非典型脑室外神经细胞瘤3例及文献复习  

Atypical extraventricular neurocytoma: a clinicopathologic analysis of 3 and review of literature

作　　者：崔华娟[1] 赖日权[1] 彭大云[1] 赖续文[1] 陈敬文[1] 

机构地区：[1]广州军区广州总医院病理科,广州510010

出　　处：《临床与实验病理学杂志》2013年第12期1337-1341,共5页Chinese Journal of Clinical and Experimental Pathology

摘　　要：目的探讨颅内非典型脑室外神经细胞瘤(atypical extraventricular neurocytoma,AEVN)的临床病理学特征。方法对3例罕见发生于颅内的AEVN临床表现、影像学、组织学形态、免疫表型及电镜下特征进行分析,并复习相关文献。结果男性2例,女性1例,年龄分别为5、36及3岁。各位于左额叶、左额颞叶及左侧基底节。组织学上,瘤细胞呈一致中等大小,弥漫分布,大部分细胞胞质透明,细胞间可见小片状分布的神经毡样结构,3例瘤细胞均有异型,核分裂象>3个/10 HPF,均见血管内皮增生,1例见坏死。免疫表型:3例瘤细胞Syn均弥漫阳性,Ki-67增殖指数>3%,NeuN呈不同程度的阳性。2例GFAP少量瘤细胞阳性。电镜下见瘤细胞胞质内有神经内分泌颗粒,细胞间见突触结构。结论 AEVN临床较罕见,组织形态学类似间变性少突胶质细胞瘤、间变性室管膜瘤等,易误诊。确诊需综合组织形态学观察、免疫组化标记及电镜特征。Purpose To explore the clinicopathological characteristics of encephalic atypical extraventricular neurocytoma. Methods The clinical manifestations, image and histopathological features, immunohistoehemical findings and electron microscopic changes were analyzed in three cases of uncommonly encephalic atypical extraventricular neurocytoma, with review of relative literature. Results Two male and one female patients aged 5, 36 and 3 years, respectively. One tumor was found in the left frontal lobe, one in the left frontal temporal lobe, and the other in the left basal ganglia. Histologically, all tumors were composed of uniform medium-sized clear cells with small neuropil-like structure. The nucleus of the tumor cells had heteromorphism and the mitoses were more than 3 per 10 high power field. Microvascular hyperplasia was noted in three cases and necrosis was seen in one case. Immunohistochemically, the tumor cells of three cases were positive for synaptophysin, Ki-67 index was more than 3% and NeuN had different degree of positive staining, and focally positive for GFAP in two cases. Electron microscopic investigation showed neuroendocrine granules in the cell cy-toplasm and synapses was present between the tumor cells. Conclusion Atypical extraventricular neurocytoma is a rare tumor, which is difficult to be distinguished histopathogically from anaplastic oligodendroglioma, anaplastic ependymoma etc. The accurate diagnosis should be relied on histopathologieal features, immunohistochemistry and electron microscopy.

关 键 词：脑室外神经细胞瘤 颅内肿瘤 非典型 免疫组织化学 

分 类 号：R739.41[医药卫生—肿瘤]