A case of Peutz - Jeghers syndrome with duodenal adenocarcinoma and obstruction.
- Author:
Kyung Il LEE
1
;
Sang Gil HAN
;
Jung Ho HEO
;
Hyung Jin KIM
;
Young Mok PAI
;
Young Hoon WHANG
;
Sung Rak CHO
;
Shin SON
Author Information
1. Department of Internal Medicine, Dae Dong Hospital, Pusan, Korea.
- Publication Type:Case Report
- Keywords:
Peutz-Jeghers syndrome;
Harmatomatous polyp;
Signet ring cell type adenocarcinoma;
Intestinal obstruction
- MeSH:
Abdominal Pain;
Adenocarcinoma*;
Adult;
Duodenum;
Foot;
Gastrointestinal Tract;
Hand;
Humans;
Intestinal Obstruction;
Intussusception;
Laparotomy;
Lip;
Lymph Nodes;
Male;
Melanins;
Mouth Mucosa;
Nausea;
Neoplasm Metastasis;
Peutz-Jeghers Syndrome;
Phenobarbital;
Polyps;
Tomography, X-Ray Computed
- From:Korean Journal of Medicine
1999;56(3):378-382
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Peutz-Jeghers syndrome is a rare autosomal dominant disorder defined by harmatomatous polyps of the gastrointestinal tracts and the occurrence of melanin spots on the lips(oral mucosa), hands and feet. This syndrome is associated with a variety of gastrointestinal and extraintestinal neoplasms, both benign and malignant. A 28-year old male was admitted to our hospital with abdominal pain and nausea for two months. He had melanin spots involving his lips, buccal mucosa, hands and feet. A small bowel series showed luminal obstruction at the forth portion of the duodenum. And abdominal CT scan showed target-like mass suggesting intussusception on the small bowel. On laparotomy, there was an annular constricting mass adherent to retroperitoneum with metastasis to preaortic lymph nodes. Palliative duodeno-jejunostomy was performed. The histologic finding disclosed signet-ring cell type adenocarcinoma.
