目的: 探讨颅内孤立性纤维性肿瘤/血管周细胞瘤（solitary fibrous tumor/hemangiopericytoma,SFT/HPC）的临床病理特征、诊断、鉴别诊断方法及预后。方法: 对20例颅内SFT/HPC患者的临床、组织病理学特征及免疫组织化学（免疫组化）检测结果进行观察,并复习相关文献进行探讨。结果: 20例SFT/HPC患者中,男性12例,女性8例,平均年龄为48岁,其中7例有脑膜瘤切除史6~19年。根据2016版最新WHO中枢神经系统肿瘤分级,SFT/HPC Ⅰ级病例1例,Ⅱ级病例13例,Ⅲ级病例6例。免疫组化结果显示,20例患者的病理组织均表达波形蛋白（vimentin）、bcl-2、CD34和STAT6,其中CD34在Ⅰ级肿瘤组织中呈强阳性、弥漫表达,而在Ⅱ级及Ⅲ级肿瘤组织中呈弱阳性表达、斑片状分布,所有病例均不表达上皮膜抗原（epithelial membrane antigen,EMA）、AE1/AE3、S-100、CD31和Ⅷ因子,Ki-67增殖指数为3%~30%。20例患者中,15例获得随访资料,随访时间为5个月至19年,5例患者复发,13例行术后放疗,2例术后未进行治疗,截至2017年3月,所有病例均存活。结论: 新版WHO将SFT和HPC归为一类,并作出新的组织学分级。本研究发现CD34、Ki-67免疫组化染色结果有助于SFT/HPC的组织学分级和鉴别诊断。
Objective: To study the clinicopathologic features, diagnosis, differential diagnosis and prognosis of intracranial solitary fibrous tumor/hemangiopericytoma(SFT/HPC). Methods: A total of 20 cases of intracranial SFT/HPC were enrolled in this study. The clinical data, pathologic characteristics and immunophenotype were studied and relevant literatures were reviewed. Results: Of these patients, 12 cases were male and 8 were female, with an average age of 48.45 years. Seven cases received meningioma resection 6-19 years before. According to the latest 2016 WHO classification, there were 1 case of grade Ⅰ, 13 cases of grade Ⅱ and 6 cases of grade Ⅲ. Immunohisochemically, tumor cells of all specimen were positive for vimentin, bcl-2, CD34 and STAT6, and were negative for EMA, AE1/AE3, S-100, CD31 and factor Ⅷ. The cells of grade Ⅰ case were strong and diffusely positive for CD34, while the cells of grade Ⅱ and grade Ⅲ cases had weak expression and patchy distribution of CD34. Ki-67 proliferation index ranged from 3% to 30%. Fifteen of the 20 patients were followed-up for 5 months to 19 years. During this period, 5 cases had disease recurrence, and 13 received postoperative radiotherapy, while 2 case did not receive any therapy. Up to March 2017, all cases were survived. Conclusions: According to the latest releas from WHO, SFT and HPC are classified as the same type of tumor, and immunohistochemistry staining of CD34 and ki-67 may be useful to grading and differential diagnosis of SFT/HPC.
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