摘要
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 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.
出处
《中华病理学杂志》
CAS
CSCD
北大核心
2005年第9期566-568,共3页
Chinese Journal of Pathology
