【摘要】 目的探讨巨细胞血管纤维瘤(giant cell angiofibrom a,GCA)临床病理学特征。方法收集3例GCA的临床病理资料,进行病理学分析,并复习相关文献。结果患者年龄为15、45和69岁,1例男性(眼睑),2例女性(肩背部、眼眶),均表现为缓慢生长的无痛性肿块,边界较清。镜下肿瘤表现出3种结构2种细胞:肿瘤细胞分密集区和稀疏区;富含血管和内衬巨细胞的假血管腔隙,腔隙间由无一定排列的增生的圆形、卵圆形、短梭形细胞组成,细胞无异型性;间质胶原化或黏液样变性,局部类似孤立性纤维瘤的形态。3例均表达CD34、CD99、vimentin、Bcl-2。结论 GCA是具有特征性的好发于成年人眶区,亦可发生于眶区以外的良性或低度恶性潜能的肿瘤,需与孤立性纤维瘤、巨细胞纤维母细胞瘤等鉴别。更多还原

【Abstract】 Purpose To investigate the clinicopatholgical features of giant cell angiofibroma(GCA).Methods The clinicopathological features were analyzed in 3 cases of GCA,and the literatures were reviewed.Results The age of the 3 patients were 15,45 and 69 years old,there was 1 male(eyelid) and 2 females(scapular region、orbital).All of the cases presented with slowly growing,painless masses.Grossly,they were well-circumscribed tumors.Microscopically,the tumors revealed three patterns and two cells: hypercellular and hypocellular area in tumor;The tumor was characterized by a prominent network of blood vessels and irregular cleftlike or sinusoid psendovascular spaces that were lined with a layer of discontinuous multinucleate giant cells,and was composed of a patternless proliferation of round,ovoid or short spindle cells.Both nuclear atypia and mitotic activity were absent.The stroma displayed a variable degree of collagenization or myxoid degeneration with some areas recapitulating the patternless pattern of solitary fibrous tumor.Immunohistochemically,tumour cells were positive for CD34,CD99,vimentin and Bcl-2.Conclusions Giant cell angiofibroma is a distinctive benign lesion or potentially a tumor of low-grade malignancy,which occurred predominantly in the orbit or extraorbital in adults.Histologically,this rare entity should be distinguished from giant cell fibroblastoma,extrapleural solitary fibrous tumor and so on.更多还原