目的　研究肝血管平滑肌脂肪瘤 ( angiomyolipoma,AML)临床、影像学、病理学特点 ,探讨如何降低本病误诊和漏诊率及提高外科疗效。方法　经病理证实的 AML9例。所有病人术前均接受 CT及 B超检查 ,部分病人还行彩色多普勒、MRI及血管造影。术前 3例临床诊断为原发性肝癌 ,1例为肝脂肪肉瘤 ,2例为 AML,3例为血管瘤。所有病人均行肝切除并随访至今。结果　剖腹探查发现肿瘤大多 ( 6/ 9)位于右肝 ,界限清晰 ,部分肿瘤有包膜。组织学检查显示肿瘤由脂肪细胞、梭形平滑肌样细胞及管壁异常增厚的小血管构成。9例中 2例病理曾诊断为原发性肝癌 ,1例诊断为肝腺瘤。HMB- 4 5单克隆抗体免疫组化检测发现全部肿瘤平滑肌样细胞均呈阳性反应。结论　肝 AML可能比以往认为的常见 ,易误诊。HMB- 4 5作为特异性免疫组化标记物对诊断有一定价值。对肝 AML行肝部分切除可获得良好效果 Objective To study the clinical, imaging and pathological features of hepatic angiomyolipoma (AML), explore how to reduce the misdiagnosis and missed diagnosis of the disease and improve the surgical efficacy. Methods 9 cases of AML confirmed by pathology. All patients underwent CT and B-ultrasound before surgery. Some patients also performed color Doppler, MRI, and angiography. Before surgery, 3 cases were diagnosed as primary liver cancer, 1 case was liposarcoma, 2 cases were AML, and 3 cases were hemangiomas. All patients underwent liver resection and follow-up so far. Results The laparotomy revealed that most of the tumors (6/9) were located in the right liver with clear boundaries and some tumors had envelopes. Histological examination revealed that the tumor consisted of adipocytes, fusiform smooth muscle-like cells, and small blood vessels with abnormally thickened walls. In 9 cases, 2 cases had pathological diagnosis of primary liver cancer, and 1 case had hepatic adenoma. Immunohistochemical detection of HMB-45 monoclonal antibody showed that all tumor smooth muscle-like cells were positive. Conclusion Liver AML may be more common than previously thought to be misdiagnosed. HMB-45 as a specific immunohistochemical marker has a certain value for diagnosis. Liver partial resection of liver AML can achieve good results