摘要： 目的：系统分析口腔颌面部恶性神经鞘瘤的影像学和病理学特征，提高对该疾病的认识。方法：收集2005—2013年我院收治并经病理检查确诊的8例口腔颌面部恶性神经鞘瘤的病历资料，系统分析其CT和MRI影像学表现，以及组织病理学特征。结果：颌骨内恶性神经鞘瘤的CT检查表现出骨质破坏、牙根吸收等恶性肿瘤特征。而软组织恶性神经鞘瘤的MRI检查表现为边界不清，T1WI呈等信号、T2WI压脂像呈高信号，信号不均匀，有不均匀强化等特点。病理学上，Vimentin免疫组织化学染色在所有病例的恶性神经鞘瘤细胞中为强阳性，S-100和Ki-67表现为部分肿瘤细胞表达阳性。CD34在7例患者肿瘤细胞中表达为阴性。结论：CT和MRI影像学检查可帮助确定颌面部恶性神经鞘瘤的性质、范围、形态和破坏程度及与周围组织的关系。Vimentin、S-100和Ki-67免疫组织化学染色可用于肿瘤术后的确诊。

关键词: 口腔颌面部；  , 恶性神经鞘瘤；  , 影像学；  , 病理学

Abstract: Objective: To review and analyze the clinical characteristics of malignant neurilemmoma in the oral and maxillofacial region in order to raise awareness of the disease. Methods: 8 patients (2005—2013) with malignant neurilemmoma, the clinicopathologic findings, immunohistochemical expressions, and imaging studies were retrospectively reviewed. Results: Microscopically, malignant neurilemmoma was very cellular and was composed of abundant spindle cells with variable numbers of abnormal mitotic figures. Streaming and palisading of nuclei were often seen. Nuclear pleomorphism may also be prominent. For immunohistochemistry, Vimentin protein was strongly positive in tumor tissues in all eight patients. S-100 and Ki-67 exhibited positive staining in part of tumor cells in all patients, whereas CD34 showed negative staining in tumor tissues in seven patients. CT image of the malignant neurilemmoma of the jaw exhibited features of malignant tumor, such as bony destruction and teeth root resorption. MRI image of the malignant neurilemmoma in the soft tissues showed ill-defined margins, heterogeneous signal and inhomogeneous enhancement. Conclusion: Microscopic separation of this lesion from other sarcomas may be difficult, making CT and MRI, and immunohistochemistry important diagnostic adjuncts.

Key words: oral and maxillofacial region, malignant neurilemmoma, radiographic image, pathology

中图分类号: 

• R782

• R739.8