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

Gloria Pelizzo; Giancarlo Conoscenti; Karim D. Kalache; Francesco Vesce; Paolo Guerrini; Luigi Cavazzini

doi:10.1002/pd.575

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

Gloria Pelizzo, Giancarlo Conoscenti, Karim D. Kalache, Francesco Vesce, Paolo Guerrini, Luigi Cavazzini

IRCCS Fondazione Policlinico San Matteo

Research output: Contribution to journal › Article › peer-review

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.

Original language	English
Pages (from-to)	292-294
Number of pages	3
Journal	Prenatal Diagnosis
Volume	23
Issue number	4
DOIs	https://doi.org/10.1002/pd.575
Publication status	Published - Apr 1 2003

Keywords

Beckwith-Wiedemann syndrome
Pancreatoblastoma
Prenatal diagnosis
Ultrasonography
Uniparental disomy

ASJC Scopus subject areas

Genetics(clinical)
Obstetrics and Gynaecology

Access to Document

10.1002/pd.575

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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.",

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AU - Pelizzo, Gloria

AU - Conoscenti, Giancarlo

AU - Kalache, Karim D.

AU - Vesce, Francesco

AU - Guerrini, Paolo

AU - Cavazzini, Luigi

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KW - Prenatal diagnosis

KW - Ultrasonography

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