Objective To explore the clinicopathological features of dysembryoplastic neuroepithelial tumor ( DNT). Methods The clinical data of one DNT patient was analyzed retrospectively. Results This case was young male. The frist symptoms were episodes of limb spasm and conscious disturbance. MRI showed the lobus temporalis with low signal on T1WI and high signal on T2WI without enhancement and mass effect. The lesions consisted of multinodular specific glioneuronal elements and microcystic finely background mucinous matrix. The tumor composed of neurons, oligodendrocyte-like cells and ast rocytes, and accompanied with cortical dysplasia. Immunohistochemical showed neuronal nuclear antigen, synaptophysin and neuron specific enolase in the neurons and some oligodendrocyte-like cells (OLC) were positivity. The S-100 protein in OLC was positive. The staining of glial fibrillary acidic protein in the astrocytes was positive. The positive rate of Ki-67 nuclear was 1 % in neoplastic cell. Conclusions DNT is mainly happened in children and the youth. A part of the patients have chronic epilepsy history. The histological characters of DNT are the neuroglia and neuronal element.%目的 探讨胚胎发育不良性神经上皮瘤(DNT)的临床病理特征.方法 回顾性分析1例DNT患者的临床资料.结果 本例为青年男性,首发症状为发作性肢体抽搐伴意识障碍.MRI显示颞叶T1WI低信号,T2WI高信号,无水肿及占位效应.病变由多结节特异性胶质神经元和微囊细纤维黏液性基质等成分组成,肿瘤细胞主要有神经元、少突胶质样细胞(OLC)和星形细胞3种成分,伴有皮质发育不良.免疫组织化学结果为神经元及部分OLC呈神经核抗原、神经突触素、神经元特异性烯醇化酶阳性表达；OLC呈S-100蛋白阳性表达；星形细胞呈胶质纤维酸性蛋白阳性表达；瘤细胞Ki-67阳性率为1％.结论 DNT好发于儿童和年轻人,部分患者有慢性癫痫病史.DNT的组织学特征是神经胶质及神经元成分.
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