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. 2011:2011:364570.
doi: 10.1155/2011/364570. Epub 2011 Jun 13.

Endopancreatic bile duct cholangiocarcinoma in a patient with peutz-jeghers syndrome

Alexandros K Charalabopoulos  1 , Sylvia P Krivan, Nikolas A Machairas, Evangelos P Misiakos, Anastasios N Machairas
Affiliations

Endopancreatic bile duct cholangiocarcinoma in a patient with peutz-jeghers syndrome

Alexandros K Charalabopoulos et al. HPB Surg. 2011.

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.

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Figures

Figure 1
Figure 1
Magnetic resonance cholangiopancreatography showing dilated biliary tree and gallbladder. Arrow points at obstruction at the lower end of the common bile duct.
Figure 2
Figure 2
Contrast-enhanced axial computer tomography scan showing multiple polyps of the stomach, duodenum, and ileum.
Figure 3
Figure 3
Intraoperative finding of diffuse rice-like metastases throughout the liver (arrows).
Figure 4
Figure 4
Excised large Peutz-Jeghers polyps from the duodenal wall.
See this image and copyright information in PMC

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