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Antenatal manifestation of congenital pancreatoblastoma in a fetus with Beckwith–Wiedemann syndrome |
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| Authors: | Dr Gloria Pelizzo Giancarlo Conoscenti Karim D Kalache Francesco Vesce Paolo Guerrini Luigi Cavazzini |
| Institution: | 1. Department of Paediatric Surgery, IRCCS Burlo Garofolo, Trieste, Italy;2. Department of Obstetrics and Gynaecology, IRCCS Burlo Garofolo, Trieste, Italy;3. Department of Obstetrics and Gynaecology, Campus Charité Mitte, Berlin, Germany;4. Neonatal Intensive Care and Obstetrics, Department for Mother and Child Health, School of Medicine, S. Anna Hospital, Ferrara, Italy;5. Department of Pathology, School of Medicine, S. Anna Hospital, Ferrara, Italy |
| 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. |
| Keywords: | pancreatoblastoma Beckwith–Wiedemann syndrome ultrasonography prenatal diagnosis uniparental disomy |