewing sarcoma (neuroepithelial tumor) of the kidney is rare and usually occurs in young adults. These tumors grow and spread to other parts of the body quickly.

Desmoplastic Small Round Cell Tumor of the Kidney

Desmoplastic small round cell tumor of the kidney is a rare soft tissue sarcoma. See the PDQ summary about Childhood Soft Tissue Sarcoma Treatment for more information.

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. These tumors can occur in one or both kidneys. Children with this type of tumor also may have pleuropulmonary blastomas, so imaging tests of the chest are done. See the PDQ summary about Unusual Cancers of Childhood Treatment for more information.

Primary Renal Synovial Sarcoma

Primary renal synovial sarcoma is a rare 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.

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, 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 that was removed for Wilms tumor, the child has an increased risk of Wilms tumor in the other kidney. Frequent follow-up testing is important for at least 7 years after the child is treated.

Having certain genetic syndromes or other conditions 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 can also increase a child's risk of developing Wilms tumor. These and other genetic syndromes and conditions 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 three 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 early and removed.

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 (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 after the child is 4 years old.

Children with aniridia and a certain gene change are screened for Wilms tumor every three 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. A second tumor is much more likely to develop in the other kidney when a child's first Wilms tumor is diagnosed before they are one year old or when embryonic cells remain in the kidney. Children with an increased risk of 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 increase the risk of renal cell cancer.

Renal cell cancer may be related to the following conditions:

  • Von Hippel-Lindau disease (an inherited condition that causes abnormal growth of blood vessels). Children with Von Hippel-Lindau disease should be checked yearly for renal cell cancer with an ultrasound of the abdomen or an MRI (magnetic resonance imaging) beginning at age 8 to 11 years.
  • Tuberous sclerosis (an inherited disease marked by noncancerous fatty cysts in the kidney).
  • Familial renal cell cancer (an inherited condition that occurs when certain changes in the genes that cause kidney cancer are passed down from the parent to the child).
  • Renal medullary cancer (a rare kidney cancer that grows and spreads quickly).
  • Hereditary leiomyomatosis (an inherited disorder that increases the risk of having cancer of the kidney, skin, and uterus).
  • Second cancers (renal cell cancer may occur several years after treatment for neuroblastoma).

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 needed 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.

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:

  • A lump, swelling, or pain in the abdomen.
  • Blood in the urine.
  • Fever for no known reason.
  • High blood pressure (headache, feeling very tired, chest pain, or trouble seeing or breathing).
  • Hypercalcemia (loss of appetite, nausea and vomiting, weakness, or feeling very tired).

Wilms tumor that has spread to the lungs or liver may cause the following signs and symptoms:

  • Cough.
  • Blood in the sputum.
  • Trouble breathing.
  • Pain in the abdomen.

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

The following tests and procedures may be used:

  • Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.

  • Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following:

  • Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. This test is done to check how well the liver and kidneys are working.

  • Renal function test : A procedure in which blood or urine samples are checked to measure the amounts of certain substances released into the blood or urine by the kidneys. A higher or lower than normal amount of a substance can be a sign that the kidneys are not working as they should.

  • Urinalysis : A test to check the color of urine and its contents, such as sugar, protein, blood, and bacteria.

  • Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. An ultrasound of the abdomen is done to diagnose a kidney tumor.

  • CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the chest, abdomen, and pelvis, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.

  • MRI (magnetic resonance imaging) with gadolinium: A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body, such as the abdomen. A substance called gadolinium is injected into a vein. The gadolinium collects around the cancer cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI).

  • X-ray of the abdomen: An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.

  • PET-CT scan : A procedure that combines the pictures from a positron emission tomography (PET) scan and a computed tomography (CT) scan. The PET and CT scans are done at the same time on the same machine. The pictures from both scans are combined to make a more detailed picture than either test would make by itself. A PET scan is a procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do.

  • Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. The decision to do a biopsy is based on one or more of the following:
    • The size of the tumor.
    • The stage of the cancer.
    • Whether cancer is in one or both kidneys.
    • Whether imaging tests clearly show the cancer.
    • Whether the tumor can be removed by surgery.
    • Whether the patient is in a clinical trial.
    A biopsy may be done before any treatment is given, after chemotherapy to shrink the tumor, or after surgery to remove the tumor.

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

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

  • How different the tumor cells are from normal kidney cells when looked at under a microscope.
  • The stage of the cancer.
  • The type and size of the tumor.
  • The age of the child.
  • Whether the tumor can be completely removed by surgery.
  • Whether there are certain changes in chromosomes or genes.
  • Whether the cancer has just been diagnosed or has recurred (come back).

The prognosis for renal cell cancer depends on the following:

  • The stage of the cancer.

The prognosis for rhabdoid tumor of the kidney depends on the following:

  • The age of the child when cancer is diagnosed.
  • The stage of the cancer.
  • Whether the cancer has spread to the brain.

The prognosis for clear cell sarcoma of the kidney depends on the following:

  • The age of the child when cancer is diagnosed.
  • The stage of the cancer.

Wilms tumor is rare in adults.

Wilms tumor in adults is harder to cure than in children.

Stages of Wilms Tumor



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. It is important to know the stage in order to plan treatment. The doctor will use results of the diagnostic and staging tests to help find out the stage of the disease.

The following tests may be done to see if cancer has spread to other places in the body:

  • Lymph node biopsy : A surgical procedure in which lymph nodes in the abdomen are removed and a sample of tissue is checked under a microscope for signs of cancer. This procedure is also called lymphadenectomy or lymph node dissection.

  • Liver function test : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by the liver. A higher than normal amount of a substance can be a sign that the liver is not working as it should.

  • X-ray of the chest and bones: An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body, such as the chest.

  • CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the abdomen, pelvis, chest, and brain, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.

  • PET-CT scan : A procedure that combines the pictures from a positron emission tomography (PET) scan and a computed tomography (CT) scan. The PET and CT scans are done at the same time on the same machine. The pictures from both scans are combined to make a more detailed picture than either test would make by itself. A PET scan is a procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do.

  • MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body, such as the abdomen, pelvis, and brain. This procedure is also called nuclear magnetic resonance imaging (NMRI).

  • Bone scan : A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones with cancer and is detected by a scanner.

  • Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. An ultrasound of the major heart vessels is done to stage Wilms tumor.

  • Cystoscopy : A procedure to look inside the bladder and urethra to check for abnormal areas. A cystoscope is inserted through the urethra into the bladder. A cystoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer.

  • Ureteroscopy : A procedure to look inside the kidney and ureter to check for abnormal areas, using a ureteroscope. A ureteroscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer. The ureteroscope is passed through the urethra into the bladder, ureter, and renal pelvis (part of the kidney that collects, holds, and drains urine).

There are three ways that cancer spreads in the body.

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

  • Tissue. The cancer spreads from where it began by growing into nearby areas.
  • Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body.
  • Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.

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.

  • Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body.
  • Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body.

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 I

In 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 in the renal sinus (the part of the kidney where it joins the ureter) or to the lymph nodes.
  • The outer layer of the kidney did not break open.
  • The tumor did not break open.
  • A biopsy was not done before the tumor was removed.
  • No cancer cells were found at the edges of the area where the tumor was removed.

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:

  • Cancer had spread to the renal sinus (the part of the kidney where it joins the ureter).
  • Cancer had spread to blood vessels outside the area of the kidney where urine is made, such as the renal sinus.

Stage III

In stage III, cancer remains in the abdomen after surgery and one 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 to or through the surface of the peritoneum (the layer of tissue that lines the abdominal cavity and covers most organs in the abdomen).
  • A biopsy of the tumor was done before it was removed.
  • The tumor broke open before or during surgery to remove it.
  • The tumor was removed in more than one piece.
  • Cancer cells are found at the edges of the area where the tumor was removed.
  • The entire tumor could not be removed because important organs or tissues in the body would be damaged.

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

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

The treatment of other childhood kidney tumors depends on the tumor type.

