中枢神经系统血管周细胞瘤的临床病理分析  

Hemangiopericytoma of central nervous system:a clinicopathological analysis

作　　者：李良[1,2] 王海[1] 袁斯明[3] 张正祥[1] 周航波[1] 马恒辉[1] 姜少军[1] 陆珍凤[1] 刘翔宇[4] 石群立[1] 

机构地区：[1]南京大学医学院临床学院南京军区南京总医院病理科,江苏南京210002 [2]南京江北人民医院病理科,210044 [3]南京大学医学院临床学院南京军区南京总医院烧伤整形科,江苏南京210002 [4]南京大学医学院临床学院南京军区南京总医院神经外科,江苏南京210002

出　　处：《诊断学理论与实践》2009年第1期67-71,共5页Journal of Diagnostics Concepts & Practice

基　　金：国家自然科学基金资助(30700874)

摘　　要：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 analyze the clinicopathological features, diagnosis and differential diagnosis of hemangiopericytoma （HPC） of the central nervous system. Methods The clinical manifestations, pathological characteristics and differential diagnosis of HPC were analyzed retrospectively by examining the data of 22 cases of HPC studied by imaging, light microscopy, immunohistochemistry and electron microscopy. Results Age of the HPC patients ranged from 18 to 66 （median 48）, and ratio of male to female was 1：1. Of the 22 cases, 16 were WHO grade Ⅱ and 6 were WHO grade Ⅲ. Nine cases had been followed-up with a period ranging from 6 months to 6 years, recurrence was seen in 3 cases and metastasis in 1. CT and MRI showed that the tumor was generally single and lobular, with abundant blood supply and flow-void sign. Calcification and ＂dural tail sign＂ were rarely seen. Immunohistochemically, vimentin and CD34 were positive in all the cases, CD99 was positive in 14 （64%）, BCL-2 in 9 （41%）. EMA was negative or focally positive. Reticular fiber staining displayed every tumor cell was surrounded by reticular fiber. Under electron microscopy, the tumor cells were encapsulatd by basal lamina-like amorphous structure, and there were no apparent intercellular desmosomes and gap junctions. Conclusions HPC is an infrequent tumor of the central nervous system and has a tendency of recurrence and metastasis. It should be differentiated from other tumors of the central nervous system.

关 键 词：中枢神经系统 血管周细胞瘤 诊断 鉴别诊断 

分 类 号：R732.2[医药卫生—肿瘤]