title: Neurofibromatosis-1 and Ampullary Gangliocytic Paraganglioma Causing Biliary and Pancreatic Obstruction.
abstract: Gangliocytic paragangliomas (GPs) are exceedingly rare tumors that arise in close proximity of the papillaof Vater. Nevertheless, jaundice is an uncommon presenting symptom, reported in only 3 of 125 casesdescribed in the literature to date, with gastrointestinal bleeding being more common.Association between GPs and neurofibromatosis 1 (NF-1), described in two patients, may be more thancasual, being the frequency in normal population 1:3000.We report an additional case of this association, presenting for the first time with simultaneous obstructionof the common biliary duct, and of the main pancreatic duct. Previous cases of GPs with jaundice and/orNF-1 are reviewed, and updating of the histogenesis presented.
authors.affiliation: Istituto di Chirurgia d'urgenza, Ospedale Maggiore,Via F. Sforza 35, 20122 Milano, Italia.
authors.forename: Laura
authors.initials: L
authors.lastname: Castoldi
authors.forename: Paolo
authors.initials: P
authors.lastname: De Rai
authors.forename: Aldo
authors.initials: A
authors.lastname: Marini
authors.forename: Stefano
authors.initials: S
authors.lastname: Ferrero
authors.forename: Valeria
authors.initials: V
authors.lastname: De Luca
authors.forename: Giorgio
authors.initials: G
authors.lastname: Tiberio