Korean Journal of Pediatrics 2006;49(2):203-207.
Published online February 15, 2006.
Gastric neurofibroma in von Recklinghausen disease : a cause of upper gastrointestinal bleeding
Bo Sang Kwon1, Jeong Ok Shim1, Jeong Kee Seo1, Hye Ran Yang1, Jae Sung Ko1, Seong Eun Jung2, Woo Sun Kim3, Gyeong Hoon Kang4
1Departments of Pediatrics, Surgery*, Radiology† and Pathology, Seoul National University College of
2Departments of Surgery, Seoul National University College of Medicine, Seoul, Korea
3Departments of Radiology, Seoul National University College of Medicine, Seoul, Korea
4Departments of Pathology, Seoul National University College of Medicine, Seoul, Korea
Von Recklinghausen disease병 환아에서 상부위장관 출혈로 발현한 위의 신경섬유종 1례
권보상1, 심정욱1, 서정기1, 양혜란1, 고재성1, 정성은2, 김우선3, 강경훈4
1서울대학교 의과대학 소아과학교실
2서울대학교 의과대학 외과학교실
3서울대학교 의과대학 진단방사선과학교실
4서울대학교 의과대학 병리학교실
Jeong Kee Seo, Email: jkseo@snu.ac.kr
Neurofibromatosis type 1 (von Recklinghausen disease, NF1) involves the central and peripheral nervous systems as well as the skin, bone, endocrine, gastrointestinal and vascular systems. The gastrointestinal neurofibroma associated with NF1 has been infrequently reported. We report our experience with a 15-year-old boy who had a gastric plexiform neurofibroma with upper gastrointestinal bleeding and underwent a tumorectomy because of massive upper gastrointestinal bleeding. We conclude that gastrointestinal bleeding and anemia in the setting of NF1 mandates complete endoscopic examination of the digestive tract to rule out neurofibromas. Surgical resection is the standard treatment.
Key Words: Neurofibromatosis , Plexiform neurofibroma , Stomach , Gastrointestinal bleeding

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