The purpose of this GeneReview is to:
Briefly describe the clinical characteristics of Wilms tumor;
Review the mechanisms of predisposition to Wilms tumor;
Provide an evaluation strategy to determine if a proband with Wilms tumor has a predisposition to Wilms tumor, identify the genetic or epigenetic mechanism for Wilms tumor, and determine risks for additional medical complications;
Review management (e.g., tumor screening) recommendations for individuals with a genetic predisposition to Wilms tumor;
Review genetic counseling issues including mode of inheritance, recurrence risk, and evaluation of relatives at risk based on the underlying genetic mechanism.
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