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HPB Surgery
Volume 2011, Article ID 364570, 4 pages
http://dx.doi.org/10.1155/2011/364570
Case Report

Endopancreatic Bile Duct Cholangiocarcinoma in a Patient with Peutz-Jeghers Syndrome

3rd Department of Surgery, Attikon University Hospital, University of Athens Medical School, 1 Rimini Street, Chaidari, 12462 Athens, Greece

Received 17 January 2011; Revised 12 March 2011; Accepted 13 April 2011

Academic Editor: Guntars Pupelis

Copyright © 2011 Alexandros K. Charalabopoulos et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Peutz-Jeghers syndrome is a rare autosomal dominant inherited disease characterized by a special type of hamartomatous gastrointestinal polyps combined with mucocutaneous melanin pigmentations. Patients with the syndrome have a high risk of developing neoplasia, with colon, small bowel, and stomach being the most common gastrointestinal sites. Herein, we present the occurrence of a rare tumor in patients with Peutz-Jeghers syndrome; a cholangiocarcinoma of the endopancreatic bile duct. A minireview is also presented. It can be concluded that cholangiocarcinoma remains a possible diagnosis in PJS patients, as in others that present with biliary obstruction. PJS patients may be at higher risk than others in view of their propensity for malignancy.