title: [A case of Peutz-Jeghers syndrome with intraductal papillary mucinous carcinoma of pancreas].
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.
authors.affiliation: Department of Internal Medicine, Chungnam National University College of Medicine, Daejeon, Korea.
authors.forename: Pyung Gohn
authors.initials: PG
authors.lastname: Goh
authors.forename: Hee Seok
authors.initials: HS
authors.lastname: Moon
authors.forename: Jae Kyu
authors.initials: JK
authors.lastname: Sung
authors.forename: Hyun Yong
authors.initials: HY
authors.lastname: Jeong
authors.forename: Kyu Sang
authors.initials: KS
authors.lastname: Song
mesh_terms: D002288:Adenocarcinoma, Mucinous / Q000175:diagnosis* / Q000473:pathology / Q000556:secondary; D002291:Carcinoma, Papillary / Q000175:diagnosis* / Q000473:pathology / Q000556:secondary; D006801:Humans; D007443:Intussusception / Q000601:surgery; D007583:Jejunum / Q000601:surgery; D008297:Male; D008875:Middle Aged; D010190:Pancreatic Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D010580:Peutz-Jeghers Syndrome / Q000150:complications / Q000175:diagnosis*; D014057:Tomography, X-Ray Computed