ORAL MANIFESTATIONS OF NEUROFIBROMATOSIS TYPE 1: CASE REPORT |
Sun-Yeon Gwon, Tae-Wan Kim, Young-Jin Kim, Hyun-Jung Kim, Soon-Hyeun Nam |
Department of Pediatric Dentistry, College of Dentistry, Kyungpook National University |
제1형 신경섬유종증 환아의 구강내 병소의 치험례 |
권순연, 김태완, 김영진, 김현정, 남순현 |
경북대학교 치과대학 소아치과학교실 |
Correspondence:
Soon-Hyeun Nam, Tel: 053-420-5964, Email: dent-ksy@hanmail.net |
Received: 7 March 2008 • Accepted: 10 June 2008 |
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Abstract |
Neurofibromatosis is an autosomal dominant disorder caused by a mutation of a tumor supressor gene on the long arm of chromosome 17. There are two types of neurofibromatosis, and development of neurofibroma is one of clinical diagnostic criteria for neurofibromatosis. The clinical signs of neurofibromatosis include as skin lesions, bone deformities, and tumors involving central nervous system. About 25% of neurofibromatosis involves oral neurofibroma. Radiographically, oral neurofibroma is well-defined unilocular radiolucency, which involves mandibular canal, mandibular foramen and mental foramen. When a lesion is small and approachable, complete resection, including 1cm of marginal connective tissue, is feasible. However, there are studies reporting that the recurrence rate after surgical resection is high and frequent recurrence may even increase the risk of malignant transformation. This case reports a patient with neurofibromatosis type I, accompanying oral neurofibroma, who shows a favorable result after surgical resection of the oral lesion. |
Key Words:
Neurofibromatosis Type 1, Neurofibroma |
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