제 1형 신경섬유종증에 동반된 위를 침범한 악성 림프종 1예

Abstract

Neurofibromatosis type 1, an autosomal dominant disorder, is clinically characterized by the presence of cafe-au-lait spots and cutaneous neurofibromas. Malignancy is a frequently cited and feared complication of neurofibromatosis. Neurofibromatosis has been reported to be associated with some CNS tumors, neurofibrosarcoma, pheochromocytoma, and nonlymphocytic leukemia. Also malignant lymphoma may develop in patients with neurofibromatosis. In Korea, only a case of lung cancer and a case of pheochromocytoma have been reported to develop in patients with neurofibromatosis type 1. Recently, we experienced a case of malignant lymphoma involving the stomach in a 70-year-old woman with neurofibromatosis type 1. An esophagogastroduodenoscopy revealed a 5 cm sized ulcerofungating mass on the greater curvature of the upper body. Histologic diagnosis of the biopsied specimen was B-cell diffuse small and large cell lymphoma. This case illustrates that malignant lymphoma should be included in the differential diagnosis of gastric tumors in patients with neurofibromatosis type 1.