A Case of Peutz-Jeghers Syndrome with Intraductal Papillary Mucinous Carcinoma of Pancreas / 대한소화기학회지
The Korean Journal of Gastroenterology
;
: 73-77, 2010.
Article
in Korean
| WPRIM
| ID: wpr-138045
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.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Pancreatic Neoplasms
/
Peutz-Jeghers Syndrome
/
Carcinoma, Papillary
/
Tomography, X-Ray Computed
/
Adenocarcinoma, Mucinous
/
Intussusception
/
Jejunum
Limits:
Humans
/
Male
Language:
Korean
Journal:
The Korean Journal of Gastroenterology
Year:
2010
Type:
Article
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