title: Ampullary adenocarcinoma in neurofibromatosis type 1. Case report and literature review.
abstract: Periampullary tumors in patients affected by Neurofibromatosis Type 1 (NF-1) are usually carcinoids or stromal tumors and, rarely, adenocarcinomas. We report a case of an adenocarcinoma of the ampulla of Vater in a 54-year-old woman with NF-1 admitted to the hospital with jaundice and undergoing pancreato-duodenectomy. Histologically, the resected specimen showed an adenocarcinoma of the ampulla as being a part of a complex atypical epithelial proliferation extended from the papilla to the mucosa of the duodenum and distal choledochus, islet-cell adenomatosis of the pancreas and multiple gastric, duodenal, jejunal stromal tumors. The ampullary and periampullary adenocarcinomas in NF-1 patients have peculiar features, suggesting a widespread predisposition to cancer development in periampullary tissues and requiring widely demolitive surgery. Moreover, they occur at a younger age than those occurring in non-NF-1 patients, may be associated with additional periampullary epithelial tumors, are often operable and may present long survival.
authors.affiliation: Department of Surgery, Institute of General Surgery, University of Parma School of Medicine, Via Gramsci 14, 43100 Parma, Italy.
authors.forename: R
authors.initials: R
authors.lastname: Costi
authors.forename: P
authors.initials: P
authors.lastname: Caruana
authors.forename: L
authors.initials: L
authors.lastname: Sarli
authors.forename: V
authors.initials: V
authors.lastname: Violi
authors.forename: L
authors.initials: L
authors.lastname: Roncoroni
authors.forename: C
authors.initials: C
authors.lastname: Bordi
mesh_terms: D000230:Adenocarcinoma / Q000150:complications / Q000473:pathology*; D014670:Ampulla of Vater / Q000473:pathology*; D003138:Common Bile Duct Neoplasms / Q000150:complications / Q000473:pathology*; D005260:Female; D006801:Humans; D008875:Middle Aged; D009456:Neurofibromatosis 1 / Q000150:complications*