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首页> 外文期刊>National Journal of Maxillofacial Surgery >Intraosseous schwannoma of the mandible and schwannoma of the spinal cord: A rare presentation of schwannomatosis – Case report and review of the literature
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Intraosseous schwannoma of the mandible and schwannoma of the spinal cord: A rare presentation of schwannomatosis – Case report and review of the literature

机译：下颌骨骨内神经鞘瘤和脊髓神经鞘瘤：神经鞘瘤病的罕见表现–病例报告和文献复习

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摘要

Schwannomatosis is a recently recognized distinct form of neurofibromatosis (NF). It is a rare condition, the incidence of which varies between 1/400,000 and 1/1.7 million. An important feature of schwannomatosis is the presence of multiple intracranial, spinal, and peripheral schwannomas in the absence of acoustic neuromas. Schwannomatosis presenting with intraosseous schwannoma of the mandible is even rarer, and only a few cases have been reported. It usually affects individuals in the third to fifth decade of life. Usually, it is sporadic in origin, but in 20% of patients, it can be familial. As a diagnostic criterion, NF2 gene is not involved in schwannomatosis. We report a case of a 48-year-old male presenting with facial pain and difficulty in chewing, and subsequent development of spastic paraplegia. Magnetic resonance imaging scan of head and neck revealed mass lesion involving infratemporal region on the left side, intraosseous lesion of the mandible, and multiple mass lesions in the neck. Acoustic nerves were not involved. Mutagen-induced chromosome sensitivity analysis test suggested no predisposition for malignancy. His clinical features are suggestive of schwannomatosis, which is a recently recognized distinct form of NF.
机译：Schwannomatosis是最近公认的神经纤维瘤病（NF）的独特形式。这是一种罕见的疾病，其发病率在1 / 400,000到1/170万之间。神经鞘瘤病的一个重要特征是在没有听神经瘤的情况下存在多个颅内，脊柱和周围神经鞘瘤。表现为下颌骨内神经鞘瘤的神经鞘瘤病更为罕见，仅报道了少数病例。它通常会影响到生命的第三到第五个十年。通常，它是零星的起源，但是在20％的患者中，它可能是家族性的。作为诊断标准，NF2基因不参与神经鞘瘤病。我们报告了一例48岁男性出现面部疼痛和咀嚼困难，以及随后的痉挛性截瘫的发展。头颈的磁共振成像扫描显示左侧左侧颞下区域，下颌骨骨内病变以及颈部多处肿块。不涉及声神经。诱变剂引起的染色体敏感性分析测试表明没有恶性倾向。他的临床特征提示神经鞘瘤病，这是最近公认的一种独特的NF形式。

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