National Cancer Institute


Posted Date: Apr 27, 2014

Expert-reviewed information summary about the treatment of Wilms tumor.

Wilms Tumor and Other Childhood Kidney Tumors Treatment

General Information About Wilms Tumor and Other Childhood Kidney Tumors

Key Points for this Section

  • Wilms tumor and other childhood kidney tumors are diseases in which malignant (cancer) cells form in the tissues of the kidney.
  • Having certain genetic syndromes or birth defects can increase the risk of Wilms tumor.
  • Having certain conditions may be associated with renal cell carcinoma.
  • Signs of Wilms tumor and other childhood kidney tumors include a lump in the abdomen and blood in the urine.
  • Tests that examine the kidney and the blood are used to detect (find) Wilms tumor and other childhood kidney tumors.
  • Wilms tumor and other childhood kidney tumors are usually diagnosed and removed in surgery.
  • Certain factors affect prognosis (chance of recovery) and treatment options.
  • Wilms tumor is rare in adults.

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 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:

  • Clear cell sarcoma of the kidney is a type of kidney tumor that may spread to the lung, bone, brain, and soft tissue.
  • 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 and brain.
  • Neuroepithelial tumors of the kidney are rare and usually occur in young adults. They grow and spread quickly.
  • 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 is a very rare type of Wilms tumor made up of cysts.
  • Renal cell carcinoma is rare in children or in adolescents younger than 15 years of age. However, it is much more common in adolescents between 15 and 19 years of age. Renal cell carcinomas can spread to the lungs, bones, liver, and lymph nodes.
  • Congenital mesoblastic nephroma is a tumor of the kidney that is usually diagnosed within the first year of life and can usually be cured. One type of congenital mesoblastic nephroma may appear on an ultrasound exam before birth or may occur within the first 3 months after the child is born. Congenital mesoblastic nephroma occurs more often in males than females.
  • Primary renal synovial sarcoma is a rare tumor of the kidney and is most common in young adults.
  • Anaplastic sarcoma of the kidney is a rare tumor that is most commonly found in children or adolescents younger than 15 years of age. Anaplastic sarcoma of the kidney often spreads to the lungs, liver, or bones. There is no standard treatment for anaplastic sarcoma.

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:

  • 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 carcinoma with an ultrasound of the abdomen or 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 carcinoma (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 carcinoma (a rare kidney cancer that grows and spreads quickly).
  • Hereditary leiomyomatosis (a disorder that increases the risk of having cancer of the kidney, skin, and uterus).
  • Second cancers (renal cell carcinoma may be found in patients several years after treatment for neuroblastoma).

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:

  • A lump, swelling, or pain in the abdomen.
  • Blood in the urine.
  • Fever for no known reason.

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:

  • 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:The number of red blood cells, white blood cells, and platelets. The amount of hemoglobin (the protein that carries oxygen) in the red blood cells. The portion of the blood sample made up of red blood cells.
  • 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.
  • 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.
  • 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, 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.
  • Abdominal x-ray: An x-ray of the organs inside 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.
  • Biopsy: The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. Whether a biopsy is done depends on one or more of the following: Cancer is in one or both kidneys.Imaging tests clearly show the cancer.The patient is on a clinical trial.A biopsy may be done before treatment, after surgery to remove the tumor, or after chemotherapy to shrink the tumor.

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:

  • How different the tumor cells are from normal kidney cells.
  • The stage of the cancer.
  • The type and size of the tumor.
  • The age of the child.
  • Whether the tumor can be completely removed in surgery.
  • Whether the cancer has just been diagnosed or has recurred (come back).
  • Whether there are any abnormal chromosomes or genes.
  • Whether the patient is treated by pediatric experts with experience in treating patients with Wilms tumor.

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

Key Points for this Section

  • Wilms tumors and other childhood kidney tumors are staged during surgery and with imaging tests.
  • There are three ways that cancer spreads in the body.
  • Cancer may spread from where it began to other parts of the body.
  • In addition to the stages, Wilms tumors are described by their histology.
  • The following stages are used for both favorable histology and anaplastic Wilms tumors:
    • Stage I
    • Stage II
    • Stage III
    • Stage IV
    • Stage V and those at high risk of developing Wilms tumor

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:

  • 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.
  • 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.
  • 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 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.

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 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:

  • Cancer was found only in the kidney and did not spread to blood vessels of the kidney.
  • The outer layer of the kidney did not break open.
  • The tumor did not break open.
  • A biopsy of the tumor was not done.
  • No cancer cells 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. 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:

  • Cancer spread to lymph nodes in the abdomen or pelvis (the part of the body between the hips).
  • Cancer 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).
  • Chemotherapy was given before surgery and a biopsy of the tumor was done during surgery to remove it.
  • The tumor broke open before or during surgery to remove it.
  • The tumor was removed in more than one piece.

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

Key Points for this Section

  • There are different types of treatment for patients with Wilms tumor and other childhood kidney tumors.
  • 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.
  • Some cancer treatments cause side effects months or years after treatment has ended.
  • Four types of standard treatment are used:
    • Surgery
    • Radiation therapy
    • Chemotherapy
    • Biologic therapy
  • New types of treatment are being tested in clinical trials.
    • Watchful waiting
    • High-dose chemotherapy with stem cell transplant
    • Targeted therapy
  • Patients may want to think about taking part in a clinical trial.
  • Patients can enter clinical trials before, during, or after starting their cancer treatment.
  • Follow-up tests may be needed.

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.
  • 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 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:

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