Neuroblastoma associated with Beckwith-Wiedemann syndrome.

This report describes an infant with Beckwith-Wiedemann Syndrome (BWS) found to have a thoracic neuroblastoma. Infants with BWS are known to have an increased incidence of tumors (10%). We believe this to be the first report of a malignant neural crest tumor in a child with BWS and also the first case occurring in familial BWS. Usually the malignancies associated with the BWS are intraabdominal; however, this malignancy was located in the chest. It is important to detect malignancy in early stages. Therefore, recommendations for screening children with BWS are discussed.