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


Pelizzo, Gloria, Conoscenti, Giancarlo, Kalache, Karim D., Vesce, Francesco, Guerrini, Paolo and Cavazzini, Luigi (2003) Antenatal manifestation of congenital pancreatoblastoma in a fetus with Beckwith-Wiedemann syndrome. Prenatal Diagnosis, 23, (4), 292-294. (doi:10.1002/pd.575).

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Original Publication URL: http://dx.doi.org/10.1002/pd.575

Description/Abstract

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.

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

Item Type: Article
Additional Information: Short Communication
ISSNs: 0197-3851 (print)
Related URLs:
Keywords: pancreatoblastoma, Beckwith-Wiedemann syndrome, ultrasonography, prenatal diagnosis, uniparental disomy
Subjects: R Medicine > RG Gynecology and obstetrics
R Medicine > RC Internal medicine > RC0254 Neoplasms. Tumors. Oncology (including Cancer)
Divisions: University Structure - Pre August 2011 > School of Medicine > Developmental Origins of Health and Disease
ePrint ID: 25896
Date Deposited: 20 Apr 2006
Last Modified: 27 Mar 2014 18:14
Contact Email Address: pelizzog@libero.it
URI: http://eprints.soton.ac.uk/id/eprint/25896

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