血管中心性胶质瘤临床病理学特征及相关文献复习  

Clinicopathological characteristics of angiocentric glioma and review of the literature

作　　者：连芳[1] 刘丽娜 赵莉红[1] 隗立峰[1] 卢德宏[1] 朴月善[1] LIAN Fang;LIU Li-na;ZHAO Li-hong;WEI Li-feng;LU De-hong;PIAO Yue-shan(Department of Neuropathology,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

出　　处：《临床与实验病理学杂志》2020年第1期24-27,共4页Chinese Journal of Clinical and Experimental Pathology

摘　　要：目的探讨血管中心性胶质瘤(angiocentric glioma,AG)的临床病理学特征、诊断及鉴别诊断。方法收集首都医科大学宣武医院病理科诊断的12例AG,回顾性分析患者的临床资料、病理学特征及预后,并复习相关文献。结果12例患者均有难治性癫痫病史,平均发病年龄9岁(2.8~24岁)。头颅核磁共振(MRI)表现为T1WI低信号,T2WI/FLAIR高信号,未见明显强化。组织学特点为均一的双极细胞,沿血管轴排列形成血管周围假菊形团。AG中GFAP、vimentin均阳性,肿瘤细胞中EMA、D2-40呈胞质弥漫阳性或核旁点灶阳性,EMA和D2-40的阳性率分别为91.7%(11/12)和100%(12/12);NeuN、IDH1 R132H、L1CAM、BRAF V600E和H3K27M均阴性。Ki-67的增殖指数为1%~5%。术后影像学检查示患者肿瘤均完整切除。随访14~110个月,患者均无癫痫发作及肿瘤复发。结论AG是以癫痫发病、生长缓慢的肿瘤,手术切除效果良好。Purpose To explicit the clinicopathological characteristics,diagnosis and differential diagnosis of angiocentric glioma.Methods 12 cases were collected from the department of pathology,Xuanwu Hospital of Capital Medical University,and the clinical data,pathological features and prognosis of the patients were analyzed retrospectively and review relevant literature.Results All 12 patients had a history of intractable epilepsy,and the mean history of pre-surgical epilepsy was 9 years(2.8-24 years).Magnetic resonance imaging(MRI)was showed with hypointense on T1-weighted images(T1WI),hyperintensity on T2-weighted images(T2WI)and bright on fluid-attenuated inversion recovery images(FLAIR).The histological features were bipolaRspindle cells arranged along the vasculaRaxis to form perivasculaRpseudo chrysanthemum clusters.The immunohistochemical results showed that all 12 cases were positive foRGFAP and vimentin,and negative foRNeuN,IDH1,R132H,L1CAM,BRAF V600E and H3K27M.The positive rate of EMA and D2-40 were 91.7%(11/12)and 100%(12/12),and the rate of Ki-67 proliferation was 1%-5%.Post-operation MRI showed that the tumoRof all 12 patients were totally resected.And then followed up foR14-110 months and none had seizure attack oRtumoRrecurrence afteRsurgery.Conclusion Angiocentric glioma is an epilepsy-associated,stable oRslow-growing tumor,and surgery resection is often curable.

关 键 词：血管中心性胶质瘤 临床病理学特征 免疫组织化学 EMA 室管膜分化 

分 类 号：R739.41[医药卫生—肿瘤]