神经节细胞胶质瘤19例临床病理分析  

Ganglioglioma: a clinicopathological study of 19 cases

作　　者：梁乐[1] 付静[1] 李大胜[2] 冷慧[1] 刘永玲[1] 姚晓香[1] 歌日乐 李云林[3] 马康平[3] LIANG Le1 , FU Jing1 , LI Da-sheng2 , LENG Hui1 , LIU Yong-ling1 , YAO Xiao-xiang1 , GE Ri-le1 , LI Yun-lin3 , MA Kang-ping3(1 Department of Pathology, 2 Department of Radiology, Belling Haidian Hospital/Haidian Section of Peking University Third Hospital, Beijing 100080, China ; 3Department of Neurosurgery, Children＇ s Hospital Affiliated to Capital Institute of Pediatrics, Belling 100020, Chin)

机构地区：[1]北京市海淀医院/北京大学第三医院海淀院区病理科,北京100080 [2]北京市海淀医院/北京大学第三医院海淀院区影像科,北京100080 [3]首都儿科研究所附属儿童医院功能神经科100020

出　　处：《临床与实验病理学杂志》2018年第3期273-278,共6页Chinese Journal of Clinical and Experimental Pathology

基　　金：北京市海淀医院/北京大学第三医院海淀院区青年科研项目(KYQ2017004)、首都儿科研究所科研基金(FX-2017-04)

摘　　要：目的探讨神经节细胞胶质瘤(ganglioglioma,GG)的临床病理特征。方法收集因难治性癫痫接受致痫病灶切除术并经病理诊断的GG 19例,回顾性分析其临床病理特征。结果患者平均发病年龄9.1岁,平均病程9.3年,头颅核磁共振检查可见异常信号表现,多位于颞叶(14/19,73.7%)。肿瘤表现出异质性,常伴有局灶性皮质发育不良(13/19,68.4%)。免疫组化标记18例CD34阳性、16例Nestin阳性,二者阳性表达率差异无显著性(P>0.05);6例BRAF-V600E阳性。结论 GG诊断应强调临床、影像和病理学特点的综合判断,需与其他肿瘤及皮质发育异常病变鉴别,CD34、Nestin免疫组化标记对诊断有重要帮助。Purpose To study the clinicopathologic fea- tures of ganglioglioma. Methods The clinicopathologic data of the cases pathologically diagnosed as ganglioglioma that underwent resection of epileptic focus were retrospectively analyzed. Results In the 19 cases studied, the mean onset age was 9. 1 years, and the duration of disease was 9. 3 years. MRI images showed abnormal signals. The majority of the site was temporal lobe （14/19, 73.7% ）. The tumors showed heterogeneity and often accompanied by focal cortical dysplasias （13/19, 68.4% ）. Immunohistoehemieal staining showed CD34 positive in 18 cases, Nestin positive in 16 eases, and BRAF-V600E pos- itive in 6 case. The positive expression rate of CD34 and Nestin did not have significant differences. Conclusion The diagnosis of ganglioglioma relies on pathological observations combined with clinical features and neuroradiological examinations. Differ- ential diagnosis should be done from other tumors or cortical dys- plasia. Immunohistochemical staining of CD34 and Nestin can help diagnosis.

关 键 词：脑肿瘤 神经节细胞胶质瘤 局灶性皮质发育不良 

分 类 号：R739.41[医药卫生—肿瘤]