신경섬유종증 환자에서 발생한 복강 내 총상형 신경섬유종 1예

Neurofibromatosis which was first described by von Recklinghausen is an autosomal dominant inheritance disease, affecting one in every 3000 to 4000 people. It is characterized by multiple pigmentations of skin and multiple neurofibromas of skin, CNS and peripheral nerve sheaths. We experienced a case of von Recklinghausen's disease in a 39-year-old female who had multiple subcutaneous nodules and had complained of epigastric pain intermittently from 3 months ago. Her family had a history of multiple subcutaneous nodules. Abdominal computed tomography scan showed well- defined, low-attenuated, round lesion through the gastrohepatic ligament and the lesser sac. Explolaparotomy and surgical excision were performed. Grossly, this lesion revealed oval-shaped, well- encapsulated benign solitary soft tissue mass. It was diagnosed as a plexiform neurofibroma of vagus nerve. Afterward, her abdominal symptoms were improved. Plexiform neurofibroma is a local invasive tumor of Schwann's cell origin, which involve the deep and large nerve trunk. (Kor J Gastroenterol 2000;36:132 - 136)