胚胎发育不良性神经上皮肿瘤13例临床病理分析  

Dysembryoplastic neuroepithelial tumour:clinical and pathological analysis of thirteen cases

作　　者：张琰[1] 巩楠 苗娜[1] 王志强 ZHANG Yan;GONG Nan;MIAO Na;WANG Zhi-qiang(The First Affiliated Hospital of Xinjiang Medical University,Wulumuqi 830054,China;不详)

机构地区：[1]新疆医科大学第一附属医院病理科,乌鲁木齐830054 [2]新疆自治区第三人民医院病理科,乌鲁木齐830054

出　　处：《诊断病理学杂志》2020年第2期101-103,106,共4页Chinese Journal of Diagnostic Pathology

基　　金：新疆维吾尔自治区自然科学基金(2019D01C287)。

摘　　要：目的探讨胚胎发育不良性神经上皮肿瘤(Dysembryoplastic neuroepithelial tumour,DNT)临床病理特点、诊断及与低级别胶质细胞瘤鉴别诊断。方法收集13例DNT,分析其临床特点,观察其病理组织学形态及免疫表型特征进行回顾性分析,并复习相关文献。结果DNT典型的临床表现为难治性癫,男性多于女性,平均年龄19.7岁。病变位于幕上,多局限于皮层或以皮层为主,影像学上呈长T1长T2异常信号,13例均行外科手术切除,术后癫症状消失。病理组织学DNT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,9例伴有皮质发育不良。免疫组织化学结果为神经元成分NeuN、NSE、NF、Syn(+),少突胶质细胞样成分S-100(+),NeuN、NSE部分(+),Ki-67<3%。结论胚胎发育不良性神经上皮肿瘤是一种少见的中枢神经系统低级别神经上皮肿瘤,其诊断主要依据病理形态学特征结合影像学表现,多发生于青少年,以颞叶、额叶居多,预后良好。Objective To investigate the clinicopathologic features,diagnosis and differential diagnosis of dysembryoplastic neuroepithelial tumour(DNT).Methods Thirteen cases of DNT were collected,and their clinical characteristics were analyzed.The histopathological morphology and immunophenotypic characteristics were analyzed retrospectively and the related literatures were reviewed.Results The typical clinical manifestation of DNT was refractory epilepsy,with more males than females,with an average age of 19.7 years.The lesions were mostly located in the temporal lobe and frontal lobe,and the lesions were mostly confined to the cortex or the cortex,and the imaging showed a long T1 and long T2 abnormal signal,13 patients underwent surgical resection,and the postoperative epileptic symptoms disappeared.The histopathologiy of DNT was mainly composed of oligodendrocytes(OLC),neurons and astrocytes,and 9 cases were with cortical dysplasia.The immunohistochemical results were positive for NeuN,NSE,NF,Syn and oligoglial samples,and NeuN and NSE were positive;ki-67 index was<3%.Conclusion Dysembryoplastic neuroepithelial tumour is benign neuroepithelial tumor that is a rare low level neuroepithelial tumors of the central nervous system;its diagnosis is mainly based on the pathological morphological features in combination with imaging findings;it develops in adolescents,most commonly in temporal lobe and frontal lobe;the prognosis is good.

关 键 词：胚胎发育不良性神经上皮肿瘤 鉴别诊断 预后 

分 类 号：R739.43[医药卫生—肿瘤]