BACKGROUND:Hepatic angiomyolipoma(HAML)is a rare hepatic mesenchymal tumor.This study was designed to explore its clinical features. METHODS:Clinical data from 26 patients who had been pathologically confirmed with HAML and had received surgical resection at our hospital were analyzed retrospectively. RESULTS:HAML was seen more frequently in females(18/26) in this series,and most of the patients presented no significant symptoms except for one who had a spontaneous rupture hemorrhage.Serum alpha-fetoprotein(AFP),carbohydrate antigen 19-9(CA19-9)and carcinoembryonic antigen(CEA) were negative in all patients.Imaging presentations were diverse.Pre-operative diagnosis was made in only 3 patients Pathological study showed that the tumor was composed of adipose tissue,smooth muscle and blood vessels in different proportions.One patient showed hepatic vessel invasion HMB-45 immunohistochemical staining was positive in all tumors.All patients underwent surgical resection without significant complications.Except for one patient who died 14 months after operation because of recurrent disease,no tumor recurrence was observed in the remaining 25 patients during a 2-3 years follow-up. CONCLUSIONS:Pre-operative diagnosis of HAML is difficult.There are potential risks of spontaneous rupture and malignant transformation.Surgical resection is the treatment of choice for HAML. METHODS: Clinical data from 26 patients who had been pathologically confirmed with HAML and had received surgical resection at our hospital were analyzed retrospectively. RESULTS: HAML was seen more frequently in females (18/26) in this series, and most of the patients presented no significant symptoms except for one who had had spontaneous rupture hemorrhage. Serum alpha-fetoprotein (AFP), carbohydrate antigen 19-9 (CA19-9) and carcinoembryonic antigen (CEA) were negative in all patients. Imaging of performed were were diverse. Pre-operative diagnosis was made in only 3 patients Pathological study showed that the tumor was composed of adipose tissue, smooth muscle and blood vessels in different proportions. One patient showed hepatic vessel invasion HMB-45 immunohistochemical staining was positive in all tumors. All patients underwent surgical resection without significant c omplications. Except for one patient who died for 14 months after operation because of recurrent disease, no tumor recurrence was observed in the remaining 25 patients during a 2-3 years follow-up. CONCLUSIONS: Pre-operative diagnosis of HAML is difficult.There are potential risks of spontaneous rupture and malignant transformation. Surgical resection is the treatment of choice for HAML.