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Wilms’ Tumor and Other Childhood Kidney Tumors

Wilms’ tumor is the most common form of kidney cancer in children. This type of cancer derives from immature kidney cells. When a child is born, some kidney cells have not yet differentiated fully into the various types of cells that make up a kidney. It takes 3 or 4 years for all the kidney cells to mature. In rare cases, some of the immature cells start to grow out of control. The result is a mass of rapidly dividing, primitive, small cells, called Wilms’ tumor. Wilms’ can affect one kidney, called unilateral, or both kidneys, called bilateral.

Other Types of Kidney Cancers

Other types of kidney cancers seen in children include clear cell sarcoma of the kidney, rhabdoid tumor of the kidney, and neuroepithelial tumor of the kidney. These rare childhood kidney cancers must be diagnosed by a pathologist who has experience with these types of renal tumors so that the correct treatment plan can be determined.


Treatment for Wilms’ tumor depends on the stage of the tumor, the characteristics of the tumor cells, and whether or not the tumor is recurrent. The first step in treatment is almost always surgery to remove the kidney. Staging is done at the time of surgery and defines the extent of the cancer in the body. In Wilms’ tumor, stage 1 is indicated when the tumor is limited to the kidney and when the tumor can be completely removed, stages 2-4 are increasing involvement outside the kidney, and stage 5 is when both kidneys are involved. More information on the stages and characteristics of the tumor cells can be found on the NCI web site:

Surgery is usually followed by chemotherapy and sometimes radiation. For the least aggressive tumors, the chemotherapy is dactinomycin and vincristine. For more aggressive tumors, the chemotherapy can include doxorubicin, cyclophosphamide, and etoposide, as well as radiation.

Clinical trials for Wilms’ tumors can be found by searching the clinical trial database on the NCI web site. Follow these directions:

    • Start at the NCI clinical trials search page.
    • Choose type of cancer: “Wilms’ tumor”
    • You will be given more choices (e.g., type of cancer and type of trial). Choose the appropriate responses and click “search. “

Symptoms of Kidney Tumors

Symptoms can include:

    • a lump, swelling, and/or pain in the abdomen
    • abnormal urine color or blood in the urine
    • constipation
    • loss of appetite
    • fever of unknown origin
    • high blood pressure
    • night sweats
    • nausea, vomiting (infrequent symptoms)


    • Just under 500 new cases of Wilms’ are diagnosed each year in the United States.
    • More than 90% of the patients survive 4 years after diagnosis.
    • Wilms’ tumors are the third most common childhood cancer.
    • Wilms’ tumors make up 6% of all childhood cancers.
    • Wilms’ tumors usually occur between 3-4 years old.

Children with certain genetic syndromes and birth defects should be screened for Wilms’ tumor every three months until age 8. 15% of children with Wilms’ also have aniridia, WAGR syndrome, Beckwith-Wiedemann syndrome, hemihypertrophy, Denys-Drash syndrome, nephroblastomatosis, or hereditary Wilms’ tumor.


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