The Beckwith-Wiedemann syndrome phenotype and the risk of cancer
H. Schneid PhD
Laboratoire d'Explorations Fonctionnelles Endocriniennes, Hopital Armand-Trousseau, Paris, France
Search for more papers by this authorM.P. Vazquez MD
Service de Chirurgie Maxillo-faciale, Hopital Armand-Trousseau, Paris, France
Search for more papers by this authorC. Vacher MD
Service de Chirurgie Maxillo-faciale, Hopital Armand-Trousseau, Paris, France
Search for more papers by this authorM. Gourmelen MD
Laboratoire d'Explorations Fonctionnelles Endocriniennes, Hopital Armand-Trousseau, Paris, France
Search for more papers by this authorS. Cabrol MD
Laboratoire d'Explorations Fonctionnelles Endocriniennes, Hopital Armand-Trousseau, Paris, France
Search for more papers by this authorCorresponding Author
Y. Le Bouc MD
Laboratoire d'Explorations Fonctionnelles Endocriniennes, Hopital Armand-Trousseau, Paris, France
Laboratorie d'Explorations Fonctionnelles Endocriniennes, Hôpital A. Trousseau, 26 Ave du Dr. A. Netter, 75571 Paris Cedex 12, FranceSearch for more papers by this authorH. Schneid PhD
Laboratoire d'Explorations Fonctionnelles Endocriniennes, Hopital Armand-Trousseau, Paris, France
Search for more papers by this authorM.P. Vazquez MD
Service de Chirurgie Maxillo-faciale, Hopital Armand-Trousseau, Paris, France
Search for more papers by this authorC. Vacher MD
Service de Chirurgie Maxillo-faciale, Hopital Armand-Trousseau, Paris, France
Search for more papers by this authorM. Gourmelen MD
Laboratoire d'Explorations Fonctionnelles Endocriniennes, Hopital Armand-Trousseau, Paris, France
Search for more papers by this authorS. Cabrol MD
Laboratoire d'Explorations Fonctionnelles Endocriniennes, Hopital Armand-Trousseau, Paris, France
Search for more papers by this authorCorresponding Author
Y. Le Bouc MD
Laboratoire d'Explorations Fonctionnelles Endocriniennes, Hopital Armand-Trousseau, Paris, France
Laboratorie d'Explorations Fonctionnelles Endocriniennes, Hôpital A. Trousseau, 26 Ave du Dr. A. Netter, 75571 Paris Cedex 12, FranceSearch for more papers by this authorAbstract
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.
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