目的:神经脂肪瘤病(LN)是由于神经外膜内脂肪和纤维化组织的大量增殖而引起的神经的大量扩张,包括脑内和脑外脂肪瘤。LN经常与软组织和/或骨过度生长有关。不幸的是,由于LN使用了许多名称(例如,纤维脂肪瘤性错构瘤,脂肪巨营养不良,等等)。为了更好地了解这种情况并评估其与神经区域过度生长的关系,作者试图汇编世界上已发表的LN案例的文献。
方法:搜索PubMed和GoogleScholar数据库,以识别有关LN病例的已发表文章,使用各种各样的术语。对所有语言的出版物进行了评估。阅读所有病例被确定为LN的出版物。提供LN明确临床病理和/或放射学证据的病例被标记为“明确”,以及显示LN特征的病例(例如,神经区域过度生长),但缺乏明确的神经受累证据,被标记为“可能”。\"
结果:初步筛选显示共2465篇论文。排除后,281篇出版物报道了明确诊断为LN的病例,120篇文章报道了可能诊断为LN的病例。作者确定了618例明确的LN和407例可能的LN病例。性别分布均衡(51%为女性)。早期诊断很常见,三分之二的患者在生命的第一个十年有症状。最常见的受影响的神经是正中神经(n=391)。神经区域过度生长很常见(62%明确的LN;78%的合并病例);在所有情况下,过度生长都是受影响神经的专区,但只有5例。
结论:作者对LN病例的文献进行了全面回顾和分析。主要发现之一是神经区域过度生长与LN有关,尤其是在生命早期出现的时候。作者认为,所有与过度生长相关的LN病例都可以从解剖学上解释,即使在少数报告的案例中,这一点并不立即明显。
Lipomatosis of nerve (LN) is a massive enlargement of a nerve due to abundant proliferation of adipose and fibrotic tissue within the epineurium-part of the spectrum of adipose lesions of nerves, including intra- and extraneural lipomas. LN has been frequently associated with soft-tissue and/or osseous overgrowth. Unfortunately, much confusion exists since many names have been used for LN (e.g., fibrolipomatous hamartoma, macrodystrophia lipomatosa, and so on). To better understand this condition and to evaluate its association with nerve-territory overgrowth, the authors attempted to compile the world\'s literature on published LN cases.
PubMed and Google Scholar databases were searched to identify published articles on LN cases, using a variety of terms. Publications in all languages were assessed. All publications with cases determined likely to be LN were read. Cases that provided clear clinicopathological and/or radiological evidence of LN were labeled as \"definite\" and cases that demonstrated features of LN (e.g., nerve-territory overgrowth) but lacked definite proof of nerve involvement were labeled as \"probable.\"
Initial screening revealed a total of 2465 papers. After exclusions, 281 publications reported cases with a definite diagnosis of LN and 120 articles reported cases with a probable diagnosis of LN. The authors identified 618 definite and 407 probable cases of LN. Sex distribution was balanced (51% female). Early diagnosis was common, with two-thirds of patients having symptoms in the 1st decade of life. The most commonly affected nerve was the median nerve (n = 391). Nerve-territory overgrowth was common (62% definite LN; 78% combined cases); overgrowth was exclusive to the territory of the affected nerve in all cases but 5.
The authors present a comprehensive review and analysis of the literature of LN cases. One of the main findings was the nerve-territory overgrowth was associated with LN, especially when present earlier in life. The authors believe that all cases of LN associated with overgrowth can be explained on anatomical grounds, even in the few reported cases in which this is not immediately obvious.