目的:探讨富于细胞性血管纤维瘤的临床病理特征和鉴别诊断。方法:对1例外阴富于细胞性血管纤维瘤进行临床组织病理学和免疫组织化学观察,并复习相关文献。结果:镜下见肿瘤有薄层纤维包膜,由短梭形细胞和大量小至中等大小的血管构成,梭形细胞分布疏密不均,可见多核瘤细胞,血管壁增厚伴广泛玻璃样变,部分血管扩张。免疫组化:肿瘤细胞Vimentin弥漫阳性,CD34弱阳性,EMA局灶阳性,而desmin,actin,ER,PR,S100,CD117均阴性。结论:富于细胞性血管纤维瘤是一种罕见的良性的间叶性肿瘤,应注意与血管肌纤维母细胞瘤,侵袭性血管粘液瘸,软组织多形性玻璃样变血管扩张性肿瘤等鉴别。 Objective: To investigate the clinicopathological characteristics and differential diagnosis of pleioblastic angiofibroma. Methods: One case of extravascularization-enriched cellular angiofibroma was observed by histopathology and immunohistochemistry. The related literatures were reviewed. Results: Microscopically, the tumor had a thin fibrous capsule surrounded by short spindle cells and a large number of small to medium-sized blood vessels. Spindle cells were unevenly distributed. The polytoplasm of the multinucleated tumor cells and thickening of the blood vessel wall were associated with a wide range of glassy Change, partial vasodilation. Immunohistochemistry showed that the tumor cells were diffusely positive for Vimentin, weakly positive for CD34 and positive for EMA, while negative for desmin, actin, ER, PR, S100 and CD117. CONCLUSION: Rich-cell angiofibroma is a rare benign mesenchymal tumor and should be differentiated from vascular myofibroblastic tumor, invasive vascular mucus lame, soft-tissue pleomorphic glass-like vasodilator.