【摘要】目的探讨肝血管平滑肌脂肪瘤的临床病理特点、诊断及鉴别诊断。 方法对3例肝血管平滑肌脂肪瘤患者有关病理检查结果进行回顾性分析。 结果肿瘤位于肝右叶2例，肝左叶1例。肿瘤直径为2～10 cm，平均6.2 cm。3例肿瘤内均见平滑肌、脂肪、畸形厚壁血管，但未见髓外造血灶。对黑色素瘤（HMB45）、结合蛋白（desmin)及肌动蛋白（actin)检查均呈阳性反应。术后随访6～36个月，未见肿瘤复发。结论肝血管平滑肌脂肪瘤由3种成分组成，病理形态变化多样，必须与多种肝肿瘤相鉴别。平滑肌细胞HMB45表达呈强阳性反应是诊断肝血管平滑肌脂肪瘤较可靠的依据。
Hepatic angiomyolipoma (HAML) is a rare benign mesenchymal tumor of the liver, which has highly variable imaging appearances, often leads to missed diagnosis and misdiagnosis. The images of 2 patients with HAML confirmed by pathology were presented in this study, and the typical imaging features of the HAML, the underlying pathophysiological mechanism, and the differential diagnosis were briefly summarized so as to deepen the understanding of HAML and to improve the diagnosis and differential diagnosis abilities of HAML, then reduce the rates of missed diagnosis and misdiagnosis of the HAML.