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目的探讨血管中心性胶质瘤(AG)临床病理学特征及预后。方法收集血管中心性胶质瘤2例,常规石蜡切片,行HE染色、免疫组化标记及电镜检测。结果目前国内外文献报道的AG共36例,本文报道2例。AG以儿童和年轻人多发,长期难治性癫是其主要表现形式,常累及额顶叶、颞叶皮质浅层。MRI检查显示实性占位。形态学上表现为单一性双极梭形肿瘤细胞围绕血管中心生长,平行或垂直于血管分布。肿瘤细胞常GFAP、vimentin、S-100和EMA(+),Syn、CgA和p53(-),增殖指数Ki-67≤1%。电镜检测肿瘤细胞呈星形细胞样分化,偶见发育不好的细胞连接,未见微绒毛及腔隙样结构。外科手术切除则癫症状消失,分别随访14个月和3个月MRI显示均无复发。结论 AG为一类新提出的神经系统肿瘤,因其临床以难治性癫为主要表现形式,临床表现和MRI又存在多样性,故诊断主要依赖于病理学。该肿瘤典型的形态学特征、免疫组化及电镜表现均提示其存在星形细胞和室管膜细胞分化,需要与多种常见的神经系统肿瘤进行鉴别。AG治疗以手术切除为主,临床愈后佳。
Objective To investigate the clinicopathological characteristics and prognosis of vascular central glioma (AG). Methods Two cases of vascular central gliomas were collected. The paraffin sections were stained with HE, immunohistochemically and electron microscopically. Results There are 36 cases of AG reported in the literature at home and abroad, two cases were reported in this paper. AG with children and young people, long-term refractory epilepsy is its main manifestations, often involving the frontal lobe, temporal cortex shallow. MRI examination showed solid space. Morphologically, monopolar bipolar spindle tumor cells grow around the center of blood vessels, parallel or perpendicular to the blood vessels. Tumor cells often GFAP, vimentin, S-100 and EMA (+), Syn, CgA and p53 (-), proliferation index Ki-67 ≤ 1%. Electron microscopy tumor cells were astrocytic like differentiation, occasionally poorly developed cell connections, no microvilli and lacunar structure. Surgical resection of the epileptic symptoms disappeared, were followed up for 14 months and 3 months MRI showed no recurrence. Conclusions AG is a new type of nervous system tumor. Because of its clinical manifestations of refractory epilepsy, clinical manifestations and MRI also have diversity, so the diagnosis mainly depends on the pathology. The typical morphological features of the tumor, immunohistochemistry and electron microscopy showed that there are astrocytes and ependymal cell differentiation, the need for identification with a variety of common nervous system tumors. AG treatment mainly by surgical resection, clinical better.