肝脏血管平滑肌脂肪瘤的诊治分析

Analysis of Diagnosis and Treatment on Hepatic Angiomyolipoma

目的总结肝脏血管平滑肌脂肪瘤（hepatic angiomyolipoma，HAML）的临床、影像及病理特点，提高对HAML的诊治水平。方法收集新疆医科大学附属肿瘤医院3例经手术切除和术后病理确诊的HAML患者，结合相关文献，分析肝HAML的临床表现、典型影像及病理学特点。结果1例术前确诊，另2例误诊为肝脏其他肿瘤。3例术后随访32-39月，均未见肿瘤复发及转移。临床上，HAML与其他肝良性肿瘤相比无特异性。因3种组织比例差异，影像学表现呈现多样性，在众多影像检查中以CT诊断率最高。常规病理检查加免疫组化可确诊及分型，其中免疫组化标记HMB-45阳性具有重要诊断意义。结论HAML是一种少见的肝脏良性肿瘤，术前诊断率低，但只要掌握其影像特点，结合临床病史及实验室检查，术前确诊已成为可能。积极手术治疗，预后良好。

Objective To increase diagnosis and treatment level of hepatic angiomyolipoma （ HAML ）, by summarizing characteristics of clinical findings, imaging appearance and pathology. Methods Three cases with HAML diagnosed by exairesis and routine pathology after operation in the hospital were collected to analyze clinical feature, typical imaging and pathological characteristic of HAML by combining with relative literatures. Results There were one case diagnosed correctly preoperatively and another two misdiagnosed as hepatic neoplasm or others. By following 32 to 39 months after operation; no recurrence and aversion were found in all three cases. Clinically, HAML was of no specificity compared with other benign tumor of liver. The imaging showed diversity due to the difference of proportion of the three tissues. Rate of CT diagnosis was the highest in all imaging examination. Routine pathology and immunohistochemistry could get final diagnosis and grouping, and HMB - 45 positive marked by immuohistochemistry was of the most importance. Conclusions HAML is rare benign tumor of liver with low rate of pre - operative diagnosis. It can be diagnosed finally before operation by knowing imaging characteristics and combing with clinical history, and laboratory examination as well. The prognosis is good by positive operation therapy.