室管膜下巨细胞星形细胞瘤的临床及病理特点分析(附2例报告)  

Clinicopathological study on 2 cases of subependymal giant cell astrocytomas

作　　者：梁乐[1] 马康平[2] 付静[1] 李云林[2] 姚晓香[1] 

机构地区：[1]北京市海淀医院/北京大学第三医院海淀院区病理科,100080 [2]首都儿科研究所附属儿童医院神经外科

出　　处：《临床神经外科杂志》2017年第3期186-189,共4页Journal of Clinical Neurosurgery

基　　金：北京市海淀医院/北京大学第三医院海淀院区青年科研项目(KYQ2017004);首都儿科研究所基金(Fx201704)

摘　　要：目的探讨室管膜下巨细胞星形细胞瘤(SEGAs)的临床、病理特征和免疫组化标记及鉴别诊断。方法分析2例SEGAs患者的临床特点,观察组织病理切片和免疫组化标记。结果 2例SEGAs患者的发病年龄均小于20岁,肿瘤位于侧脑室壁,由肥胖型肿瘤细胞/节细胞样巨细胞和长梭形肿瘤细胞组成,均伴钙化。免疫组化显示,肿瘤细胞表达GFAP、S-100、vimentin、Map-2、Nestin、SOX-2、Caspase-3、Caspase-9、Bax,不表达Neun、CD133、CD34、Ki-67。结论 SEGAs常伴结节硬化复合症,可能起源于多潜能的神经干细胞,存在凋亡机制的激活;需与肥胖细胞型星形细胞瘤、室管膜瘤、脉络丛乳头状瘤、室管膜下瘤进行鉴别。Objective To study the clinicopathology,immunohistochemical characteristic and the differential diagnosis of subependymal giant cell astrocytomas （SEGAs）.Methods Two cases of SEGAs were studied by clinicopathological study and immunohistochemistry.Results Two cases of SEGAs occurred during the first two decades of life,arising in the wall of the lateral ventricles and were composed of gemistocytes and sweeping bundles of spindle cells with calcification.Immunohistochemistry showed GFAP,S-100,vimentin,Map-2,Nestin,SOX-2,Caspase-3,Caspase-9 and Bax positive;Neun,CD133,CD34 and Ki-67 negative.Conclusions SEGAs occur commonly in tuberous sclerosis complex,may arise from multipotent neural stem cells,have activation of apoptotic mechanisms and should be differentiated with gemistocytic diffuse astrocytoma,ependymoma,choroid plexus papilloma and subependymoma.

关 键 词：室管膜下巨细胞星形细胞瘤 结节性硬化 免疫组化 

分 类 号：R446.8[医药卫生—诊断学]