胚胎发育不良性神经上皮肿瘤8例临床及病理分析  

Dysembryoplastic Neuroepithelial Tumor:A Clinicopathologic Analysis of 8 Cases

作　　者：袁玲玲[1,2] 许霞[1] 张尚福[1] 王世风[1] 陈仕高[1] 

机构地区：[1]四川大学华西医院病理科,四川成都610041 [2]湖北省郧阳医学院附属十堰市太和医院病理科,湖北郧阳442000

出　　处：《华西医学》2009年第9期2347-2351,共5页West China Medical Journal

摘　　要：目的：探讨胚胎发育不良性神经上皮肿瘤（DNT）的临床、影像及病理学特征、诊断及鉴别诊断。方法：回顾性分析8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,进行光镜和免疫组织化学染色观察,并获得6例的随访资料。结果：胚胎发育不良性神经上皮肿瘤男性7例,女性1例,年龄为5-19岁,平均年龄13岁,5例以癫痫小发作为主要临床表现,病变均位于幕上,以皮层为主,影像学检查均无明显的占位效应及瘤周水肿。肿瘤细胞主要由少突胶质样细胞（OLC）、神经元和星形细胞组成,4例伴有皮质发育不良。免疫组织化学结果为神经元及部分少突胶质样细胞呈嗜铬素A、突触素及S-100阳性表达;少突胶质样细胞呈胶质纤维酸性蛋白（GFAP）阴性表达,而星形细胞呈GFAP阳性表达;Ki-67抗原标记指数〈1%。结论：胚胎发育不良性神经上皮肿瘤为WHOⅠ级良性肿瘤,可结合临床、影像及病理学表现明确诊断,预后良好,无需放疗和化疗。Objective： To investigate the clinical, radiological, pathological characteristics, diagnosis and differential diagnosis of dysembryoplastic neuroepithelial tumor （DNT）. Methods： Clinical and radiological databases of 8 cases of DNT were retrospectively analyzed with light microscopic and immunohistochemical observation and 6 patients were followed up. Results： The age of patients ranged from 5 to 19 years （mean age = 13 years）, It is more common in men than in women obviously. Most presented with partial seizures in clinic. All tumors were located in the supratentorial cerebral cortex. There was no peritumoral edema or space-occupying effect on radiologic examina tion. The tumor composed of oligodendrocyte-like cells, mature neurons and astrocytes, In most cases, the foci of cortical dysplasia were found in adjacent areas. Immunohistochemical study demonstrated positivity for chromogranin A, synaptophysin, and S-100 protein in the neurons and some oligodendrocyte-like cells. The staining of glial fibrillary acidic protein in the oligodendrocyte-like cells was negative, but the astrocytes was positive. Ki 67 nuclear antigens were lower than 1 % in all cases. Conclusion： DNT is a benign tumor（WHO I ）. The correct diagnosis thorough understanding of the clinical, radiological, pathological features has great value, radiotherapy and chemotherapy are contraindicated.

关 键 词：胚胎发育不良性神经上皮肿瘤 临床 病理 诊断 

分 类 号：R739.41[医药卫生—肿瘤]