1. ¹Department of Surgery, Section of Anatomic Pathology, University of Parma School of Medicine, Parma, Italy
2. ²Institute of General Surgery, and Department of Pathology, Section of Anatomic Pathology, University of Parma School of Medicine, Parma, Italy

Correspondence: Cesare Bordi, M.D., Istituto di Anatomia ed Istologia Patologica, Via Gramsci 14, 43100 Parma, Italia; e-mail: cesare.bordi@unipr.it; fax: 39-0521-292710.

Accepted 12 July 2001.

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.