目的:探讨中枢神经系统血管周细胞瘤(HPC)的临床病理特征、诊断与鉴别诊断。方法:回顾22例HPC患者的临床病理资料,通过影像、光镜、免疫组化及电镜进行观察分析,并结合文献对其临床表现、病理特点及鉴别诊断进行探讨。结果:本研究HPC患者的发病年龄从18～66岁,中位年龄48岁,男女发病比例为1∶1。22例中,WHOⅡ级16例,WHOⅢ级6例。9例患者随访6个月到6年,其中3例复发,1例转移。HPC的CT和MRI常表现为单发肿瘤,呈分叶状,血供丰富,有血管流空征象,钙化及“硬膜尾”征少见。免疫组化示波形蛋白(vimentin)和CD34均为阳性,CD99阳性14例(64%),BCL-2阳性9例(41%),上皮膜抗原(EMA)阴性或灶性阳性。网状纤维染色后可见单个瘤细胞被网状纤维包绕。电镜下可见单个瘤细胞周围包绕基板样无定形结构,细胞间无完整的桥粒和缝隙连接。结论:中枢神经系统HPC较罕见,易复发和转移,需与中枢神经系统的其他肿瘤鉴别。 Objective: To investigate the clinical and pathological features, diagnosis and differential diagnosis of central nervous system pericyte tumor (HPC). Methods: The clinical and pathological data of 22 patients with HPC were retrospectively analyzed and observed by imaging, light microscopy, immunohistochemistry and electron microscopy, and their clinical manifestations, pathological features and differential diagnosis were discussed. Results: The age of onset of HPC patients in this study ranged from 18 to 66 years, with a median age of 48 years. The incidence rate of male to female was 1: 1.22. There were 16 WHO Ⅱ and 6 WHO Ⅲ. Nine patients were followed up for 6 months to 6 years, of which 3 patients relapsed and 1 patient had metastases. CT and MRI HPC often showed single tumor, lobulated, rich in blood supply, signs of vascular flow, calcification and “hard tails ” sign rare. Immunohistochemistry showed that both vimentin and CD34 were positive, CD99 positive in 14 cases (64%), BCL-2 positive in 9 cases (41%), epithelial membrane antigen (EMA) negative or focal positive. After reticulated fiber staining showed that a single tumor cells surrounded by reticular fibers. Electron microscopy showed a single tumor cells surrounding the substrate around the amorphous structure, cells without complete desmosomes and gap junctions. Conclusion: Central nervous system HPC is rare, easy to relapse and metastasis, need to identify with other tumors of the central nervous system.