Cancer Risk and Incidence in Beckwith-Wiedemann Syndrome
Beckwith-Wiedemann Syndrome (BWS) is the most common cancer predisposition and growth disorder that leads to cancer in children. Children with BWS can develop hepatoblastoma (liver cancer) and/or Wilms tumor (kidney cancer). The clinical definition of BWS has recently been revised to include children with subtle features. The cancer risk for children with BWS based on this new definition is unclear. In order to develop better cancer screening guidelines, this research focuses on understanding what the risk for cancer is in patients with BWS and whether the incidence of specific clinical feature(s) increases that risk. Additionally, this work will evaluate current cancer screening to determine if screening is effective at diagnosing cancer at earlier stages and leads to better outcomes for these children. This study will be performed using the BWS Registry developed at Children’s Hospital of Philadelphia and then will be extended to create a National BWS Research Network including other children’s hospitals across the United States.