Korean J Med.  1999 Mar;56(3):378-382.

A case of Peutz - Jeghers syndrome with duodenal adenocarcinoma and obstruction

• Lee KI
• Han SG
• Heo JH
• Kim HJ
• Pai YM
• Whang YH
• Cho SR
• Son S

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.

Keyword

Peutz-Jeghers syndrome; Harmatomatous polyp; Signet ring cell type adenocarcinoma; Intestinal obstruction

MeSH Terms

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
Melanins
Phenobarbital