胚胎发育不良性神经上皮瘤的临床病理学特点

Clinicopathologic features of dysembryoplastic neuroepithelial tumor

目的探讨胚胎发育不良性神经上皮瘤(DNT)的临床病理特征及免疫组化特点。方法结合临床、影像、HE染色和免疫组化方法对6例DNT患者资料进行回顾性分析。结果 6例DNT患者均以癫痫为主要临床症状,病变均位于幕上结构(颞叶3例、左顶叶2例、右额叶1例)。CT检查显示病变均呈低密度改变,MRI检查显示病灶均呈长T1长T2信号,无病变周围水肿及占位效应,增强扫描后均未见明显强化。按DNT病理组织学分型:单纯型1例,复杂型5例。复杂型5例均伴有局灶性皮质发育不良。免疫组化染色结果显示,少突胶质样细胞Olig2、S100表达强阳性,少部分表达神经元核抗原(NeuN)、微管相关蛋白2(Map2)、胶质纤维酸性蛋白和nestin;神经元成分呈NeuN和Map2强阳性表达,CD34阳性表达仅见血管内皮细胞。结论 DNT是一种良性病变,需结合临床、影像、病理学特征进行诊断,免疫组化方法有助于确诊。

Objective To evaluate the clinicopathologic features and immunohistochemieal characteristics of dysembryoplastic neuroepithelial tumor （DNT）. Methods Clinical features, imaging （CT and MRI）, HE staining and immunohistochemical methods were used to analyse the data of 6 cases with DNT retrospectively. Results All of the 6 patients presented with drug：resistan＇t epileptic seizures. All lesions located in the supratentorial region and the cerebral cortex was involved, localization of lesions was in the temporal （n= 3）, left frontoparietal （n= 2） and right frontal lobes （n= 1）. CT scan showed moderately hypodense lesion. All lesions were hypointense on Tl-weighted images and hyperintense on T2 weighted images as well as devoid of peritumoral edema or mass effect. MRI signal intensity of cystic lesions was homogeneous on T2 weighted images which was equal to or slightly higher than that of cerebrospinal fluid. Pathohistologically, the DNT were classified into two subtypes： simple form （n= 1） , complex form （n=5） . Focal cortical dysplasia were found in the 5 complex form cases. Immunohistochemical staining showed that the oligodengroglial-like cells （OLC） expressed Olig2 and S100 strongly positive, a few of those OLC expressed NeuN, Map2, GFAP and nestin. Neurons were positive for NeuN and Map2. CD34 was found positive only in vascular endothelia. Conclusions DNT was a benign tumor （WHO I ）. The correct diagnosis required combination of the clinical, radiologieal and pathological features, immunohistoehemisty could be helpful.