부산대학교 도서관

A 17-year-old girl presented to an emergency room clinic of nearby hospital for abdominal pain developed after dinner. Abdominal contrast-enhanced CT scan showed a pelvic tumor. She was diagnosed as having a right ovarian tumor, and was referred to the department of gynecology in our hospital for the treatment of this tumor. Following gynecological examination, a tumor of the small intestine was diagnosed, and then she was referred to our department for surgery. Abdominal contrast-enhanced CT scan showed a 9-cm tumor with irregular margin at the pelvis. A small intestinal tumor was suspected, and laparoscopic tumor extirpation was performed. A lobulated tumor 9cm in size was found in the pelvic cavity, and it was clarified to have originated from the small intestinal wall. It was twisted clockwise by about 540 degrees at the neck without apparent ischemic changes of the small intestine. The cut surface of the tumor looked whitish and multinodular. The histological findings revealed that spindle-shaped cells composed of the tumor, which were immunohistochemically positive for c-kit. The diagnosis of this tumor was small intestinal GIST, and a mutation analysis of the c-kit gene revealed this tumor to have a mutation in exon 11. She underwent adjuvant chemotherapy with imatinib. We report small intestinal GIST in a juvenile patient, which is a very rare entity, with a review of the literature.