目的　探讨眼眶巨细胞血管纤维瘤的组织病理学特征 ,侧重于诊断和鉴别诊断。方法　采用HE染色和免疫组化标记 ,对 2例发生于眼眶的巨细胞血管纤维瘤进行临床病理学分析。结果　 2例均表现为眼眶部位缓慢性生长的无痛性肿块。大体上肿瘤境界较清楚 ,镜下显示特征性改变 :肿瘤的主要成份由无结构性增生的圆形、卵圆形和短梭形细胞组成 ,增生的瘤细胞无异型性 ;肿瘤内富含血管及内衬不连续性多核巨细胞的裂隙样或血窦样假血管性腔隙 ;肿瘤的间质伴有不同程度的胶原变性或粘液样变性 ,部分区域可形成类似孤立性纤维瘤中无结构样的结构。免疫组化标记显示瘤细胞和多核巨细胞表达CD34和Vimentin。 2例均作了局部切除 ,术后随访均健在。结论　巨细胞血管纤维瘤是一种好发于成年眼眶的良性或具潜在低度恶性的软组织肿瘤 ,局部切除多可治愈。组织学上必须与巨细胞纤维母细胞瘤、胸膜外孤立性纤维瘤、多核细胞血管组织细胞瘤及血管瘤样纤维组织细胞瘤等相鉴别 Objective To investigate the histopathological features of orbital giant cell angiofibroma, focusing on the diagnosis and differential diagnosis. Methods The histopathological analysis of 2 cases of giant cell vascular fibroma in the orbit was made by HE staining and immunohistochemical staining. Results Both of the two cases showed painless masses with slowly growing orbital parts. In general, the realm of the tumor is clearer and the characteristic changes are observed microscopically. The main components of the tumor are composed of round, oval and short spindle cells without structural proliferation, and the tumor cells are hyperplastic. The tumor is rich in blood vessels And fissure-like or sinusoids-like pseudo-vascular lacuna lined with discontinuous multinucleated giant cells; interstitial tumor with varying degrees of collagen degeneration or myxoid degeneration, some areas can form a similar solitary fibromatosis without structure Kind of structure. Immunohistochemical markers showed that tumor cells and multinucleated giant cells express CD34 and Vimentin. 2 cases were made partial resection, follow-up are healthy. Conclusion Giant cell hemangioma is a benign or potentially low-grade soft tissue tumor that occurs in adult eyes. Local excision can be cured. Histologically, it must be differentiated from giant cell fibroblastoma, extranodal solitary fibrous tumor, multinucleated hemangioblastoma and hemangioma-like fibrous histiocytoma