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目的血管中心性胶质瘤(angiocentric glioma,AG)是2007年世界卫生组织新定义的一种具有独特临床病理特点的中枢神经系统实体肿瘤。本文报道1例AG致儿童难治性癫痫,并对相关文献进行了回顾。方法对一例7岁AG患儿采用术中皮层脑电监测再次定位致痫灶范围,并在显微镜下全切。并对其临床表现、影像学、病理检查及手术方法结合文献进行分析。结果本例患儿临床表现为药物难以控制的癫痫发作。头颅核磁共振扫描显示病变位于右顶叶皮质,大脑镰旁,T1低信号(长T1),T2高信号(长T2),FLAIR高信号,增强无明显强化,同时合并有左侧颞极蛛网膜囊肿。手术显微镜下全切除,组织病理学检查示:肿瘤细胞GFAP、S-100蛋白、Vimentin、EMA、CD99和D2-40阳性。Ki-67/MIBk-1标记指数约为1%。结论目前国内外文献报道的52例AG患者大多以癫痫发作为临床表现。这一新定义的实体肿瘤在影像学上有特征性的表现,但确诊依赖于组织病理学检查。术中采取皮层脑电定位致痫病灶,扩大切除病灶后患者预后良好。本例患儿术后随访半年未见复发及癫痫发作。
Objective angiocentric glioma (AG) is a new type of central nervous system solid tumor with unique clinicopathological features defined by the World Health Organization in 2007. This article reports 1 case of AG-induced childhood intractable epilepsy, and the relevant literature was reviewed. Methods A 7-year-old AG children with intraocular cortical EEG monitoring re-positioning of the range of epileptic foci, and under the microscope, the whole. And its clinical manifestations, imaging, pathological examination and surgical methods combined with the literature. Results The clinical manifestations of this case were seizure-refractory drugs. The skull MRI showed that the lesion was located in the right parietal cortex, the falx next to the brain, the T1 low signal (long T1), T2 high signal (long T2) and FLAIR high signal without significant enhancement, Cyst. Total resection under operation microscope, histopathological examination showed: tumor cells GFAP, S-100 protein, Vimentin, EMA, CD99 and D2-40 positive. Ki-67 / MIBk-1 labeling index is about 1%. Conclusion 52 cases of AG patients reported in the literature at home and abroad are mostly clinical manifestations of seizures. The newly defined solid tumor has imaging features, but the diagnosis depends on histopathology. Intraoperative cortical EEG positioning epileptogenic lesions, expand the prognosis of patients with good excision. In this case, no recurrence and seizure occurred in six months after operation.