中枢神经系统血管母细胞瘤74例临床病理学分析  

Central nervous system hemangioblastomas:a clinicopathological analysis of 74 cases

作　　者：李恒[1] 钟文[1] 黄亮亮[1] 孙思柏 陈杰[1] 吴海波[1] LI Heng;ZHONG Wen;HUANG Liang-liang;SUN Si-bai;CHEN Jie;WU Hai-bo(Department of Pathology,South Branch of Anhui Provincial Hospital,Hefei 230036,China)

机构地区：[1]安徽省立医院南区病理科,合肥230036

出　　处：《诊断病理学杂志》2018年第8期552-555,共4页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨中枢神经系统血管母细胞瘤(HB)的临床病理特点和免疫表型。方法回顾性分析74例中枢神经系统HB的临床病史、病理学形态和免疫组化标记结果。结果 74例中男性44例,女性30例,平均发病年龄43.1岁。肿瘤多发生于小脑,也可见于松果体、脑干和脊髓等部位。其中有13例为复发病例,6例为van Hippel-Lindau(VHL)综合征。肿瘤大小0.5~4 cm不等。组织学上HB由间质细胞和薄壁血管构成。免疫组化染色间质细胞NSE和α-inhibin(+),部分病例S-100和D2-40(+),薄壁血管CD34和CD31等血管标记(+)。结论中枢神经系统HB较少见,部分为VHL综合征。组织形态上HB有其独有的特点,免疫组化有助于辅助诊断。Objective To investigate the clinicopathological and immunological phenotypes of central nervous system hemangioblastoma（ HB）.Methods Clinicopathological features and the immunophenotypes of 74 HBs patients were retrospectively analyzed.Results There were 44 males and 30 females,and the average age was 43.1 years.The tumor most frequently affected the cerebellum,the pineal gland,brain stem and spinal cord might also be involved.There were13 cases of recurrence,and 6 cases as a component of van Hippel-Lindau（ VHL） disease.The size of tumors were 0.5 to4 cm.Histologically,HB was composed of stromal cells and thin-walled vessels.Immunohistochemically,the stromal cells were positive for NSE andα-inhibin,and S100 and D2-40 were also detected in some cases.CD31 and CD34 stains highlighted the capillary network of the tumor vasculature.Conclusions HB is a rare tumor in central nervous system,a portion of cases in the setting of VHL syndrome.HB has its unique histomorphological characteristics,and the immunohistochemical profile can aid in diagnosis.

关 键 词：中枢神经系统 血管母细胞瘤 VHL综合征 免疫组化 

分 类 号：R739.4[医药卫生—肿瘤]