Korean J Gastroenterol.  2010 Jan;55(1):73-77. 10.4166/kjg.2010.55.1.73.

A Case of Peutz-Jeghers Syndrome with Intraductal Papillary Mucinous Carcinoma of Pancreas

Affiliations
  • 1Department of Internal Medicine, Chungnam National University College of Medicine, Daejeon, Korea. jeonghy@cnuh.co.kr
  • 2Department of Pathology, Chungnam National University College of Medicine, Daejeon, Korea.

Abstract

Peutz-Jeghers syndrome (PJS), which is characterized by multiple hamartomatous polyps of the gastrointestinal tract and mucocutaneous pigmentation, is a rare autosomal dominant disease. This syndrome is often represented as a surgical emergency with complications of the polyps such as intussusception, small bowel obstruction, bleeding, and volvulus. In particular, many studies have reported that patients with this syndrome have a high risk of gastrointestinal or extragastrointestinal malignancy including gastric, duodenal, jejunal, ileal, and colonic carcinoma as well as malignancies involving other organs such as the gallbladder, biliary tract, pancreas, tonsils, breast, and reproductive system. However, there are few reported cases of an association of this syndrome with extraintestinal malignancy. In addition to that, there is no reported case of this syndrome with malignant tumor or intraductal papillary mucinous tumor of pancreas in Korea. We experienced a case of PJS accompanying intraductal papillary mucinous carcinoma of the pancreas, therefore we report this case with literatures reviewed.

Keyword

Peutz-Jeghers syndrome; Intraductal papillary mucinous carcinoma

MeSH Terms

Adenocarcinoma, Mucinous/*diagnosis/pathology/secondary
Carcinoma, Papillary/*diagnosis/pathology/secondary
Humans
Intussusception/surgery
Jejunum/surgery
Male
Middle Aged
Pancreatic Neoplasms/complications/*diagnosis/pathology
Peutz-Jeghers Syndrome/complications/*diagnosis
Tomography, X-Ray Computed

Figure

  • Fig. 1. MRCP finding. Complex mass with multiple cluster like cystic lesions suggesting mixed type of IPMN (focal main duct and branch duct) and moderate dilatation of main pancreatic duct with smooth tapering at ampulla of Vater were observed.

  • Fig. 2. Abdominal CT finding. Cystic lesions (about 4 cm) in uncinate process of pancreatic head were observed (head arrows) and jejunojejunal intussusception (about 10 cm) due to jejunal polyps was observed (arrows).

  • Fig. 3. Macroscopic view of surgical resection specimen. (A) Cluster like mass of pancreatic head was observed. (B) Marked dilatation of porximal jejunum (intussusception site) due to jejunal polyp (head arrow) was noted.

  • Fig. 4. Histologic features of jejunal polyp & pancreatic mass. (A) Arborizing bundles of smooth muscle surrounded by small intestinal glandular epithelium was noted (H&E stain, ×40). (B) Papillary ingrowing malignant cell with mucin secreating was found and normal pancreatic islet structure was destructed (H&E stain, ×400).


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