Antenatal manifestation of congenital pancreatoblastoma in a fetus with Beckwith-Wiedemann syndrome.
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.
Pelizzo
Gloria
G
Department of Paediatric Surgery, IRCCS Burlo Garofolo, Trieste, Italy. pelizzog@libero.it
Conoscenti
Giancarlo
G
Kalache
Karim D
KD
Vesce
Fortunato
F
Guerrini
Pietro
P
Cavazzini
Luigi
L
D001506:Beckwith-Wiedemann Syndrome / Q000150:complications / Q000000981:diagnostic imaging* / Q000473:pathology; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008423:Maternal Age; D008875:Middle Aged; D009373:Neoplasms, Germ Cell and Embryonal / Q000150:complications / Q000000981:diagnostic imaging* / Q000601:surgery; D010190:Pancreatic Neoplasms / Q000150:complications / Q000000981:diagnostic imaging* / Q000601:surgery; D011247:Pregnancy; D018566:Pregnancy, High-Risk; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D016216:Ultrasonography, Prenatal