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Wilms Tumor and Other Childhood Kidney Tumors

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

Wilms tumor

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

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 cancer that occurs mostly in infants and young children. It 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.
  • 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.
  • 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 mesoblastic nephroma may appear on an ultrasound exam before birth or may occur within the first 3 months after the child is born. 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 developing 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. Parents who think their child may be at risk should discuss this with the child's doctor.

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:

  • WAGR (Wilms tumor, aniridia, abnormal genitourinary system, and mental retardation) syndrome
  • Beckwith-Wiedemann syndrome
  • Hemihypertrophy
  • Denys-Drash syndrome
  • Cryptorchidism
  • Hypospadias

Children with these genetic syndromes and birth defects should be screened for Wilms tumor every three months until age 8. An ultrasound test may be used for screening.

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

These and other symptoms may be caused by kidney tumors. Other conditions may cause the same symptoms. A doctor should be consulted if any of the following problems occur in the child:

  • 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.

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.

Stages of Wilms Tumor and Other Childhood Kidney Tumors

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

The process used to find out if cancer has spread outside of the kidney to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment.

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

CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the chest or 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 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.

There are three ways that cancer spreads in the body.

The three ways that cancer spreads in the body are:

  • Through tissue. Cancer invades the surrounding normal tissue.
  • Through the lymph system. Cancer invades the lymph system and travels through the lymph vessels to other places in the body.
  • Through the blood. Cancer invades the veins and capillaries and travels through the blood to other places in the body.

When cancer cells break away from the primary (original) tumor and travel through the lymph or blood to other places in the body, another (secondary) tumor may form. This process is called metastasis. The secondary (metastatic) tumor is the same type of cancer as the primary tumor. For example, if breast cancer spreads to the bones, the cancer cells in the bones are actually breast cancer cells. The disease is metastatic breast cancer, not bone 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:

  • Cancer had spread out of the kidney to nearby soft tissue.
  • Cancer had spread to blood vessels of the kidney.
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

In stage V, cancer cells are found in both kidneys when the disease is first diagnosed. Each kidney will be staged separately as I, II, III, or IV.

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:

  • Pediatric surgeon or urologist
  • Radiation oncologist
  • Rehabilitation specialist
  • Pediatric nurse specialist
  • Social worker

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.

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.

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.

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 spinal column, 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.

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 section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied.

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.

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.

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 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

For some types or stages of cancer, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you.

Stage I Wilms Tumor

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

  • Nephrectomy with lymph node removal followed by combination chemotherapy.
  • A clinical trial of nephrectomy with lymph node removal, followed by watchful waiting or new combinations of chemotherapy.

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

  • Nephrectomy with lymph node removal followed by combination chemotherapy and radiation therapy to the flank (either side of the body between the ribs and hipbone) of the body.
  • A clinical trial of nephrectomy with lymph node removal, followed by new combinations of chemotherapy with radiation therapy.
Stage II Wilms Tumor

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

  • Nephrectomy with removal of lymph nodes, followed by combination chemotherapy.
  • A clinical trial of nephrectomy with removal of lymph nodes, followed by new combinations of chemotherapy.

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

  • Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy.
  • A clinical trial of nephrectomy with removal of lymph nodes, followed by new combinations of chemotherapy with radiation therapy.
Stage III Wilms Tumor

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

  • Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy.
  • A clinical trial of nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and new combinations of chemotherapy.

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

  • Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy.
  • Combination chemotherapy given before or after nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen.
  • A clinical trial of nephrectomy with removal of lymph nodes, followed by new combinations of chemotherapy with radiation therapy.
Stage IV Wilms Tumor

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

  • Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy. If cancer has spread to the lungs, patients will also receive radiation therapy to the lungs.
  • A clinical trial of nephrectomy with removal of lymph nodes, followed by chemotherapy for cancer that has spread only to the lungs, and by radiation therapy to the abdomen. If cancer in the lungs remains after chemotherapy, radiation therapy may be given.
  • A clinical trial of nephrectomy with removal of lymph nodes, followed by chemotherapy, and radiation therapy to all places where cancer has spread.

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

  • Combination chemotherapy given before or after nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen. If cancer has spread to the lungs, patients will also receive radiation therapy to the lungs.
  • A clinical trial of nephrectomy with removal of lymph nodes, followed by new combinations of chemotherapy with radiation therapy.
Stage V Wilms Tumor

Treatment of stage V Wilms tumor may be different for each patient. A biopsy of the kidneys is followed by chemotherapy to shrink the tumor. Second-look surgery is done to remove as much of the cancer as possible. This may be followed by more chemotherapy and/or radiation therapy if cancer remains after surgery.

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 of cancer.

Inoperable Tumors

Sometimes the tumor is inoperable (cannot be removed by surgery) because it is too close to important organs or blood vessels or because it is too large to remove. In this case, chemotherapy may be given to reduce the size of the tumor so it may be removed in surgery. If the tumor does not shrink enough after chemotherapy, radiation therapy may be given to shrink it further so that surgery may be done. This may be followed by more chemotherapy and/or more radiation therapy.

Clear Cell Sarcoma of the Kidney

There is no standard treatment for clear cell sarcoma of the kidney. Treatment is usually within a clinical trial and may include nephrectomy with lymph node removal, with or without radiation therapy to the abdomen, and new combinations of chemotherapy.

Rhabdoid Tumor of the Kidney

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

Neuroepithelial Tumor of the Kidney

There is no standard treatment for neuroepithelial tumor of the kidney. Treatment is usually within a clinical trial. It may be treated in the same way that Ewing family of tumors or primitive neuroectodermal tumors are treated.

Mesoblastic Nephroma

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

Renal Cell Cancer

Treatment of renal cell cancer is usually nephrectomy with removal of lymph nodes. If cancer has spread, treatment may include biologic therapy or surgery to remove the primary tumor.

Nephroblastomatosis

Treatment of nephroblastomatosis may include nephrectomy with lymph node removal followed by combination chemotherapy. A partial nephrectomy may be done to keep as much kidney function as possible.

Recurrent Wilms Tumor and Other Childhood Kidney Tumors

Treatment of recurrent Wilms tumor may be within a clinical trial of combination chemotherapy, surgery, and radiation therapy, with or without stem cell transplant, using the child's own blood stem cells.

Treatment of recurrent clear cell sarcoma of the kidney, 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 PDQ Cancer Clinical Trials Registry that are now accepting patients with 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.

To learn more

U.S. residents may call the National Cancer Institute's (NCI's) Cancer Information Service toll-free at 1-800.4.CANCER (1.800.422.6237) Monday through Friday from 9:00 a.m. to 4:30 p.m. Deaf and hard-of-hearing callers with TTY equipment may call 1.800.332.8615. Information about ongoing clinical trials is available from the NCI Web site

For more information from the NCI, please write to this address:

NCI Public Inquiries Office
Suite 3036A
6116 Executive Boulevard, MSC8322
Bethesda, MD 20892-8322

Source: National Institutes of Health, National Cancer Institute

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This information is provided by the Cleveland Clinic and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition.  This document was last reviewed on: 9/11/2009...#6219