胚胎发育不良性神经上皮瘤的临床、病理与影像诊断进展

胚胎发育不良性神经上皮瘤是一类少见的中枢神经系统肿瘤,好发于儿童幕上皮层和皮层下区域,临床以部分复杂性癫痫发作为主要症状,神经系统体征阴性,肿瘤以囊性成分为主,瘤周无水肿,无占位效应,"倒三角征"和瘤内分隔为其影像学表现特征。在组织病理诊断困难的情况下,结合临床和影像学表现可以对胚胎发育不良性神经上皮瘤做出正确的诊断。肿瘤术后效果良好,不易复发。本病的准确诊断对治疗方案的选择有重要意义。

Clinical, Pathological and Imaging Diagnosis Progresses of Dysembryoplaslic Neuroepithelial Tumor

Dysembryoplastic neuroepithelial tumor was a rare tumor of central nervous system. It often occur in supratentorial region and cerebral cortex in the enfant. Most of patients had partial seizures, but the neurological deficits were absent. The tumor were a predominant cystic component, absence of peri-tumoral edema and space-occupying effect, intra-tumoral＂ septations＂ and reveased triangle in shape were the characteristic neuro-radiological features. Radiologic examination may be helpful to the diagnosis of dysembryoplastic neurcepithelial tumor when pathological findings are inconclusive. There was excellent prognosis after surgical excision,local recurrence was rare. The identification of the tumor has the therapeutic and prognostic implication.