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:

  • Physical problems, such as heart problems or problems during pregnancy.
  • Changes in mood, feelings, thinking, learning, or memory.
  • Second cancers (new types of cancer).

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.

Five 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. Sometimes a kidney transplant (surgery to remove the kidney and replace it with a kidney from a donor) is done when the cancer is in both kidneys and the kidneys are not working well.

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 as much of the kidney working as possible. A partial nephrectomy is also called renal-sparing surgery.

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 of the cancer coming 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.

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). 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). Combination chemotherapy is treatment using two or more anticancer drugs.

The way the chemotherapy is given depends on the type and stage of the cancer being treated. Systemic chemotherapy is used to treat Wilms tumor and other childhood kidney tumors.

Sometimes the tumor cannot be removed by surgery because it is too close to important organs or blood vessels, too large to remove, cancer is in both kidneys or the patient has trouble breathing because cancer has spread to the lungs. In this case, chemotherapy may be given to reduce the size of the tumor before surgery in order to save as much healthy tissue as possible. Radiation therapy is given after surgery.

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.

Interferon and interleukin-2 (IL-2) are types of biologic therapy used to treat childhood renal cell cancer. Interferon affects the division of cancer cells and can slow tumor growth. 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 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.

High-dose chemotherapy with stem cell transplant may be used to treat recurrent Wilms tumor.

New types of treatment are being tested in clinical trials.

Information about clinical trials is available from the NCI Web site.

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



Stage I Wilms Tumor

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

Treatment of stage I anaplastic Wilms tumor may include:

  • Nephrectomy with removal of lymph nodes followed by combination chemotherapy and radiation therapy to the flank (either side of the body between the ribs and hipbone) of the body.

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:

Treatment of stage II anaplastic Wilms tumor may include:

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:

Treatment of stage III anaplastic Wilms tumor may include:

  • Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy.
  • Combination chemotherapy given before nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen.
  • Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy.

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:

Treatment of stage IV anaplastic Wilms tumor may include:

  • Combination chemotherapy given before nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen. If cancer has spread to other parts of the body, patients will also receive radiation therapy to those areas.
  • Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy. If cancer has spread to other parts of the body, patients will also receive radiation therapy to those areas.

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 patients at high risk of developing bilateral 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.

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.

Treatment Options for Other Childhood Kidney Tumors



Renal Cell Cancer (RCC)

Treatment of renal cell cancer usually includes:

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 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.

Rhabdoid Tumor of the Kidney

There is no standard treatment for rhabdoid tumor of the kidney. Treatment 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 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.

Clear Cell Sarcoma of the Kidney

Treatment of clear cell sarcoma of the kidney may include:

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.

Congenital Mesoblastic Nephroma

Treatment for congenital mesoblastic nephroma usually includes:

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.

Ewing Sarcoma (Neuroepithelial Tumor) of the Kidney

There is no standard treatment for Ewing sarcoma (neuroepithelial tumor) of the kidney. Treatment may include:

It may also be treated in the same way that Ewing sarcoma or primitive neuroectodermal tumors are treated. See the PDQ summary about Ewing Sarcoma Treatment for more information.

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with Ewing sarcoma/peripheral primitive neuroectodermal tumor (PNET). 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.

Desmoplastic Small Round Cell Tumor of the Kidney

There is no standard treatment for desmoplastic small round cell tumor of the kidney. Treatment may include:

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with childhood desmoplastic small round cell 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.

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

The treatment of nephroblastomatosis depends on the following:

  • Whether the child has abnormal groups of cells in one or both kidneys.
  • Whether the child has Wilms tumor in one kidney and groups of abnormal cells in the other kidney.

Treatment of nephroblastomatosis may include:

Treatment of Recurrent Childhood Kidney Tumors

Treatment of recurrent Wilms tumor may include:

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

  • Combination chemotherapy, surgery, and/or 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.

Changes to This Summary (10/24/2014)

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.

Changes were made to this summary to match those made to the health professional version.

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.

PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH.

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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