摘要： 目的 探讨脑膜血管周细胞瘤的临床表现、影像学和病理学特征。方法与结果 男性患者，40 岁，头痛3 月余，加重2 周伴左侧肢体肌力下降。头部MRI 显示右侧颞叶占位性病变，T₁WI 等信号、T₂WI 混杂信号，增强扫描明显强化。手术全切除肿瘤。术中可见肿瘤位于颅中窝，体积较大，血供丰富，无包膜。组织形态学，肿瘤细胞密集，界限不清，胞核呈圆形、椭圆形或短梭形，有一定异型性，核分裂象易见（4/10 个高倍视野）；厚壁血管和血窦丰富，部分血管腔隙呈“鹿角”状。免疫组织化学染色，肿瘤细胞弥漫性表达CD34 和波形蛋白，局灶性表达上皮膜抗原，Ki-67 抗原标记指数为17% ~ 20%。术后辅助放射治疗，随访20 个月，肿瘤未复发。结论 脑膜血管周细胞瘤极易误诊为脑膜瘤，但二者预后相差较大，鉴别诊断具有重要意义。明确诊断需依靠病理学检查。

关键词: 血管肿瘤, 周细胞, 脑膜, 免疫组织化学, 病理学

Abstract: Objective  To investigate the clinical, neuroimaging and pathological features of meningeal hemangiopericytoma.  Methods  One case of meningeal hemangiopericytoma was reported, and the relevant literatures were also reviewed.  Results  A 40-year-old male had caught a headache for about 3 months with muscle weakness in the left limb, and became progressively serious for 2 weeks. Brain MRI displayed a space-occupying lesion in the right temporal lobe with equal signals in T₁WI, mixed signals in T₂WI and obvious enhancements. In surgery, the tumor was found to be located in the cranial fossa, and was completely removed. The tumor was large, with rich blood supply, and had no capsule. In histology, the neoplasm was composed of dense spindle cells with mild atypia. The boundary of the tumor cells was unclear. The nuclei were circular, oval or spindle with obvious mitoses (4/10 HPF). There were plenty of thick-wall blood vessels and blood sinuses with characteristic "staghorn" shape. In immunohistochemistry, CD34 and vimentin (Vim) were positive, epithelial membrane antigen (EMA) was focally positive and Ki-67 labeling index was 17%-20%. Postoperative radiotherapy was adopted and no relapse was found during the 20-month follow-up period. Conclusions  The meningeal hemangiopericytoma is easy to be misdiagnosed as meningioma, however, the prognosis of meningeal hemangiopericytoma is quite worse, thus the differential diagnosis is very important. A clear diagnosis often depends on pathological examination.

Key words: Vascular neoplasms, Pericytes, Meninges, Immunohistochemistry, Pathology