目的探讨发生于胸壁上皮样血管肉瘤的临床病理特征、病理诊断及鉴别诊断。方法对1例原发于胸壁的上皮样血管肉瘤进行组织形态学、免疫组织化学分析,并复习相关文献。结果肿瘤大体形态有主瘤结及卫星瘤节的特点,镜下观察其瘤细胞呈上皮样排列,异型性明显,免疫组化结果显示Vim强阳性,CD31阳性,CD34阳性。结论发生于胸壁的上皮样血管肉瘤较少见,术前确诊较困难,须与原发于胸膜的恶性间皮瘤、转移癌、恶性黑色素瘤等鉴别。 Objective To investigate the clinicopathological features, pathological diagnosis and differential diagnosis of thoracic wall-like angiosarcoma. Methods One case of primary angiomyolipoma of the thoracic wall was analyzed by histomorphology and immunohistochemistry, and the related literatures were reviewed. Results The general morphology of the tumor had the features of main tumor nodes and satellites. The tumor cells showed epithelial arrangement and obvious atypia. Immunohistochemical results showed that Vim was strongly positive, CD31 positive and CD34 positive. Conclusions Epithelioid angiosarcomas, which occur in the chest wall, are rare and difficult to diagnose before surgery. They should be differentiated from primary malignant mesothelioma in the pleura, metastatic carcinoma and malignant melanoma.