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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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
|Digital Object Identifier (DOI):||doi:10.1002/pd.575|
|Additional Information:||Short Communication|
|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
|Date Deposited:||20 Apr 2006|
|Last Modified:||31 Mar 2016 11:48|
|RDF:||RDF+N-Triples, RDF+N3, RDF+XML, Browse.|
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