临床与实验病理学杂志2024，Vol.40Issue(5)：476-479,4.DOI:10.13315/j.cnki.cjcep.2024.05.006
胚胎发育不良性神经上皮肿瘤33例临床病理分析
Neuroepithelial tumors with embryogenesis dysplasia:a clinicopathological analysis of 33 cases
摘要
Abstract
Purpose To investigate the clinical,imaging,pathological features,differential diagnosis and prognosis of em-bryonic dysplastic neuroepithelial tumor(DNT).Methods The clinical data of 33 DNT patients were collected.Immunohis-tochemical results of Olig2,NeuN,Syn,GFAP,IDH1,CD34,BRAF V600E,H3K27M and Ki67 were examined by EnVision method.Histopathological morphology and immunohistochemical features were retrospectively analyzed and relevant literature was reviewed synchronously.Results A total of 28 cases were fol-lowed up,including 10 females and 18 males.The age of onset was 4～57 years,with average 24.97 years.Refractory epilepsy was a common symptom.The lesions were mainly located in the supratentorial cortex,and most of them were well-defined,lobu-lated,cystic or cystic and solid.Microscopically,DNT was mainly composed of oligodendrocytes,neurons floating in the mucous matrix,and proliferative astrocytes.Calcification was rare,and no necrosis or microvascular hyperplasia was ob-served.Immunohistochemistry showed that neurons expressed NeuN and Syn,oligodendrocytes expressed Olig2,and prolifera-tive astrocytes expressed GFAP;p53 was mostly wild-type,and the proliferation index of Ki67 was low(mostly less than 4％).Conclusion DNT is a benign tumor with mixed glial and neu-ronal structures.Precise pathological diagnosis needs to be care-fully considered with imaging characteristics,microscopic mor-phology,immunohistochemistry and molecular test results if nec-essary.Prognosis after complete surgical resection is good.关键词
中枢神经系统肿瘤/胚胎发育不良性神经上皮肿瘤/鉴别诊断/预后Key words
central nervous system tumors/embryonic dysplas-tic neuroepithelial tumors/differential diagnosis/prognosis分类
医药卫生引用本文复制引用
夏轶,郑舒静,熊佶,杜尊国,胡小木,胡星,张莹..胚胎发育不良性神经上皮肿瘤33例临床病理分析[J].临床与实验病理学杂志,2024,40(5):476-479,4.基金项目
国家自然科学基金(82072692)、复旦大学附属华山医院院内科研启动基金(HSBY2020002) （82072692）
