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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Data di pubblicazione: | 2003 | |
Titolo: | Antenatal manifestation of congenital pancreatoblastoma in a fetus with Beckwith-Wiedemann syndrome. | |
Autori: | G. Pelizzo,G. Conoscenti,K. D. Kalache,F. Vesce,P. Guerrini,L. Cavazzini AUTHOR CORRECTIONS: Two of the author names in the above paper, published in the April issue of Prenatal Diagnosis (2003; 23: 292–294), were supplied erroneously. The correct names are Fortunato Vesce and Pietro Guerrini. The authors would like to apologise for any inconvenience caused (PRENATAL DIAGNOSIS Prenat Diagn 2003; 23: 771) | |
Rivista: | PRENATAL DIAGNOSIS | |
Parole Chiave: | Beckwith-Wiedemann Syndrome; complications/pathology/ultrasonography, Female, Humans, Infant; Newborn, Maternal Age, Middle Aged, Neoplasms; Germ Cell and Embryonal; complications/surgery/ultrasonography, Pancreatic Neoplasms; complications/surgery/ultrasonography, Pregnancy, Pregnancy; High-Risk, Tomography; X-Ray Computed, Treatment Outcome, Ultrasonography; Prenatal | |
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. | |
Digital Object Identifier (DOI): | 10.1002/pd.575 | |
Handle: | http://hdl.handle.net/11392/1683531 | |
Appare nelle tipologie: | 03.1 Articolo su rivista |