肝血管平滑肌脂肪瘤的诊断和治疗  

Diagnosis and treatment of hepatic angioleiomyolipoma: A case report and literature review

作　　者：李永杰[1] 刘文艺[1] 刘莉[1] 周耿[1] 方孟园 

机构地区：[1]遵义医学院附属医院肝胆外科,贵州省遵义市563000

出　　处：《世界华人消化杂志》2014年第20期2946-2951,共6页World Chinese Journal of Digestology

摘　　要：目的:探讨肝血管平滑肌脂肪瘤(hepatic angiomyolipoma,HAML)的临床及影像学表现、病理特点、鉴别诊断、治疗及预后.方法:报道H A M L临床病例1例,检索1987-2014年国内文献,取其中资料相对完整的540例,对541例HAML临床表现、实验室检查、影像学检查、手术方式、病理学检查和随访资料进行临床分析.结果:541例HAML患者以女性多见,无明显症状361例(66.7%),实验室检查多无异常,影像学诊断阳性率:B超1.4%(4/283)、CT 37.4%(131/350)、MRI 16.17%(38/235),且CT诊断价值较高(CT vs MRI:χ2=11.321,P=0.001;CT vs B超:χ2=42.105,P=0.000),病理诊断免疫组织化学指标肿瘤细胞表达黑色素瘤抗体-45(human melanoma black-45,HMB-45)表达阳性率97.8%(357/365)、平滑肌激动蛋白(smooth muscle actin,SMA)阳性率93.7%(207/221)、Vimentin阳性率62.5%(30/48)、CD-34阳性率40.5%(51/126)、CD-117阳性率39.0%(48/123),外科手术治疗521例(96.3%),术后良性预后98.7%(231/234).结论:HAML缺乏特征性临床表现,影像学表现多样,术前诊断正确率低、误诊率高,有待病理及免疫组织化学确诊,首选手术治疗,预后良好.AIM： To investigate the clinical features, imaging manifestations, pathological characteristics, differential diagnosis, treatment and prognosis of hepatic angiomyolipoma（HAML）. METHODS： A case of HAML diagnosed at our hospital is reported. A review of the literature was performed to retrospectively analyze 540 cases reported in China between 1987 and 2014.RESULTS： HAML was more frequently found in females（2.7：1）. Most of the patients（66.7%） presented no significant symptoms, and tumor marks were negative in all the patients. CT performed better for preoperative diagnosis than MRI（CT vs MRI： χ^2 = 11.321, P = 0.001） and ultrasound. Immunohistochemical analyses revealed that the positive expression rates of human melanoma black-45 antigen（HMB-45）, smooth muscle actin（SMA）, Vimentin, CD34 and CD117 in HAML were 97.8%（357/365）, 93.7%（207/221）, 62.5%（30/48）, 40.5%（51/126） and 39.0%（48/123）, respectively. The expression of HMB-45 was considered to have great significance in confirming the diagnosis. Of all the patients, 521（96.3%） underwent surgical inter-vention, and recurrence or metastasis was found in only 3 patients during follow-up.CONCLUSION： HAML lacks specific symptoms or signs, has diverse imaging manifestations, and can be easily misdiagnosed. Pathological and immunohistochemical analyses are essential for diagnosis, and surgical treatment has a good prognosis.

关 键 词：肝血管平滑肌脂肪瘤 影像学检查 免疫组织化学 

分 类 号：R735.7[医药卫生—肿瘤]