胚胎发育不良性神经上皮肿瘤附6例病例分析

Dysembryoplastic neuroepithelial tumor:analysis of 6 cases

目的探讨胚胎发育不良性神经上皮肿瘤(DNT)的临床、病理学及影像特征,以及影像诊断和鉴别诊断。方法回顾性分析6例胚胎发育不良性神经上皮肿瘤患者的临床和影像学资料,并获得6例的随访资料;男性4例,女性2例;年龄为7～38岁,平均年龄18岁,大部分以癫痫发作为主要临床表现,大部分肿瘤均位于幕上,少部分肿瘤位于幕下,以皮层为主;肿瘤细胞主要由少突胶质样细胞(OLC)、星形细胞和神经元组成,1例合并有皮质发育不良及钙化。结果胚胎发育不良性神经上皮肿瘤MRI检查的共性特征为T1低信号,T2高信号,边界清楚,影像学检查均无明显的占位效应及瘤周水肿,少部分有钙化。结论胚胎发育不良性神经上皮肿瘤是一种中枢神经系统良性神经元肿瘤,影像及病理正确诊断具有现实意义,肿瘤切除预后良好,无需放疗和化疗。

Objective To investigate the clinical, pathological, radiological characteristics, as well as diagnosis and differential diagnosis of dysembryoplastie neuroepithelial tumor （DNT）. Methods The clinical and radiological data of 6 cases of DNT were retrospectively analyzed and 6 patients were followed up. The age of patients ranged from 7 to 38 years old （mean age= 18 years old）, including 4 males and 2 females. Seizures were the main clinical manifestations of most ca- ses. Most of the tumors were located in supratentorial. And a few tumors located in subtentorial, In most cases, lesions occurred in the cortex mainly. The tumor was composed of oligodendrocyte like cells, mature astrocytes and neurons. And 1 case presented with cortex hypogenesis. Results The common features of MRI examination of DNT showed T1 low signal, T2 high signal and the boundary were dear. There was no peritumoral edema or space occupying effect on radiological examination. A few had calcification. Conclusion DNT is a benign tumor （WHO I ）. The correct radiological and pathological diagnosis has great value. The prognosis of patients after resection of the tumor is good. Radiotherapy and chemotherapy are not necessary.