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

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...

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Bibliographic Details
Published in:HPB Surgery 2011, Vol.2011 (2011), p.20-23
Main Authors: Charalabopoulos, Alexandros K., Krivan, Sylvia P., Machairas, Nikolas A., Misiakos, Evangelos P., Machairas, Anastasios N.
Format: Article
Language:English
Subjects:
Cancer
Case Report
Genetic aspects
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Summary: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.
ISSN:0894-8569
1607-8462
DOI:10.1155/2011/364570