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KMID : 0358920080350030556
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Journal of the Korean Academy of Pedodontics
2008 Volume.35 No. 3 p.556 ~ p.561
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ORAL MANIFESTATIONS OF NEUROFIBROMATOSIS TYPE 1: CASE REPORT
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Kwon Soon-Yeon
Nam Soon-Hyeun
Kim Young-Jin
Kim Hyun-Jung
Kim Tae-Wan
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Abstract
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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.
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KEYWORD
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Neurofibromatosis Type 1, Neurofibroma
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