【摘要】目的探討肝血管平滑肌脂肪瘤的臨床病理特點、診斷及鑒別診斷。 方法對3例肝血管平滑肌脂肪瘤患者有關病理檢查結果進行回顧性分析。 結果腫瘤位于肝右葉2例，肝左葉1例。腫瘤直徑為2～10 cm，平均6.2 cm。3例腫瘤內均見平滑肌、脂肪、畸形厚壁血管，但未見髓外造血灶。對黑色素瘤（HMB45）、結合蛋白（desmin)及肌動蛋白（actin)檢查均呈陽性反應。術后隨訪6～36個月，未見腫瘤復發。結論肝血管平滑肌脂肪瘤由3種成分組成，病理形態變化多樣，必須與多種肝腫瘤相鑒別。平滑肌細胞HMB45表達呈強陽性反應是診斷肝血管平滑肌脂肪瘤較可靠的依據。
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.