原发于中枢神经系统的孤立性纤维性肿瘤/血管外皮瘤60例临床病理学分析  

Solitary fibrous tumor/hemangiopericytoma of central nervous system: a clinicopathological analysis of 60 cases

作　　者：管瑜 王璇 吴楠 陆珍凤 石群立 吴晋蓉 Guan Yu;Wang Xuan;Wu Nan;Lu Zhenfeng;Shi Qunli;Wu Jinrong(Department of Pathology,Nanjing Jinling Hospital,Nanjing 210002,China;Department of Pathology,Xuzhou Mining Hospital,Jiangsu Province,Xuzhou 221006,China)

机构地区：[1]解放军东部战区总医院病理科,南京210002 [2]江苏省徐州市矿山医院病理科,221006

出　　处：《中华病理学杂志》2019年第1期31-36,共6页Chinese Journal of Pathology

摘　　要：目的探讨原发于中枢神经系统的孤立性纤维性肿瘤/血管外皮瘤的临床病理特点、影像学特征以及诊断和鉴别诊断,以提高对此类肿瘤的认识。方法回顾性分析2008年1月1日至2016年12月31日解放军东部战区总医院收治的60例原发于中枢神经系统的孤立性纤维性肿瘤/血管外皮瘤的临床及影像学资料、组织学形态以及免疫组织化学结果,复习相关文献并随访。结果60例病例中男性26例,女性34例,年龄14~85岁,中位年龄49岁。临床表现主要为头痛、头晕伴恶心呕吐。影像学均表现为与脑膜关系密切的占位性病变,部分可伴随坏死及脑膜"鼠尾征"。组织学表现为富含大量薄壁裂隙状血管的梭形细胞肿瘤,根据肿瘤级别不同呈现不同的细胞形态及坏死等组织学特征。100.0%(60/60)病例表达波形蛋白;98.3%(59/60)病例表达STAT6,其中1例为局部阳性表达;61.7%(37/60)病例弥漫表达CD34;Ki-67阳性指数在1%~50%;不同程度见CD99、bcl-2、上皮细胞膜抗原(EMA)和SSTR2表达。均不表达S-100蛋白、SOX10、E-cadherin、胶质纤维酸性蛋白(GFAP)。40例获得随访资料,随访时间为术后6~82个月,平均40个月,存活30例,死亡10例。结论原发于中枢神经系统的孤立性纤维性肿瘤/血管外皮瘤发病率低,术前准确诊断有一定困难。STAT6对诊断本病有高灵敏度和特异度。治疗多为手术切除并联合放化疗。预后与发病部位、组织学分级、Ki-67阳性指数、是否全切以及融合基因变异体存在相关性。Objective To investigate the clinical manifestations,imaging features,clinicopathologic features,and differential diagnosis of solitary fibrous tumors/anginoblastomas(SFT/HPCs)originating in the central nervous system.Methods Sixty cases of SFT/HPCs originating in the central nervous system were collected at Nanjing Jinling Hospital,from January 1,2008 to December 31,2016.The clinical data,imaging data,histomorphologic changes and immunohistochemical finding were analyzed in the sixty cases.Results The 60 cases included 26 males and 34 females,aged 14 to 85(median 49)years.The main clinical manifestations were headache,dizziness with nausea and vomiting.Radiologically,the tumors were large,enhancing,solid and cystic masses attached to the dura.Histopathologically,the neoplasms were composed of spindle cells with oval nuclei,inconspicuous nucleoli and moderate amount of eosinophilic cytoplasm arranged in fascicles with areas of hyalinized stroma,myxoid changes and a staghorn vascular pattern.Immunohistochemically,tumor cells of all cases were positive for vimentin(100.0%,60/60),STAT6(98.3%,59/60),CD34(61.7%,37/60),and the tumor cells were typically positive for CD99,bcl-2,EMA and SSTR2 as well.Negative for S-100 protein,SOX10,E-cadherin,GFAP.Ki-67 index ranged from 1% to 50%.Forty cases were followed up for 6 to 82 months with average of 40 months,30 patients were alive and 10 patients died.Conclusions Central nervous system SFT/HPCs can be aggressive and relapses may occur several years after diagnosis.STAT6 is highly sensitive and specific for the diagnosis.Complete tumor resection is optional treatment followed by radiotherapy and chemotherapy.There is a correlation between the prognosis and the location of the disease,the histological grade,Ki-67 index,and fusion gene variants.

关 键 词：中枢神经系统肿瘤 血管周上皮样细胞瘤 STAT6转录因子 预后 孤立性纤维性肿瘤 

分 类 号：R739.4[医药卫生—肿瘤]