A case of Peutz-Jegher syndrome associated with advanced small intestinal carcinoma metastasizing to the ovary

A 33-year-old woman was given a diagnosis of Peutz-Jeghers syndrome at the age of 2 years, and had undergone laparotomy 4 times due to abdominal pain and/or intussusception. She had abdominal distention and visited the department of gynecology of our hospital. Computed tomography showed massive ascites and a right ovarian tumor, and cytology of the ascites indicated adenocarcinoma. Ovarian cancer with peritoneal dissemination was diagnosed. Gynecologists performed a right salpingo-oophorectomy, however, an intestinal tumor 100 cm distal to the Treitz ligament was found. Partial resection of the small intestine was performed by gastrointestinal surgeons. The intestinal tumor was a papillary semipedunculated mass 6 cm in diameter and exposed to the serous membrane. Pathological examination revealed moderately differentiated adenocarcinoma concomitant with adenoma and hamartoma components. The ovarian tumor was histologically diagnosed to be metastasis from the carcinoma of the small intestine. Twelve cases of Peutz-Jeghers syndrome associated with small intestinal carcinoma have been reported in Japanese literature, and most of their carcinomata are thought to have originated from polyps of the small intestine. Patients with Peutz-Jeghers syndrome should be closely followed up, taking care of malignancy of the gastrointestinal tract and other organs.