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.
D001506:Beckwith-Wiedemann Syndrome / Q000150:complications*; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D009447:Neuroblastoma / Q000150:complications* / Q000151:congenital / Q000473:pathology; D010179:Pancreas / Q000473:pathology; D013899:Thoracic Neoplasms / Q000150:complications* / Q000151:congenital / Q000473:pathology