摘要目的 由于中枢神经系统(CNS) 孤立性纤维性肿瘤(SFT)与血管外皮瘤(HPC)的分子遗传学特征一致,2016 WHO CNS肿瘤分类将二者合为SFT/HPC,并分为3级.本文主要探讨CNS SFT/HPC的临床病理学特征、诊断、鉴别诊断及临床预后等.方法 收集71例CNS SFT和HPC进行回顾性研究和重新分级,对组织形态学、免疫组织化学和临床影像资料进行分析,并进行随访及复习相关文献.结果 71例患者中男性37例,女性34例;年龄3~77岁,中位年龄48岁.WHO Ⅰ级12例(17%)、WHOⅡ级26例(37%)和WHOⅢ级33例(46%).镜下可表现为经典的SFT表型、HPC表型或二者混合.免疫表型上,97%(69/71)的病例表达STAT6,其中96%(66/69)的病例弥漫强阳性;bcl-2、CD99及波形蛋白表达率均约为90%;CD34表达率随着肿瘤级别升高而降低,平均表达率为78%,10%(7/71)的病例不同程度表达SSTR2a,其中1例胞质强阳性;极少数病例局灶表达上皮细胞膜抗原、CD57、S-100蛋白;Ki-67阳性指数1%~50%.34例随访8~130个月,其中12例复发(35%),2例(6%)发生肝转移.结论 CNS SFT/HPC相对少见,不同级别之间临床病理和预后之间明显重叠或过渡.STAT6是目前诊断该肿瘤较为特异的标志物.手术切除为首选治疗手段,Ⅱ级和Ⅲ级易复发及转移,最常转移部位为肝脏.

abstractsObjective As solitary fibrous tumor (SFT) and hemangiopericytoma (HPC) share the same molecular genetics features, the 2016 WHO classification of central nervous system (CNS) tumors had created the combined term SFT/HPC and assigns three grades.This study aims to investigate the clinicopathologic characteristics, diagnosis, differential diagnosis and prognosis of CNS SFT/HPC.MethodsSeventy-one cases of CNS SFT and HPC were retrospectively reclassified and studied.Histopathological, immunohistochemical and imaging features were analyzed.The follow-up data were analyzed.ResultsThere were 37 male and 34 female patients.The median age was 48 years (range, 3-77 years).Twelve cases (17%) were WHO grade Ⅰ, 26 (37%) were WHO grade Ⅱ and 33 (46%) were WHO grade Ⅲ.Microscopically the tumor could show traditional SFT phenotype, HPC phenotype or mixed phenotype.Immunochemically, 97%(69/71) were positive for STAT6, with 96%(66/69)showing diffuse strong staining.Approximately 90% were diffusely positive for bcl-2, CD99 and vimentin.The expression rate of CD34 decreased with increasing tumor grade, and the mean expression rate was 78%.SSTR2a was variably expressed in 10% (7/71) of cases including one case showing strong cytoplasmic staining.A few cases expressed EMA, CD57 and S-100 focally.The Ki-67 index ranged from 1% to 50%.Thirty four patients were followed up for 8-130 months;12 patients(35%)had recurrences, and two (6%) had liver metastases.Conclusions CNS SFT/HPC is relatively uncommon.There was significant morphological overlap or transition between different grades.STAT6 is a specific marker for the diagnosis of this tumor.Surgical resection is the preferred treatment.WHO grade Ⅱ and Ⅲ SFT/HPC show rates of local recurrence and systemic metastasis, with liver being the most common site of extracranial metastasis.