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

Research output: Contribution to journalArticlepeer-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 languageEnglish
Pages (from-to)292-294
Number of pages3
JournalPrenatal Diagnosis
Volume23
Issue number4
DOIs
Publication statusPublished - Apr 1 2003

Keywords

  • Beckwith-Wiedemann syndrome
  • Pancreatoblastoma
  • Prenatal diagnosis
  • Ultrasonography
  • Uniparental disomy

ASJC Scopus subject areas

  • Genetics(clinical)
  • Obstetrics and Gynaecology

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