摘要目的　研究肝血管平滑肌脂肪瘤的临床病理学特点，并介绍一种新抗体A103的诊断意义。 方法　对10例肝血管平滑肌脂肪瘤的临床资料进行复习，并用石蜡切片进行组织化学、免疫组化检测（包括A103），其中3例进行电镜观察。 结果　10例AML中8例女性，2例男性，平均年龄44岁。临床无症状，影像学检查偶然发现。大体上为境界清楚但无包膜的肿块；光镜下由三种成分(厚壁血管、梭形或上皮样平滑肌细胞及脂肪)混合组成。免疫组化检查瘤细胞均表达A103，HMB-45及平滑肌标记。随访10例病人均无复发。结论　血管平滑肌脂肪瘤的三种主要组成成分的比例和分布各异，致使组织学形态变化多端。确切的病理诊断还需借助于免疫组化，尤其是A103对鉴别诊断有重要意义。

abstractsObjective　To study the clinicopathologic features of hepatic angiomyolipoma (AML) and to investigate the feasibility of a new antibody-A103 as a diagnostic aid for AML.Methods　Ten cases of AML were retrieved from hospital records and analyzed morphologically. Immunohistochemistry was performed on paraffin-embedded tissues with a panel of antibodies, including antibody-A103.Results　There were eight women and two men, with ages ranging from 38-58 years (median 45.7). Clinically, nine cases were asymptomatic and found by imaging techniques. None of the patients had associated tuberous sclerosis. All tumors were sharply demarcated from the surrounding liver parenchyma. Histologically they were composed of a heterogeneous mixture of three components: thick-walled blood vessels, spindle or epithelioid smooth muscle cells and adipose tissue. All tumors showed a strong immunoreactivity to A103, HMB-45 and smooth muscle actins. Follow-up information on all 10 cases showed a benign course with no signs of recurrence. Conclusions　Hepatic AML is a rare mesenchymal tumor of the liver. A103 is a promising marker for a pathologic diagnosis of hepatic AML.