A Case of Duodenal Adenocarcinoma in the Peutz-Jeghers Syndrome / 대한내과학회지

ABSTRACT

The Peutz-Jeghers syndrome is an autosomal dominant disease characterized by hamartomatous polyps in the gastrointestinal tract and mucocutaneous melanin pigmentation. Although these polyps are believed to have little potential for malignancy, and the disease was believed to have a relatively benign course, it recently has been recognized that patients with this syndrome are at increased risk for the development of cancer at gastrointestinal and nongastrointestinal sites. A 33-year-old male patient was admitted because of vomiting and abdominal pain for 3 months duration. A diagnosis of Peutz-Jeghers syndrome was made 3 years ago by multiple hamartomatous polyps confined to the colon and mucocutaneous pigmentation. A barium study showed abrupt string like luminal narrowing at the 4th portion of the duodenum. On laparotomy, there was an annular constricting mass involving the serosa of duodenum with multiple metastasis to liver, so a segmental resection of small bowel followed by chemotherapy was performed. The histologic finding was adenocarcinoma.