제1형 신경섬유종증 환아의 구강내 병소의 치험례
Oral manifestations of neurofibromatosis type 1: Case report
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초록
신경섬유종증은 상염색체 우성 유전성 질환으로, 17번 염색체의 장완에 위치한 종양 억제 유전자의 변성에 의해 발생한다. 이는 두가지 형이 있으며 신경섬유종의 발생은 제1형 신경섬유종증의 임상적 진단 기준 중 하나이다. 신경섬유종증의 임상적 증상으로는 피부병소, 골변형, 중추신경계의 종양 등이 있으며 환자의 25%가 구강내 신경섬유종을 보인다. 악골내 신경섬유종은 드물며 방사선학적으로 하악공, 하악관, 이공을 포함하고 단방성으로 잘 경계된 방사선 투과성으로 나타난다. 신경섬유종은 하나 또는 그 이상의 병소에서 신경육종으로 전이된다는 점에서 임상적으로 중요하며 현재 특이한 치료법은 없으나, 외과적 절제술이 좋은 치료법으로 여겨지고 있다. 본 증례는 제1형 신경섬유종증 진단을 받은 환아로, 구강내 신경섬유종의 외과적 절제 후 양호한 결과를 보여 이를 보고하는 바이다.
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.
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