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

作　　者：夏轶 郑舒静 熊佶 杜尊国 胡小木 胡星 张莹 XIA Yi;ZHENG Shujing;XIONG Ji;DU Zunguo;HU Xiaomu;HU Xing;ZHANG Ying(Department of Pathology,Huashan Hospital,Fudan University,Shanghai 200040,China;Department of Pathology,Fujian Province,Zhangzhou Hospital,Zhangzhou 363000,China;Department of Ultrasound,Huashan Hospital,Fudan University,Shanghai 200040,China)

机构地区：[1]复旦大学附属华山医院病理科,上海200040 [2]福建省漳州市医院病理科,漳州363000 [3]复旦大学附属华山医院超声科,上海200040

出　　处：《临床与实验病理学杂志》2024年第5期476-479,共4页Chinese Journal of Clinical and Experimental Pathology

基　　金：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)。

摘　　要：目的探讨胚胎发育不良性神经上皮肿瘤(dysembryoplastic neuroepithelial tumor,DNT)的临床病理特征及预后。方法收集33例DNT患者的临床资料,采用免疫组化EnVision两步法检测Olig2、NeuN、Syn、GFAP、IDH1、CD34、BRAF V600E、H3K27M和Ki67的表达,观察其病理组织学形态及免疫组化特征进行回顾性分析,并复习相关文献。结果33例中可随访28例,其中女性10例,男性18例;发病年龄4~57岁,平均24.97岁;临床以难治性癫痫为常见症状。DNT主要发生在幕上区域,以皮层为主。肿瘤大多界限清楚,呈分叶状、囊性或囊实性结构。镜检:DNT主要由少突胶质样细胞、漂浮在黏液基质中的神经元和增生星形胶质细胞组成,偶见钙化,未见坏死或微血管增生。免疫表型:神经元表达NeuN、Syn,少突胶质样细胞表达Olig2,增生的星形胶质细胞表达GFAP,p53多为野生型,Ki67增殖指数较低(多数<4%)。结论DNT是一种良性混合性胶质神经元肿瘤,病理诊断需结合影像学特点、镜下形态、免疫组化结果及必要的分子检测,手术完整切除后患者预后良好。Purpose To investigate the clinical,imaging,pathological features,differential diagnosis and prognosis of embryonic dysplastic neuroepithelial tumor(DNT).Methods The clinical data of 33 DNT patients were collected.Immunohistochemical 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 followed 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,lobulated,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 observed.Immunohistochemistry showed that neurons expressed NeuN and Syn,oligodendrocytes expressed Olig2,and proliferative 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 neuronal structures.Precise pathological diagnosis needs to be carefully considered with imaging characteristics,microscopic morphology,immunohistochemistry and molecular test results if necessary.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 

分 类 号：R739.41[医药卫生—肿瘤]