中线部位和脑室节细胞胶质瘤临床病理分析及文献复习  

Midline structures ganglioglioma and ventricle ganglioglioma:a clinicopathologic study and review of literature

作　　者：田梦雨 王雷明[1] 张萌 熊艳蕾 高敏 滕梁红[1] TIAN Meng-yu;WANG Lei-ming;ZHANG Meng;XIONG Yan-lei;GAO Min;TENG Liang-hong(Department of Pathology,Beijing Xuanwu Hospital,Capital Medical University,Beijing 100053,China)

机构地区：[1]首都医科大学宣武医院病理科,北京100053

出　　处：《诊断病理学杂志》2024年第3期190-194,共5页Chinese Journal of Diagnostic Pathology

基　　金：北京市医院管理中心临床医学发展专项经费(ZYLX202113);首都卫生发展科研专项项目(首发2020-2-2016);北京市临床重点专科项目。

摘　　要：目的探讨中线部位和脑室节细胞胶质瘤的临床病理及分子遗传学特点。方法收集11例中线部位和脑室节细胞胶质瘤患者的临床特征、免疫表型及分子检测结果,并复习总结相关文献。结果镜下观察节细胞胶质瘤由异常神经元和肿瘤性增生的胶质细胞构成,免疫表型NeuN、MAP2可标记肿瘤中的神经元成分,GFAP和Olig-2标记出了肿瘤性神经胶质细胞成分;分子遗传学常存在BRAFV600E点突变,但也可发现存在其他形式BRAF基因以及MAPK信号通路基因的改变。随访病例多数预后良好。结论发生在中线部位及脑室的节细胞胶质瘤较为罕见,对于发生于不典型部位的这种低级别神经上皮肿瘤,要将其纳入鉴别诊断中,可在形态学基础上,结合免疫组织化学以及分子检测结果进行综合分析。Objective To investigate the clinicopathological characteristics,molecular features of midline structures ganglioglioma and ventricle ganglioglioma.Methods The data of clinical features,immunophenotype and molecular biological tests result were collected.Results Microscopically,gangliogliomas were composed of abnormal neurons and glial cells with neoplastic hyperplasia.Immunohistochemically,the tumor cells showed positivity with GFAP,Olig-2,NeuN,MAP2.Most common mutation were BRAFV600E mutation.In gangliogliomas lacking BRAFV600E mutations,other oncogenic mutations in the BRAF gene were often present.Most patients had excellent prognosis.Conclusion Gangliogliomas that occur in midline structures or ventricle are rare.When you find a low-grade neuroepithelial tumors that occur in atypical sites,the diagnosis of ganglioglioma should be taken in consideration.The precise diagnosis shouId be confirmed by morphology,immunohistochemistry and necessarily molecular biological tests.

关 键 词：节细胞胶质瘤 病理学特征 脑干 脊髓 脑室 

分 类 号：R365[医药卫生—病理学]