急速な増悪経過を示したvon Recklinghausen病合併GISTの1例

抄録

A 70-year-old woman with von Recklinghausen’s disease was admitted for abdominal distention. Abdominal CT showed a huge solid tumor in the right abdomen and laparotomy was performed. Because the tumor originated from the ileum and invaded the ascending colon, we performed tumorectomy with right hemicolectomy. Pathological examination showed a high-risk GIST, with a tumor size of 110×100 mm, a mitotic rate of 50/50, and positive staining for KIT. The postoperative course was uneventful and the patient was discharged 15 days after the operation. Two weeks after the discharge, the patient was rehospitalized for anorexia. Abdominal CT revealed multiple liver, lung and peritoneal metastases. The patient was treated with imatinib mesylate, however, the tumors continued to grow rapidly. The patient died 51 days after the surgery. Herein, we have reported a case of von Recklinghausen’s disease with a rapidly growing GIST.