[A case of Peutz-Jeghers syndrome with intraductal papillary mucinous carcinoma of pancreas].
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.
Goh
Pyung Gohn
PG
Department of Internal Medicine, Chungnam National University College of Medicine, Daejeon, Korea.
Moon
Hee Seok
HS
Sung
Jae Kyu
JK
Jeong
Hyun Yong
HY
Song
Kyu Sang
KS
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