Abstract: Due to lack of clinical and imaging features，the diagnosis of malignant neurilemmoma of the lung（MNL） needs to be performed in accordance with the results of imaging exanimation combined with immunohistochemistry and other examinations.Based on the diagnosis and management of one MNL case of ours and review of other 55 MNL cases reported in domestic and international studies，we summarized the diagnosis and treatment of MNL，with a view to increasing clinicians' awareness level of this disease.

Key words: Neurilemmoma, Lung, Diagnosis

摘要： 肺内恶性神经鞘瘤缺乏特异性临床表现及影像学特征，其确诊尚需要影像学检查结合病理及免疫组化检查等多种方法的共同分析。为此，本文报道了1例肺内恶性神经鞘瘤的诊治过程，并结合国内外55例肺内神经鞘瘤的文献资料，对该病进行归纳总结，旨在提高临床医生对肺内神经鞘瘤的认识。

关键词: 神经鞘瘤, 肺, 诊断