非典型性脑室外中枢神经细胞瘤一例报告及文献复习

Atypical extraventricular central neurocytoma： report of one case and literature review

目的 探讨提高非典型性脑室外中枢神经细胞瘤（EVN）诊疗水平的策略和方法.方法 对2010年1月南京大学医学院附属鼓楼医院神经外科收治的1例非典型性EVN的临床表现、影像学表现、病理学特征及显微外科手术预后等进行回顾性分析.结果 肿瘤位于左侧额部,与脑组织分界清楚,MRI检查T1WI相表现为等信号或稍低信号,T2WI相表现为略高信号,增强后强化不均匀,术前诊断为脑膜瘤.术中见肿瘤组织呈灰红色,质软、血供一般,快速病理倾向为高级别胶质瘤.术后免疫组化肿瘤细胞示GFAP部分（＋）,S100（＋）,NeuN（＋）,Syn（＋）,Olig2（＋）,CgA（＋）,NSE（＋）,EMA（-）,NF（＋）,CD99（-）,P53个别细胞（＋）,Ki-67约10%（＋）;提示增殖指数高,考虑为非典型性EVN.结论 EVN影像学表现虽具有一定的特征,但免疫组化结果对确诊很重要.显微手术是治疗最佳手段,预后可能与肿瘤的间变性和增殖指数有关.

Objective To explore the diagnosis and treatment of atypical extraventricular central neurocytoma. Methods We received 1 patient with atypical extraventricular central neurocytoma in January, 2010; the clinical and imaging manifestations, the pathologic features and microsurgical outcome were retrospectively analyzed; and the related literature was reviewed and compared. Results The lesions located at left frontal lobe, with clear boundaries to the brain tissue. Equal or slightly lower signals on MRI T1-weighted images （T1WI） and T2-weighted images （T2WI） was shown;inhomogeneous enhancement was noted on contrast-enhanced T1WI and T2WI. Meningioma was considered in the preoperative diagnosis. The tumor was grayish red and soft, and had general blood supply detected during the operation. Quick pathological diagnosis was made and it tended to be high-grade glioma. Immunohistochemistry indicated that the tumor cells were partly GFAP（＋）, S100（＋）NeuN（＋）, Syn（＋）, Olig2（＋）, CgA（＋）, NSE（＋） and NF（＋）, while those were EMA（-） and CD99（-）; some P53 cells and about 10% Ki-67 cells were positive. These results prompted that these tumors having high proliferation index and atypical extraventricular neurocytoma was considered. Conclusion The imaging manifestation of EVN has certain features while immunohistochemistry is very important for its diagnosis. The best treatment is to perform microsurgery; the prognosis may be related to proliferation index of the tumor.