Korean J Gastrointest Endosc > Volume 34(2); 2007 > Article
Korean Journal of Gastrointestinal Endoscopy 2007;34(2): 83-87.
제1형 신경섬유종증 환자에서 발생한 위선암 및 복강 내 신경초종 1예
임동한 · 황현철 · 오준석 · 김현주 · 이화목 · 안진광 · 김광진 · 정종윤 · 박원일 · 양웅석
봉생병원 내과
A Case of Early Gastric Adenocarcinoma and Intraabdominal Schwannoma in a Patient with Neurofibromatosis Type I
Dong Han Im, M.D., Hyun Chul Whang, M.D., Joon Seok Oh, M.D., Hyun Ju Kim, M.D., Hwa Mock Lee, M.D., Jin Kwang An, M.D., Kwang Jin Kim, M.D., Jong Yun Cheong, M.D., Won Il Park, M.D. and Ung Suk Yang, M.D.
Division of Gastroenterology, Department of Internal Medicine, Bong Seng Memorial Hospital, Busan, Korea
Abstract
Neurofibromatosis is an autosomal dominant hereditary disorder with an overall incidence of one in 3,000∼4,000, and type 1 (Von Recklinghausen's neurofibromatosis) characterized by the presence of multiple cutaneous neurofibromas, axillary and groin freckling, and cafe- au-lait spot. The neurofibromatosis type 1 gene is a tumor suppressor gene. Patients with the neurofibromatosis type 1 are at increased risk of developing nervous system neoplasm, including plexiform neurofibromas, optic gliomas, ependymomas, meningiomas, astrocytomas, and pheochromocytomas. Neurofibromas may undergo secondary malignant degeneration and sarcomatous changes. Patients with neurofibromatosis type 1 show a high incidence of Wilm's tumor, rhabdomyosarcoma, nonlymphocytic leukemia, and pheochromocytoma but the gastrointestinal involvement appears to be relatively rare and usually consists of neurofibroma, ganglioneuroma, and leiomyoma. We have identified a case of early gastric adenocarcinoma and intraabdominal schwannoma in a 65-year-old man afflicted with neurofibromatosis type 1.
Key Words: Neurofibromatosis type I, Adenocarcinoma, Schwannoma
주요어: 제1형 신경섬유종증, 위선암, 신경초종
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