乏脂性肾血管平滑肌脂肪瘤患者9例临床分析

Clinical diagnosis and treatment of renal angiomyolipoma with less adipose tissue

目的 提高对乏脂性肾血管平滑肌脂肪瘤的认识,探讨其诊治方法.方法 报告9例经本院收治的乏脂性肾血管平滑肌脂肪瘤的临床资料,回顾性分析其临床特征及影像学表现.9例患者均无明显临床症状.术前超声检查均提示低回声占位,占位可见血流信号.CT检查8例平扫期未见脂肪样低密度.肿瘤动脉期呈现明显强化,CT值最高可达100 Hu,实质期肿瘤持续均匀强化.术前诊断为肾脏恶性肿瘤.另1例患者平扫可见局部点状低密度灶,增强扫描点状低密度灶无强化.分析所有患者临床资料并检索Pubmed和CBM数据库,对该疾病相关文献进行复习.结果 所有患者均接受手术治疗.1例因患者原因施行根治性肾切除术,余8例患者接受保留肾单位的肾部分切除术.其中6例行腹腔镜肾部分切除术,2例行开放肾部分切除术.术后病理提示肾血管平滑肌脂肪瘤.所有患者均获随访9～36个月,未见肿瘤复发.结论 乏脂性肾血管平滑肌脂肪瘤临床少见,对病灶CT的细微分析能够给诊断带来帮助,对于乏脂性肾血管平滑肌脂肪瘤特别是体积较小的肿瘤应该力争行保留肾单位的手术,以减少盲目肾切除造成的过度治疗.

Objective To explore the diagnosis and treatment of renal angiomyolipoma with less adipose tissue.Methods Clinical data of 9 cases of renal angiomyolipoma with less adipose tissue were reported,and the clinical characteristics and image findings were retrospectively analyzed.None of the 9 patients presented special clinical manifestations.All patients had undergone ultrasonography and showed a hypoecho mass,with obvious blood flow signal.The CT scan did not show fatlike low density at the plain scan in 8 cases,which showed obvious enhancement at the arterial phase scan,and the highest CT value was 100 Hu.The mass showed continuous homogeneous enhancement at parenchymal phase scan.The 8 cases were diagnosed as malignant renal tumor before surgery.The other one case showed low density dots at the plain scan and no enhancement at the arterial phase scan.The relevant articles were searched in Pubmed and CBM databases and clinical data were reviewed.Results All patients had undergone operation.One patient had radical nephrectomy for his own reason and the other 8 patients had undergone nephron sparing surgery.Of the 8 cases,6 received laparoscopic partial nephrectomy and 2 open partial nephrectomy.Pathological tests confirmed renal angiomyolipoma.During the follow-up of 9 to 36 months,no tumor recurrence was found.Conclusions Renal angiomyolipoma with less adipose tissue is a rare disease,which can be diagnosed with subtle analysis of CT scan.Nephron sparing surgery should be taken for renal angiomyolipoma with less adipose tissue,especially for the small volume of tumor,so that excessive treatment of nephrectomy can be avoided.