DNT临床病理分析五例

Clinicopathological analysis of 5 cases with dysembryoplastic neuroepithelial tumor

目的探讨胚胎发育不良性神经上皮肿瘤(dysembryoplastic neuroepithelial tumor,DNT)的临床病理学特征。方法复习与总结武汉大学中南医院病理科2016年7月-2019年11月诊断为DNT的5个病例的临床、影像、病理资料。结果 5例DNT,均为男性,年龄12~22岁,患者常出现癫痫症状。影像学表现:5例DNT MRI T;WI均呈低信号,3例在T;WI呈低信号,2例呈稍低信号,1例病灶周围见少许水肿信号,3例病灶显示轻度强化。病理学改变:5例均可见"特殊胶质神经元成分",表现为与皮质表面垂直的柱状结构,柱状结构是由少突胶质细胞样细胞沿轴突排列构成,这些柱状结构之间可见淡嗜酸性的黏液基质,基质内漂浮着正常神经元,5例DNT均为简单型,1例为复杂型。免疫组化特点:少突胶质细胞样细胞表达少突胶质细胞转录因子-2 (Olig-2),神经元表达神经元核抗原(NeuN)、神经突触素(Syn),星形细胞表达胶质纤维性酸性蛋白(GFAP),细胞增殖核抗原(Ki-67)水平极低,约1%。结论外科手术治疗DNT具有较好的效果,本文报道的5例均未复发。

Objective To explore clinicopathological characteristics of dysembryoplastic neuroepithelial tumor(DNT).Methods 5 cases of these DNT in the department of Pathology,Zhongnan Hospital of Wuhan University from July 2016 to November 2019 were evaluated by Clinical,imaging and pathological data,and reviewed the literature.Results Clinical manifestations:5 cases of DNT were all male,12-22 years old,with symptoms of epilepsy.Imaging findings:5 cases of DNT showed low signal on MRI T;WI,3 cases showed low signal on T;WI,2 cases showed slightly low signal,1 case showed a little edema around the lesion,and 3 cases showed mild enhancement.Pathological findings:Five case all showed a specific "glioneuronal element",which is characterized by columns oriented perpendicularly to the cortical surface,formed by bundles of axons lined by small oligodendrocyte-like cells.Between these columns,neurons appear to float in mucoid matrix.Four of the five DNT were simple and one was complex.Immunohistochemistry:oligodendrocyte-like cells positive for olig-2,neuronal positive for neun and syn,Scattered,interspersed stellate astrocytes positive for GFAP.The index of Ki-67 was very low(about 1%).Conclusions After complete surgical resection,most of DNT can be cured,and 5 cases reported in this article have not recurred.