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.

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

VESCE, Fortunato;
2003

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.
G. P. e. l. i. z. z. o., . G. Conoscenti; Vesce, Fortunato; Guerrini, . P.; Cavazzini AUTHOR CORRECTIONS: Two of the author names in the above paper, . L.; published in the April issue of Prenatal, Diagnosis; The correct names are Fortunato Vesce, were supplied e. r. r. o. n. e. o. u. s. l. y.; The authors would like to apologise for any inconvenience caused, Pietro G. u. e. r. r. i. n. i.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11392/1683531
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