家族性中枢神经系统血管网织细胞瘤的病理学观察

目的：探讨家族性中枢神经系统（CNS）血管网织细胞瘤（angioreticuloma,ARM）的病理特点及其组织来源.方法：应用光镜、免疫组织化学染色及电镜,对4例手术的CNS ARM患者的肿瘤组织和6例脑外伤患者手术后的脑组织（作为对照）进行病理学观察.结果：HE染色肿瘤组织可见丰富的毛细血管将间质细胞分割呈巢状分布,间质细胞体积大,因胞浆富含类脂滴而呈细小的空泡状;外伤的对照脑组织未见肿瘤成分,由神经元细胞及神经胶质细胞组成,局灶区域神经元细胞变性、坏死;AMR间质细胞波形蛋白（vimentin）、血管内皮生长因子受体（VEGF）、α抑制素（inhibin）及神经元特异性烯醇化酶（NSE）染色均呈强阳性;AMR内皮细胞CD31、CD34及vimentin均呈强阳性表达;但是胶质纤维酸性蛋白（GFAP）、角蛋白抗原（CK）、上皮膜抗原（EMA）染色表达均阴性;增殖细胞核抗原（Ki-67）标记指数,除1例第2次手术为2％,余均为1％;透射电子显微镜观察：AMR中的毛细血管由周细胞和内皮细胞构成,内皮细胞的胞浆内可见到Weibel-Palade小体;间质细胞位于毛细血管网之间,间质细胞呈椭圆形,细胞核大,染色深,胞浆中可见较丰富的脂质空泡.结论：家族性CNS AMR可能来源于成血管间叶组织,间质细胞是肿瘤细胞,肿瘤细胞的增殖活性并不高.

Pathologic Study on Familial Central Nervous System Hemangioblastoma

Objective：To analyze pathologic characteristics of a familial central nervous system （CNS） angioreticuloma （ARM）,and to explore its histological origin.Methods：Light microscopy,electron microscopy and immunohistochemical staining were applied for pathological observation of tumor tissues of 4 patients with CNS AMR and 6 traumatic brain tissues after operation.Results：HE staining showed abundant capillaries splitting the stromal cells into nested distribution.Interstitial cells were large in size,cytoplasm was rich in lipid droplets and was finely vacuolated.Tumor cells were not found in traumatic brain tissues which were composed of neuron and neuroglia cells,neuron degeneration and necrosis were seen in focal regions.AMR interstitial cells strongly positively expressed vimentin,vascular endothelial growth factor （VEGF）,α-inhibin,and neuron specific enolase （NSE）;AMR endothelial cells strongly positively expressed CD31,CD34 and vimentin; but glial fihrillary acidic protein （GFAP）,cytokeratin （CK） and epithelial membrane antigen （EMA） staining were all negative.Proliferating cell nuclear antigen （Ki-67） labeling index was 1％,except for in one case who accepted second operation was 2％.Electron microscopy observation：capillaries of AMR were composed of endothelial cells and pericytes,and Weibel-palade bodies were visible in the cytoplasm of endothelial cells; and interstitial cells were located between the blood vessels.Stromal cells were oval in shape,with large nuclei,pale stained,and cytoplasm contained abundant lipid droplets.Conclusions：Familial of CNS AMR may originate from hematopoietic mesenchymal tissue,and the proliferative activity of the tumor cells is not high.