title: Pancreatoblastoma associated with incomplete Beckwith-Wiedemann syndrome: case report and review of the literature.
abstract: A case of pancreatoblastoma (PB) in a 2 month-old male infant with incomplete Beckwith-Wiedemann syndrome is presented. Clinical examination disclosed left hemihypertrophy, macroglossia, bilateral exophthalmos, and enlargement of the left testis. Imaging with ultrasound and computed tomography scan showed a well-defined, heterogeneous, and grossly cystic mass arising from the head of the pancreas. Serum alpha-fetoprotein (AFP) level was elevated. The tumor was completely resected, and the histological analysis showed PB. The patient's recovery was uneventful, and AFP returned to normal values after surgery. The child has been disease-free for 5 years, and his serum AFP remained within normal values. Six other examples of this association, PB, and Beckwith-Wiedemann syndrome are recorded in the literature. The risk of developing tumor in this syndrome (complete and incomplete form) increases when hemihypertrophy is present, and the need for routine screening examination is warranted. Beckwith-Wiedemann syndrome was suggested to be a favorable biological marker for survival in children who have intraabdominal tumors.
authors.affiliation: Department of Pediatric Surgery, Complejo Hospitalario Xeral-Cíes, Pizarro 22, 36204 Vigo, Spain. rmuguerza@msn.com
authors.forename: Rosa
authors.initials: R
authors.lastname: Muguerza
authors.forename: Alberto
authors.initials: A
authors.lastname: Rodriguez
authors.forename: Emilia
authors.initials: E
authors.lastname: Formigo
authors.forename: Margarita
authors.initials: M
authors.lastname: Montero
authors.forename: José L
authors.initials: JL
authors.lastname: Vázquez
authors.forename: Cristina
authors.initials: C
authors.lastname: Páramo
authors.forename: Carmen
authors.initials: C
authors.lastname: Campos
mesh_terms: D001506:Beckwith-Wiedemann Syndrome / Q000150:complications*; D006801:Humans; D007223:Infant; D008297:Male; D009373:Neoplasms, Germ Cell and Embryonal / Q000150:complications* / Q000601:surgery; D010190:Pancreatic Neoplasms / Q000150:complications* / Q000601:surgery; D000509:alpha-Fetoproteins / Q000032:analysis
chemical_list: D000509:alpha-Fetoproteins