AIM:To summarize the experience of the diagnosis andtreatment of hepatic angiomyolipoma(HAML).METHODS:The clinical,imaging and pathological features,and treatment strategies of 26 patients with HAML treatedat the authors’ institute between October 1998 and January2003 were retrospectively analyzed.All the patientsreceived liver resection and were followed up till the study.Immunohistochemical assays were performed with a panelof antibodies.RESULTS:There was an obvious female predominance(21:5),and most of the patients(18/26)had no symptoms.Heterogeneous high echo was found in ultrasonography andpunctiform or filiform vascular distribution pattern was foundin color Doppler-sonography in most of the lesions(21/26).All of the 5 lesions further enhanced with Levovist showedearly and prolonged enhancement.At contrast-enhancedspiral CT,the soft-tissue components of 24 lesions weremarkedly enhanced in the arterial phase and 18 lesionsremained enhanced in the portal venous phase.MRI wasperformed in 9 patients,and showed hypointensity orhyperintensity on T1-weighted images and heterogeneoushyperintensity on T2-weighted images.Histopathologically,all lesions were composed of adipose tissues,smooth muscleand blood vessels with different proportions.Most lesionsshowed positive immunohistochemical staining for HMB45(26/26),A103(24/26)and SMA(24/26).All of the 26 patientsshowed a benign course with no sign of recurrence.CONCLUSION: Preoperative radiological diagnosis of HAML is possible. The demonstration of intratumoral fat and central vessels is helpful in the diagnosis. HMB45, A103 and SNA are promising markers for pathologic diagnosis of HAML, and surgical resection is effective for the treatment of HAML. AIM: To summarize the experience of the diagnosis and treatment of hepatic angiomyolipoma (HAML). METHODS: The clinical, imaging and pathological features, and treatment strategies of 26 patients with HAML treatedat the authors’ institute between October 1998 and January 2003 were retrospectively analyzed. All the patientsreceived liver resection and were followed up till the study. Immunohistochemical assays were performed with a panel of antibodies .RESULTS: There was an obvious female predominance (21: 5), and most of the patients (18/26) had no symptoms. Heterogeneous high echo was found in ultrasonography and punctiform or filiform vascular distribution pattern was foundin color Doppler-sonography in most of the lesions (21/26). All of the 5 lesions further enhanced with Levovist showedearly and prolonged enhancement. A contrast-enhancespiral CT, the soft-tissue components of 24 lesions were markedly enhanced in the arterial phase and 18 lesions were enhanced in the portal venous phase. MRI wasperform ed in 9 patients, and showed hypointensity orhyperintensity on T1-weighted images and heterogeneoushyperintensity on T2-weighted images. Histopathologically, all lesions were composed of adipose tissues, smooth muscle and blood vessels with different proportions. Host lesionsshowed positive immunohistochemical staining for HMB45 (26 / 26), A103 (24/26) and SMA (24/26). All of the 26 patientsshowed a benign course with no sign of recurrence. CONCLUSION: Preoperative radiological diagnosis of HAML is possible. The demonstration of intratumoral fat and central vessels is helpful in the diagnosis. HMB45, A103 and SNA are promising markers for pathologic diagnosis of HAML, and surgical resection is effective for the treatment of HAML.