A Case of Peutz-Jeghers Syndrome with Multiple Intussusception and Rectal Adenocarcinoma / 대한소화기내시경학회지

ABSTRACT

Peutz-Jeghers syndrome (PJS) is a rare disease of autosomal dominant inheritance, which is characterized by hamartomatous gastrointestinal polyps and mucocutaneous melanin pigmentation. PJS often presents as surgical emergen cies with complications of the polyps, such as intussusception, small bowel obstruction, bleeding and volvulus. Intussusception caused by PJS polyps is often observed in the small bowel, but intussusception which involving small and large bowel concommittantly is not so much. The association between PJS and an increased risk for cancer has been controversial. Recent studies have shown PJS have a increased risk for both gastrointestinal and extraintestinal cancer. We report a case of PJS with rectal adenocarcinoma and multiple intussusceptions involving small and large bowel concommittantly.