126例中枢神经系统孤立性纤维性肿瘤/血管周细胞瘤临床病理特征及预后分析  

Clinicopathological characteristics and prognosis of central nervous system solitary fibrous tumor/hemangiopericytoma:an analysis of 126 cases

作　　者：徐婉妮 王映梅 李青 张丽英 XU Wan-ni;WANG Ying-mei;LI Qing;ZHAGN Li-ying(Department of Pathology,Xijing Hospital,Air Force Military Medical University,Xi'an 710032,China)

机构地区：[1]空军军医大学西京医院病理科,西安710032

出　　处：《诊断病理学杂志》2021年第10期801-806,共6页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨原发于中枢神经系统孤立性纤维性肿瘤/血管周细胞瘤(SFT/HPC)的临床病理特征及预后相关因素。方法收集西京医院病理科2006-10-13—2020-06-10诊断的126例原发于中枢神经系统的SFT/HPC临床、影像及病理学资料,分析临床病理特征、组织学形态、免疫表型特征,并获取随访资料进行预后分析。结果本组病例中,男性62例,女性64例,中位年龄44岁(范围2~71岁),平均年龄44.8岁,临床表现与发病部位有关,多表现为头痛头晕、恶性呕吐、肢体麻木等症状。MRI示:不规则或类圆形等或稍高或稍低信号影,边界较清,增强扫描明显强化,可见脑膜尾征,FLAIR呈稍高信号。根据WHO(2016)中枢神经系统分级标准,Ⅰ级9例,Ⅱ级60例,Ⅲ级57例,其中Ⅱ级和Ⅲ级的病例细胞密度增加,异型性明显,多出现显著的间质玻璃样及黏液样变性、水肿、肿瘤性坏死、出血等,常见颅骨和脑组织侵犯等组织学形态。免疫组化:STAT6(41/47,87.23%)表现为典型的核强(+),CD34(114/121,94.21%),19例为局灶(+),其余均为胞质强(+),CD99(75/86,87.21%)、Bcl-2(82/98,83.67%)、vimentin(70/75,93.33%)不同程度(+)。PR(19/56,33.93%)、EMA(18/112,16.07%)、S-100(4/87,4.60%)、SMA(6/52,11.54%)均为局灶(+),Ki-67增殖指数1%~90%,GFAP、desmin均(-)。94例获得随访信息,随访时间为术后0.5~228个月,其中39例无病生存;45例患者术后复发,其中6例因病去世;10例出现转移,同时8例伴复发,其中2例因病去世。结论SFT/HPC为中枢神经系统较少见的肿瘤,多与脑膜关系密切,组织学形态多样,免疫标记STAT6为其诊断特异而敏感的指标。该肿瘤的临床预后除了与WHO的三级分级系统相关,同时组织形态出现显著的间质玻璃样及黏液样变性、水肿、肿瘤性坏死、出血以及侵犯颅骨和脑组织的病例,更易出现复发及转移。因此,对于中枢神经系统孤立性纤维性肿瘤/血管周细胞瘤的诊断,有必要将该形态学Objective To explore the clinicopathological characteristics and prognostic factors of solitary fibrous tumor/hemangiopericytoma(SFT/HPC)of the central nervous system(CNS).Methods A total of 126 cases of CNS SFT/HPC were collected on the clinical,imaging and pathological data,histological morphology and immunophenotype,follow-up information and prognostic analysis at the Department of Pathology of Xijing Hospital,from October 13,2006 to June 10,2020.Results Among the cases in this group,there were 64 women and 62 men,with age at presentation ranging from 2 to 71 years(average,44.8 years).The clinical manifestations were related to the site of the disease,with symptoms such as headache,dizziness,nausea and vomiting,and numbness of the limbs.MRI showed irregular or round-like or slightly higher or lower signal shadow,with clear boundary,enhanced scan,obvious meningeal tail sign.FLAIR showed slightly higher signal.According to the 2016 World Health Organization(WHO)classification of the CNS tumors,there were 9 cases of grade I,60 cases of grade II,and 57 cases of grade III.Among them,the cell density of grade II and III cases had increased,and some cases showed some malignant alterations,including obvious atypia,significant interstitial hyaline and mucoid degeneration,edema,tumor necrosis,and histological morphology such as invasion of skull and brain tissue.Immunohistochemical staining showed that STAT6(41/47,87.23%)was typically nuclear positive.CD34(114/121,94.21%)was cytoplasmic positive and focally positive in 19 cases.CD99(75/86,87.21%),Bcl-2(82/98,83.67%)and vimentin(70/75,93.33%)were positive to varying degrees.PR(19/56,33.93%),EMA(18/112,16.07%),S-100(4/87,4.60%),and SMA(6/52,11.54%)were focally positive.Ki-67 index was 1%~90%.The tumor was negative for GFAP and Desmin.Follow-up information was available for 94 patients,ranging from 0.5 to 228 months:39 cases were alive,45 cases relapsed after surgery,6 cases died of illness,10 cases developed metastasis;8 cases had recurrence with 2 cases died.Conclusion S

关 键 词：中枢神经系统 孤立性纤维性肿瘤/血管周细胞瘤 STAT6 预后分析 

分 类 号：R732.2[医药卫生—肿瘤]