肝脏血管平滑肌脂肪瘤的诊断和治疗  

Diagnosis and treatment of hepatic angiomyolipoma

作　　者：丁光辉[1] 吴孟超[2] 杨广顺[2] 杨甲梅[2] 丛文铭[2] 

机构地区：[1]同济大学附属上海市第十人民医院肝胆外科,上海200072 [2]第二军医大学东方肝胆外科医院,上海200438

出　　处：《第二军医大学学报》2010年第6期615-620,共6页Academic Journal of Second Military Medical University

摘　　要：目的分析并总结肝脏血管平滑肌脂肪瘤(AML)的临床表现和诊断,探讨其治疗策略。方法对1992-2006年第二军医大学东方肝胆外科医院手术病理证实的79例肝AML临床资料进行回顾性分析研究,总结其诊治经验。结果患者女58例,男21例。发病年龄17～69岁,平均(43±8.14)岁,均无合并肾血管平滑肌脂肪瘤或结节性硬化症。有临床症状者25例。肿瘤大小1.0～25cm,平均(6.1±4.08)cm,均为单发肿瘤。肝右叶53例,左叶22例,尾状叶4例。术前正确诊断者41例(52%)。自发性破裂出血1例。79例均手术切除,无手术死亡或严重并发症。术后随访3～13年,1例术后6年复发,1年后死亡。结论肝血管平滑肌脂肪瘤无特异性临床症状,综合影像学检查有助于术前正确诊断,但鉴别诊断困难,特别是对直径小于5cm的肿瘤术前难以确诊。最终诊断依赖于病理组织学检查和HMB-45免疫组化染色。手术切除是治疗肝血管平滑肌脂肪瘤的安全、有效方法。应警惕潜在恶性和自发性破裂出血可能,一旦诊断明确宜尽早手术,术后应密切随访。Objective To analyze the clinical features and diagnosis of hepatic angiomyolipoma patients,and to investigate its treatment strategy.Methods The clinical data of 79 patients with pathologically-confirmed hepatic angiomyolipoma,who were treated in the Eastern Hepatobiliary Surgery Hospital during 1992-2006,were retrospectively reviewed.Results The patients included 58 women and 21 men with a median age of （43±8.14） years old （ranging 17 to 69 years）.None of the patients was found complicated with renal angiomyolipoma or tuberous sclerosis complex.Twenty-five patients were symptomatic on presentation.All the patients had a single hepatic lesion,with the tumor size ranging 1.0-25 cm and a median of （6.1±4.08） cm.Fifty-three tumors were located in the right lobe,22 in the left lobe,and 4 in the caudate lobe.Preoperative diagnoses were correctly made in 41 （52%） patients.Spontaneous rupture of tumor occurred in 1 patient.All the patients underwent surgical treatment without operative mortality and serious morbidity.The patients were regularly followed-up for 3-13 years.Tumor recurrence occurred in one patient 6 years after the surgery,and the patient died 1 year later.Conclusion Hepatic angiomyolipoma has no specific clinical signs and symptoms.Comprehensive imaging studies can help to make correct diagnosis,but the differential diagnosis is difficult,especially for small tumors with diameter less than 5 cm.The final diagnosis depends on pathologic examination and HMB-45 special staining.Surgical resection is safe and effective for the disease.Attention should be paid to the potential of malignant transformation and risk of rupture as a life-threatening complication.Early operation is needed once a diagnosis is made,and the patients should be followed up after operation.

关 键 词：肝肿瘤 血管平滑肌脂肪瘤 诊断 肝切除术 

分 类 号：R735.7[医药卫生—肿瘤]