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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 and summarize the clinical manifestations and diagnosis of hepatic angiomyolipoma (AML) and discuss its treatment strategy. Methods The clinical data of 79 cases of liver AML confirmed by surgery and pathology from the Eastern Hepatobiliary Surgery Hospital of the Second Military Medical University from 1992 to 2006 were retrospectively analyzed and the experience of diagnosis and treatment was summarized. Results The patient was female 58 cases, male 21 cases. The age of onset was 17 to 69 years (mean, 43 ± 8.14 years), with no renal angiomyolipoma or tuberous sclerosis. 25 cases of clinical symptoms. Tumor size 1.0 ~ 25cm, mean (6.1 ± 4.08) cm, are single tumors. 53 cases of right lobe, left lobe in 22 cases, caudate lobe in 4 cases. The correct diagnosis before surgery in 41 cases (52%). Spontaneous rupture of a case of bleeding. All 79 cases were surgically removed without any operative death or serious complications. The patients were followed up for 3 to 13 years. One patient relapsed 6 years after operation and died one year later. Conclusions Hepatic angiomyolipoma has no specific clinical symptoms. The comprehensive imaging examination is helpful for the correct diagnosis before operation, but the differential diagnosis is difficult, especially for the tumors less than 5 cm in diameter. The final diagnosis relies on histopathology and HMB-45 immunohistochemical staining. Surgical resection is a safe and effective method for the treatment of hepatic angiomyolipoma. Should be alert to potentially malignant and spontaneous rupture may, once the diagnosis should be as soon as possible surgery, postoperative follow-up should be closely.