胚胎发育不良性神经上皮肿瘤4例临床病理观察

Dysembryoplastic neuroepithelial tumor: a clinicopathological analysis of four cases

目的探讨胚胎发育不良性神经上皮肿瘤(DNT/DNET)的临床病理特征、免疫表型及诊断要点。方法收集4例胚胎发育不良性神经上皮肿瘤患者的临床资料,结合相关文献复习其组织病理学特征及免疫组化特点。结果 4例患者均以耐药性难治性癫■发作就诊。组织学表现均可见特征性的'胶质神经元成分',即形态一致的少突胶质样细胞呈圆柱状、缎带样排列,形成微囊样结构;微囊内含嗜碱性黏液样基质,较大的神经元漂浮在黏液样基质中。免疫组化:较小的少突胶质样细胞S-100、Olig-2(+),漂浮的神经元Syn、MAP-2、NeuN(+),周围增生的星形胶质细胞GFAP(+),肿瘤细胞ATRX(+),IDH1(-),Ki-67增殖指数极低(约1%)。结论胚胎发育不良性神经上皮肿瘤是一种少见的良性混合性神经元-胶质肿瘤,具有较为独特的病理形态学特征及免疫表型,手术完整切除,绝大部分预后良好。本文报道的4例患者均行手术切除治疗,术后随访至今,无复发。

Objective To investigate the clinicopathological features,immunohistochemical phenotype and diagnostic points of dysembryoplastic neuroepithelial tumor(DNT/DNET).Methods The clinical characteristics were collected in 4 cases of DNT,the phathological features and immunohistochemical phenotype were retrospectively analyzed.Results The 4 patients were all treated for refractory epilepsy.Histologically,they all exhibited a specific'glioneuronal element'.The morphologically consistent oligodendrocyte-like cells arranged in columns and ribbon-like,forming a microcystic structure with'floating neurons'in basophilic mucoid matrix.Immunohistochemistry showed that oligodendrocyte-like cells expressed S-100 and Olig-2,floating neuron cells expressed Syn,MAP-2,and NeuN.The expression of GFAP was positive in scattered astrocytes.ATRX was positive while IDH1 was negative.The index of Ki-67 was very low(about 1%).Conclusions DNT is a rare and benign mixed neuronal-glial tumor with specific histophathological features and immunohistochemical phenotype.Most of the prognosis is excellent after complete surgical resection.All patients are under follow-up with no recurrence.