胚胎发育不良性神经上皮瘤的临床病理及影像学特征

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例尢任何症状,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。

Clinicopathologic and radiologic features of dysembryoplastic neuroepithelial tumors

Objective To study the clinicopathologic features and radiologic findings of dysembryoplastic neuroepithelial tumor (DNT). Methods The clinical presentations, radiologic findings, histologic features and immunophenotype of 9 cases of DNT were analyzed. Results The age of patients ranged from 12 to 51 years (mean age=32 years). Most presented with partial seizures, sometimes accompanied by transient aphasia, agraphia and decreased visual acuity. One case was asymptomatic and discovered incidentally during a routine check-up. All patients had no neurological deficit found on physical examination. All tumors were located in the supratentorial cerebral cortex. There was no peritumoral edema or space-occupying effect on radiologic examination. The tumors involved either frontal lobe (number=4), temporal lobe (number=4), frontoparietal lobe (number=1) . Two cases showed cystic changes. Two histologic variants of DNT were recognized: simple (number=3) and complex (number=6). Simple variant was composed mainly of the glioneuronal element, accompanied by surrounding oligodendrocyte-like cells, and the complex variant contained a low-grade glioma component, in addition to the glioneuronal element and sometimes foci of cortical dysplasia. Conclusions DNT is a benign tumor with excellent prognosis after surgical excision. Local recurrence is rare. Complex variant of DNT needs to be distinguished from other types of low-grade glioma.