Neurofibromatosis-1 and Ampullary Gangliocytic Paraganglioma Causing Biliary and Pancreatic Obstruction
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Updated: Sep 26, 2026

Application of Robot-assisted Pancreaticobiliary Junction Resection in Benign Duodenal Tumors
Published on: December 20, 2024
Laura Castoldi1, Paolo De Rai, Aldo Marini
1Istituto di Chirurgia d'urgenza, Ospedale Maggiore,Via F. Sforza 35, 20122 Milano, Italia.
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.
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