胚胎发育不良性神经上皮肿瘤12例临床病理分析  

Dysembryoplastic neuroepithelial tumor:a clinicopathologic analysis of 12 cases

作　　者：齐雪岭[1] 罗麟 姚坤[1] 陈引[1] 

机构地区：[1]北京三博脑科医院病理科,北京100093 [2]北京天坛神经外科研究所病理室,北京100050

出　　处：《诊断病理学杂志》2011年第6期424-427,共4页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨胚胎发育不良性神经上皮肿瘤(DNT)的临床病理特点、影像学特征、治疗及预后。方法应用光镜及免疫组化染色对12例DNT进行病理观察及分析。结果临床症状主要表现为不同形式的癫发作,神经系统检查无明显阳性体征。MRI病变呈囊实性改变,以囊性为主,病灶多呈倒三角形、扇形或椭圆形和不规则形。镜下具有神经元形态特点的大细胞散在分布于有一定排列特点的少枝样胶质细胞及星形细胞之间,或悬浮于微囊黏液湖中。免疫组化示似神经元的大细胞神经核抗原(NeuN)(+),密集分布的小细胞Syn(+),少突胶质细胞特异性抗体Olig-2(+),散在分布的星形细胞胶质纤维酸性蛋白(GFAP)和vimentin(+),增殖细胞核抗原Ki-67活性极低。术后随访2个月至3年,无复发。结论 DNT属于良性肿瘤(WHOⅠ级),手术切除完整则预后良好。病理诊断须结合影像学特点及免疫组化结果进行综合分析,需与分化良好的星形细胞瘤囊性变相鉴别。Objective To investigate the clinical and pathological features,imaging characteristics,treatment and prognosis of dysembryoplastic neuroepithelial tumor（DNT）.Methods Light microscopy and immunohistochemistry were used to conduct the pathological observation and analysis in 12 cases of DNT.Results The clinical symptoms were mainly different forms of epilepsy,and had no other sign of nervous system.On MRI,the lesions showed predominantly cystic-solid appearance,mainly cystic lesions.Lesions were inverted triangles,fan-shaped or oval,irregular shape,with the angle of the lesions,and advanced multi-point to the ventricles,widebase sitting on the cortex,and showed no peritumoral edema effect and tumor boundary were clear.Microscopically,histological structure was the large cells which had morphological characteristics of neurons scattered between the oligodendrocytes and astrocytes,or suspended mucoid matrix of the microcysts.Immunohistochemistry showed that neuron-like big cells expressed NeuN,densely distributed small cells expressed Syn and Olig-2.The expression of GFAP and vimentin was positive in scattered astrocytes.The expression of Ki-67 was very low.We followed up for 2 months-3 years,with no recurrence,and all patients still were under follow-up.Conclusions DNT is a benign tumor（WHO Grade Ⅰ）,with excellent prognosis after complete surgical resection.Pathological diagnosis needs to be combined with imaging features and immunohistochemical results,to avoid confusing with well-differentiated cystic astrocytoma.

关 键 词：胚胎发育不良性神经上皮肿瘤 临床病理 免疫组化 影像学 

分 类 号：R739.4[医药卫生—肿瘤]