A case of Peutz-Jeghers syndrome combined with metastatic adenocarcinoma with unknown primary origin / 대한내과학회지

ABSTRACT

The Peutz-Jeghers syndrome, characterized by mucocutaneous pigmentation and gastrointestinal polyposis, appeares to be transmitted in an autosomal dominant fashion. This syndrome is clinically important because of complications caused by gastrointestinal polyps, such as abdominal pain, gastrointestinal bleeding and intussusception often leading to intestinal obstruction. The possibility of malignany change in the polyp has been a controversial issue. The hamatomatous polyps is not regarded to be premalignanct lesion, generally. But in patients with this syndrome, increased rates of both intestinal and extraintestinal malignancies has been reported. This report is concerned with our experience with a case of 46-year old male patients with Peutz-Jeghers syndrome who had an metastatic adenocarcinoma with unknown primary origin.