Volume 23, Issue 4 p. 292-294
Short Communication

Antenatal manifestation of congenital pancreatoblastoma in a fetus with Beckwith–Wiedemann syndrome

Dr Gloria Pelizzo

Corresponding Author

Dr Gloria Pelizzo

Department of Paediatric Surgery, IRCCS Burlo Garofolo, Trieste, Italy

Dipartimento di Chirurgia Pediatrica, Istituto Burlo Garofolo, Via dell'Istria n. 65/1, 34100 Trieste, Italy.Search for more papers by this author
Giancarlo Conoscenti

Giancarlo Conoscenti

Department of Obstetrics and Gynaecology, IRCCS Burlo Garofolo, Trieste, Italy

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Karim D. Kalache

Karim D. Kalache

Department of Obstetrics and Gynaecology, Campus Charité Mitte, Berlin, Germany

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Francesco Vesce

Francesco Vesce

Neonatal Intensive Care and Obstetrics, Department for Mother and Child Health, School of Medicine, S. Anna Hospital, Ferrara, Italy

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Paolo Guerrini

Paolo Guerrini

Neonatal Intensive Care and Obstetrics, Department for Mother and Child Health, School of Medicine, S. Anna Hospital, Ferrara, Italy

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Luigi Cavazzini

Luigi Cavazzini

Department of Pathology, School of Medicine, S. Anna Hospital, Ferrara, Italy

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First published: 05 March 2003
Citations: 27

Abstract

An Erratum has been published for this article in Prenatal Diagnosis 23(9) 2003, 771

Antenatal detection of an isolated abdominal cyst was found to be a pancreatoblastoma in a female fetus with Beckwith–Wiedemann syndrome. Prenatal and post-natal features and management of this very rare tumour are discussed. Molecular investigation disclosed a mosaic paternal 11p15 uniparental disomy in the tumoral cells. The prognosis of a congenital pancreatoblastoma is good if complete surgical excision is achieved. However, the association with Beckwith–Wiedemann syndrome requires a prolonged follow-up because of the increased risk of developing malignant tumours. Copyright © 2003 John Wiley & Sons, Ltd.