Ampullary adenocarcinoma in neurofibromatosis type 1. Case report and literature review.
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.
Costi
R
R
Department of Surgery, Institute of General Surgery, University of Parma School of Medicine, Via Gramsci 14, 43100 Parma, Italy.
Caruana
P
P
Sarli
L
L
Violi
V
V
Roncoroni
L
L
Bordi
C
C
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*