THE BECKWITH-WIEDEMANN-SYNDROME PHENOTYPE AND THE RISK OF CANCER

Beckwith-Wiedemann syndrome (BWS) comprises of a number of childhood a bnormalities, often associated with one or more tumors. Thirty-eight p atients were investigated to determine clinical and/or biological sign s associated with a tumor presence. Our patients exhibited a higher in cidence of tumor development (21%) than that previously reported, unde rlying the care with which such patients should be followed, when part icular clinical features are observed: visceromegaly affecting three o rgans (liver, kidney, spleen), and also family history (with sign of B WS such as macroglossia, omphalocele, hemihypertrophy, embryonic tumor ), high body weight at birth (greater than or equal to +2 standard dev iations) and diastasis recti. (C) 1997 Wiley-Liss, Inc.