中枢神经系统孤立性纤维性肿瘤5例并文献复习

5 Cases of Solitary Fibrous Tumor of the Central Nervous System and Literature Review

目的探讨中枢神经系统孤立性纤维性肿瘤的临床病理特点及鉴别诊断。方法对5例中枢神经系统孤立性纤维性肿瘤行常规HE和免疫组织化学染色检查,并随访;同时结合相关文献复习,对其临床病理特点进行分析。结果该类病变形态学表现多样性,呈现为密集增生的梭形肿瘤细胞排列成席纹状及血管外皮瘤样结构;另见细胞疏松区域,肿瘤细胞散在呈星芒状,伴胶原纤维玻璃样变。梭形细胞Vimentin、CD34、CD99、STAT6、Bcl-2均表达阳性;随着肿瘤级别的增高,Ki-67增殖指数亦增高(5%~20%);p53在中、高级别的病变中呈阳性表达。全组病例中1例患者复发,1例失访。结论原发于中枢神经系统的孤立性纤维性肿瘤非常少见,属于中间型肿瘤,有复发的倾向。组织形态学特点是重要的诊断依据,免疫组织化学检查可辅助其诊断及鉴别诊断。

Objective To investigate the clinicopathological features and differential diagnosis of solitary fibrous tumors of the central nervous system.Methods Routine HE and immunohistochemical staining were performed in 5 cases of solitary fibrous tumors of the central nervous system,and follow-up was conducted.At the same time,their clinicopathological features were analyzed in conjunction with the review of relevant literature.Results The morphologic features of the lesions were diverse.The main lesions were densely proliferated spindle tumor cells arranged into sheet striations and hemangiopericytoma-like structures.In addition,the area of loose cells is also seen.The tumor cells are scattered in a star-awn shape,accompanied by collagen fiber hyaline changes.Vimentin,CD34,CD99,STAT6 and Bcl-2 were positively expressed in spindle cells.With the increase of tumor grade,the Ki-67 value-added index increased(5%-20%).p53 was positively expressed in mid-and high-level lesions.In the whole group,1 patient relapsed and 1 lost to follow-up.Conclusion Solitary fibrous tumors originating in the central nervous system are rare,which are intermediate neoplasms with a tendency to recur.Histomorphology is an important diagnostic basis,and immunohistochemical examination can assist the diagnosis and differential diagnosis.