肝血管平滑肌脂肪瘤的外科治疗

The clinical characteristics and surgical treatment of hepatic angiomyolipoma

目的探讨肝血管平滑肌脂肪瘤的临床表现、病理学特征、诊断和外科治疗。方法回顾性分析我院1990年1月至2006年12月诊治的22例肝血管平滑肌脂肪瘤（hepatic angiomyolipoma，HAML）患者的临床资料。结果22例患者中男6例，女16例，平均年龄48．2岁。17例患者无临床症状，所有患者均无肝炎病史，均无肾血管平滑肌脂肪瘤病史。女性患者无口服避孕药史。血AFP、CEA、CA19-9、CA242均正常。术前超声、CT、MRI诊断准确率分别为21％（4／19）、23．5％（4／17）、25％（2／8）。肿瘤均为单发，直径4—17cm，均无完整包膜。22例患者均行手术切除，17例行肝肿瘤不规则切除术，5例行左半肝切除术。1例患者术后第2天死于并发症，1例患者出现轻微胆漏，术后病理免疫组化HMB45均表达阳性，4例见髓外造血明显。随访率91％（20／22），随访时间6个月至17年，术后无肿瘤复发。结论肝血管平滑肌脂肪瘤无特异性临床症状，术前影像学检查难以确诊，应积极的手术治疗。

Objective To evaluate the clinical presentation, pathologic features, diagnosis and treatment of hepatic angiomyolipoma （HAML）. Methods Retrospective analysis was made on 22 HAML cases in terms of clinical findings, images, pathologic examination and surgical treatment. Results Of the 22 patients, 6 were male and 16 were female. The average age was 48. 2 years. Seventeen patients had no clinical symptoms. None had the history of hepatitis virus infection. None of them was complicated with renal AML. None had the history of taking oral contraceptives in the sixteen female patients. Serum AFP,CEA, CA19-9,CA242 levels were all within normal limits. The correct diagnostic rate of BUS, CT and MRI was 21% （4/19）,23.5% （4/17）and 25% （2/8）, respectively. All tumorts were single nodule with a diameter of 4 - 17 cm, without complete capsula. All these 22 patients underwent tumor resection, including partial liver resection in 17 patients, left hemihepatectomy in 5. One patient died of postoperative complication, one patient suffered from temporary bile leak. The HMB45 positive rate by immunohistochemical method was 100%. Extramedullary hemopoiesis was found in 4 patients. Follow up of 6 months to 17 years in 91% （20/22） patients found no tumor recurrence. Conclusions HAML had no specific symptoms. The preoperative imaging diagnosis is difficult. Surgical resection is the therapy of choice for HAML.