Predispostion to Wilms tumorWilms tumor, or nephroblastoma, represents ~90% of all pediatric renal tumors (5) and affects 1:10,000 children less than 15 years of age (6). Wilms tumor is an embryonal tumor that arises in a kidney cell with pluripotent differentiation potential. Most cases are unilateral (95%), sporadic, and curable with nephrectomy. Chemotherapy and radiation are also utilized in certain situations. In contrast, Wilms tumor associated with a cancer predisposition syndrome tends to occur at an earlier age and is more often associated with bilateral disease. The median age of diagnosis of patients with a Wilms tumor is 38 months, Review Article on Pediatric Urologic Malignancies

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