The Beckwith-Wiedemann syndrome phenotype and the risk of cancer

Beckwith‐Wiedermann syndrome (BWS) comprises of a number of childhood abnormalities, often associated with one or more tumors. Thirty‐eight patients were investigated to determine clinical and/or biological signs associated with a tumor presence. Our patients exhibited a higher incidence of tumor development (21%) than that previously reported, underlying the care with which such patients should be followed, when particular clinical features are observed: visceromegaly affecting three organs (liver, kidney, spleen), and also family history (with sign of BWS such as macroglossia, omphalocele, hemihypertrophy, embryonic tumor), high body weight at birth (⩾ +2 standard deviations) and diastasis recti. Med. Pediatr. Oncol. 28:411–415, 1997. © 1997 Wiley‐Liss, Inc.