颅内孤立性纤维性肿瘤/血管周细胞瘤20例临床病理特征分析

Intracranial solitary fibrous tumor/hemangiopericytoma: a clinicopathologic study of 20 cases with review of literature

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/HPCⅠ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 in-tracranial 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 Ⅲcases 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 re-ceived 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 im-munohistochemistry staining of CD34 and ki-67 may be useful to grading and differential diagnosis of SFT/HPC.