Last Modified : 2006-08-03
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.
Other kidney tumors
Clear cell sarcoma of the kidney, rhabdoid tumor of the kidney, neuroepithelial tumor of the kidney, and renal cell cancer are also childhood kidney tumors, but they are not related to Wilms' tumor.
Anything that increases your risk of getting a disease is called a risk factor. 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 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:
The following tests and procedures may be used:
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:
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' tumors, 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:
The histology (how the cells look under a microscope) of the tumor affects the prognosis and may be favorable or unfavorable. Tumors with a favorable histology respond better to treatment than those with unfavorable histology.
In stage I, the tumor was completely removed by surgery and all of the following are true:
In stage II, the tumor was completely removed by surgery and no cancer cells are found at the edges of the area where the cancer was removed. Also, one of the following is true:
In stage III, cancer remains in the abdomen after surgery and at least one of the following is true:
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 VIn 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 cancer is cancer that has recurred (come back) after it has been treated.
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. Clinical trials are taking place in many parts of the country. Information about ongoing clinical trials is available from the NCI Web site. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team.
Children with Wilms' tumor or other childhood kidney tumors should have their treatment planned by a team of doctors with expertise 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 may refer you to other doctors who have experience and expertise 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:
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 increase the chances of a cure, is called adjuvant therapy. Sometimes, a second-look surgery is done to see if cancer remains after chemotherapy or radiation therapy.
Radiation therapyRadiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells. 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.
ChemotherapyChemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells 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 therapyBiologic 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. These include the following: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.
This summary section refers to specific treatments under study in clinical trials, but it may not mention every new treatment being studied. Information about ongoing clinical trials is available from the NCI Web site.
Late effects may be caused by cancer treatment.Some cancer treatments cause side effects that continue or appear months or years after cancer treatment has ended. These are called late effects. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information.) It is important that parents of children who are treated for cancer know about the possible late effects caused by certain treatments. After several years, some patients develop another form of cancer as a result of their treatment with chemotherapy and radiation. Clinical trials are ongoing to find out if lower doses of chemotherapy and radiation can be used.
Treatment of stage I Wilms' tumor with either favorable or unfavorable histology may include the following:
This summary section refers to specific treatments under study in clinical trials, but it may not mention every new treatment being studied. Information about ongoing clinical trials is available from the NCI Web site.
Treatment of stage II Wilms' tumor with favorable histology is usually nephrectomy with removal of lymph nodes, followed by combination chemotherapy.
Treatment of stage II Wilms' tumor with unfavorable histology is usually nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy.
Treatment of stage III Wilms' tumor with either favorable or unfavorable histology is usually nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy.
Treatment of stage IV Wilms' tumor with either favorable or unfavorable histology is usually nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy. Some patients may also receive radiation therapy to the lungs.
Treatment of stage V Wilms' tumor may be different for each patient. Treatment is usually chemotherapy to shrink the tumor, followed by surgery 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.
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.
There is no standard treatment for clear cell sarcoma of the kidney. Treatment is usually within a clinical trial and may include nephrectomy, followed by radiation therapy to the abdomen and combination chemotherapy. Some patients may also receive radiation therapy to the lungs.
This summary section refers to specific treatments under study in clinical trials, but it may not mention every new treatment being studied. Information about ongoing clinical trials is available from the NCI Web site.
There is no standard treatment for rhabdoid tumor of the kidney. Treatment may be within a clinical trial and may include combination chemotherapy.
This summary section refers to specific treatments under study in clinical trials, but it may not mention every new treatment being studied. Information about ongoing clinical trials is available from the NCI Web site.
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's family of tumors or primitive neuroectodermal tumors are treated. See the PDQ summary on Ewing's Family of Tumors Treatment for more information.
Information about ongoing clinical trials is available from the NCI Web site.
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. See the PDQ summary on Renal Cell Cancer Treatment for more information.
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, rhabdoid tumor, and neuroepithelial tumor of the kidney is usually within a clinical trial.
This summary section refers to specific treatments under study in clinical trials, but it may not mention every new treatment being studied. Information about ongoing clinical trials is available from the NCI Web site.