Wilms Tumor Predisposition

Excerpt

Purpose: This GeneReview is intended to help clinicians determine if a genetic basis can be identified in an individual with Wilms tumor in order to provide families with natural history and recurrence risk information.

Goal 1: Briefly describe the clinical characteristics of Wilms tumor.

Goal 2: Review the mechanisms of predisposition to Wilms tumor.

Goal 3: 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.

Goal 4: Review genetic counseling issues including mode of inheritance, recurrence risk, and evaluation of relatives at risk based on the underlying genetic mechanism.

Goal 5: Review management (e.g., tumor screening) recommendations for individuals with a genetic predisposition to Wilms tumor.