Asymmetry and Tumor Formation in Beckwith-Wiedemann Syndrome

Background

Beckwith-Wiedemann syndrome (BWS) is a childhood cancer predisposition disorder in which up to 25% of children develop cancer. Children with BWS are screened for tumors with abdominal ultrasounds every 3 months until they are 8 years old. Two of the common features of BWS are enlarged organs and hemihypertrophy, a condition where one side or part of the body is bigger than the other. The goal of this project is to determine if tumors are more likely to develop in the enlarged organ and/or on the larger side of the body. We have developed and currently manage the only active BWS registry in the United States.

Project Goal

Using the registry data, we will look at overall growth, organ size, body symmetry, and tumor formation in children with BWS and hemihypertrophy. We will look for relationships between asymmetry, the size of particular organs, and the conditions during tumor development. The analysis performed will also be used to create normal growth curves for BWS. Enlarged kidney sizes of hemihypertrophy patients will be analyzed to formulate and refine tumor-screening protocols for these patients. This work will help to improve our ability to predict which patients are at greatest risk for cancer.