【摘要】 目的探讨脑脊髓血管母细胞瘤的临床病理特征及间质细胞的起源，为该肿瘤的病理诊断和鉴别诊断提供病理形态依据。方法对33例脑脊髓血管母细胞瘤作回顾性临床病理观察，其中8例进行GFAP、Vimmentin、CD34、Mac387．、a－AT、CK免疫组化标记；3例作了电镜观察。结果发病年龄从7～75岁，平均37．9岁；27例发生于小脑，占81．8％，其他在脑干、脊髓、第四脑室占18．2％；22例发生囊性变（66．7％）；有颅内高压症状者26例，占78．79％，共济失调者19例，占57．58％，二者同存者15例，占45．45％；术后复发者4例，占12．12％。肿瘤内间质细胞对GFAP、Pac387、a－AT、CK缺乏阳性表达，而Vimmentin阳性。电镜证实肿瘤细胞具有血管内皮细胞分化特征。结论脑脊髓血管母细胞瘤是一种好发于成人，临床以颅内高压症状为主要特征，其间质细胞具有表达间叶细胞特性的术后易复发的肿瘤。更多还原

【Abstract】 Objectives To assess the clinicopathological features and histogenesis of stromal cells of hemangioblastoma in brain and spinal cord . Methods The clinicopathological features of 33 cases of hemangioblastoma in brain and spinal cord were investigated , 8 cases were studied by immunohistochemical technique (GFAP, vimentin , CD34, Mac87, a-AT, CK), 3 cases were studied by electron microscopy . Results Clinical manifestations : Their ages ranged from 7 to 74 years old (aversge,37.9), the tumor was located in the cerebella in 27 cases (81 .8% ), in truncus encephalicus , spinal cord and the ventriculus quartus in the rest (18.2% ), there were cyctic changes of the tumor in 22 cases (66.7% ), symptoms of intracranial hypertension in 26 cases (78.9% ), dystonic movements in 19 cases (57.6% ), both intracranial hypertension and dystonic movements in 15 cases (45.5% ), and 4 cases (12. 1% ) recurred . The stomal cells of hemangioblastoma were negative to GFAP, Mao387, a-AT and CK, whereas positive to vimentin expression , and electron microscopic examination showed that the tumor cells had the characteristic of endotheliocyte deviation. Conclusions Hemangioblastoma mostly occurs in adults. Its main clinical characteristic isintracranial hypertension . It is a neoplasm easily to be recurrent and its stromal cells have vimentin expression.更多还原