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目的探讨胚胎发育不良性神经上皮瘤(DNT)的临床病理特点及鉴别诊断。方法对9例DNT的临床表现、影像学特点、病理形态特征进行观察,并进行了免疫组织化学SP法检测。结果患者年龄范围12～51岁,平均32岁,大部分病例以癫痫小发作为主要临床表现,个别伴有一过性失语、失写、视力下降等,1例无任何症状,仅在体检中发现。神经系统检查无运动和感觉缺损体征。病变部位均位于幕上结构,以皮层为主,影像学检查无瘤周水肿及占位效应。额叶4例,颞叶4例,额顶叶1例,2例呈囊性。病理组织学DNT分为两型:单纯型3例,复杂型6例。单纯型DNT肿瘤仅由神经胶质神经元成分构成,周围有少突胶质细胞样细胞。复杂型DNT除了神经胶质神经元成分和(或)局灶性皮层发育不良外,还具有其他低级别胶质瘤成分。结论DNT是一种良性肿瘤,手术效果良好,不易复发,复杂型DNT需与各种低级别胶质瘤相鉴别。
Objective To investigate the clinicopathological features and differential diagnosis of embryonic dysplastic neuroepithelioma (DNT). Methods The clinical manifestations, imaging features and pathomorphologic features of 9 cases of DNT were observed and detected by immunohistochemical SP method. Results The patients ranged in age from 12 to 51 years with an average of 32 years old. Most of the cases were mainly epileptic seizures. One case had transient aphasia, loss of literacy and decreased visual acuity. One case had no symptoms and was found only in physical examination . Nervous system examination without signs of motor and sensory impairment. Lesions are located in the supratentorial structure, the main cortex, imaging examination without tumor edema and placeholder effect. 4 cases of frontal lobe, 4 cases of temporal lobe, 1 case of frontal parietal lobe, 2 cases of cystic. Pathological DNT divided into two types: simple type in 3 cases, 6 cases of complex type. Simple DNT tumors consist only of glial neuronal components, surrounded by oligodendrocyte-like cells. In addition to glial neuronal components and / or focal cortical dysplasia, complex DNT has other low-grade gliomas. Conclusion DNT is a benign tumor. The operation effect is good and it is difficult to relapse. DNT complicated with various low grade gliomas should be differentiated.