About Wilms' Tumor

What is Wilms’ Tumor?

Wilms’ tumor is a malignant tumor that occurs in the kidney. The kidneys filter waste out of the bloodstream, creating urine which passes into the bladder. 

It is named for a German surgeon, Max Wilms who published the first comprehensive review of the disease in 1899.  It is also known as nephroblastoma – nephro meaning kidney and a blastoma is a tumor made up of embryonic tissue that has not fully developed.

Facts about Wilms’ Tumor

• There are approximately 500 new cases in the US annually.  Wilms’ tumor accounts for approximately 8% of all cancers in children.
• The incidence of this tumor peaks at about 3 years of age and becomes less common as children get older.  It is very uncommon after the age of six.
• The causes of Wilms’ tumor are not known although some tumors appear to result from changes in one or more of several genes.
• The most common sign of Wilms' tumor is a lump or mass in the abdomen in an otherwise well appearing child.
• There are other rarer tumors of the kidney that are also diagnosed in children.  These tumors are different than Wilms based on their appearance under the microscope as well as their clinical characteristics and their treatment.

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Children’s Oncology Group Kidney Tumors Committee

The Children’s Oncology Group Kidney Tumors Committee is focused on improving the cure rate while decreasing the amount of treatment, thus decreasing toxicities experienced by the patient both during treatment and long term. In addition to improving Wilms’ tumor therapy, researchers have uncovered important insights into the biology and genetics of Wilms’ tumor.

Children’s Oncology Group research has revealed several Wilms’ tumor genes and new biological markers have been identified. These will be used to predict those patients at greatest risk for tumor relapse.  One of these prognostic markers, called “loss of heterozygosity,” is already being used in current COG trials to assign treatment.  The assignment of treatment based upon risk of relapse will allow patients to receive more individualized treatment for their specific type of tumor.

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