肝脏血管平滑肌脂肪瘤合并结节硬化的影像诊断和介入治疗

目的：探讨肝脏血管平滑肌脂肪瘤（HAML）合并结节硬化的影像诊断、鉴别诊断和介入治疗。方法：分析2例HAML合并结节硬化的临床表现、影像学表现和介入治疗方法。结果：HAML合并结节硬化具有以下表现；（1）女性2例，无乙型肝炎及肝硬化病史，肝功能及甲胎蛋白正常；（2）超声为多发的边界清楚的强回声肿块，CT为有明确脂肪密度的肿块，DSA为血供丰富，早期肝静脉引流，肿瘤染色明显。（3）10例介入治疗，术后随访42个月病灶有所缩小，另1例肝脏病灶未处理，随访10个月，肝脏病灶无变化。结论：HAML合并结节硬化的影像学表现有一定特征性，超选择动脉栓塞是治疗HAML合并结节硬化的有效方法。

The diagnostic imaging and interventional therapy of hepatic angiomyolipoma associated with tuberous sclerosis

Objective To evaluate imaging diagnosis, differential diagnosis, surgery and interventional therapy of hepatic angiomyolipoma associated with tuberous sclerosis.Methods Clinical features, imaging appearances and interventional therapy for 2 cases of HAML in TS were retrospectrively analyzed. Results The features of HAML in TS are as following:(1) Two cases were female with no history of hepatitis and hepatocirrhosis, liver function test and alpha feto protein were normal. (2) the tumor was shown as a multiple hyperechoic mass with sharp margin on ultrasonography; well defined, fat density and intensity mass on computerized tomography (CT); hypervascular mass with early drainage of hepatic vein and tumor staining on angiography. (3) One case underwent interventional therapy. After 42 month follow up, the tumor was found to be diminished in size. Another case was under monitored with only the enucleating of left renal mass. The hepatic tumor showed no change after 10 months.Conclusions The imaging appearances of HAML in TS are relatively characteristic. Superselective arterial embolization is an effective treatment for HAML in TS.