一名69岁女性因镜下血尿，经计算机断层扫描（CT）检查发现左肾肿瘤，被转诊至我院治疗。增强CT显示左肾下极有一个5厘米的肿瘤。怀疑为左肾细胞癌 (RCC) (cT1bN0M0)。此外，左肾静脉和性腺静脉在动脉期扩张和强化；怀疑肾动静脉瘘（RAVF）。此外，有多处局灶性动脉扩张，提示存在多发性血管畸形。遗传性主动脉疾病，包括血管性埃勒斯-当洛斯综合征（vEDS），是一个令人担忧的问题。一般来说，由于血管脆性，不建议 vEDS 患者进行手术。因此，对遗传性主动脉疾病（包括 vEDS）的基因进行了面板分析；未发现包括 COL3A1 在内的候选基因中的致病变异。在与患者详细讨论后，她在左肾动脉经导管动脉栓塞（TAE）后接受了左肾切除术。我们准备了用于主动脉闭塞的球囊导管，作为大出血的预防措施；事实并非如此，因为只发生了少量的术中出血。因此，在不使用球囊导管的情况下成功地进行了肾切除术。患者恢复顺利，第8天出院。病理检查显示透明细胞肾细胞癌（pT1a）和肿瘤附近的RAVF。在此，我们报告了这例伴有 RAVF 和多发性动脉畸形的左侧肾细胞癌病例，通过基因检测评估术前风险，随后进行肾动脉 TAE 和开放性肾切除术，成功治疗了该病例。

A 69-year-old woman was referred to our hospital for the treatment of a left renal tumor found by computed tomography (CT) during examination for microscopic hematuria. Contrast-enhanced CT showed a 5 cm tumor in the inferior pole of the left kidney. Left renal cell carcinoma (RCC) (cT1bN0M0) was suspected. In addition, the left renal and gonadal veins were dilated and enhanced in an arterial phase; renal arteriovenous fistula (RAVF) was suspected. Moreover, there were multiple focal arterial dilatations, suggesting the presence of multiple vascular malformation. Hereditary aortic disease, including vascular Ehlers-Danlos syndrome (vEDS), was a concern. In general, surgery is not recommended for patients with vEDS, due to vascular fragility. As such, a panel analysis of genes for hereditary aortic diseases, including vEDS, was performed; no pathogenic variants in candidate genes including COL3A1 were identified. After detailed discussions with the patient, she underwent a left nephrectomy, following transcatheter arterial embolization (TAE) of the left renal artery. We prepared a balloon catheter for aortic occlusion as a preventative measure for massive bleeding; this was not the case, as only a small amount of intraoperative bleeding occurred. Thus, the nephrectomy was performed successfully without using the balloon catheter. The patient recovered uneventfully and was discharged on day 8. Pathological examination showed clear-cell RCC (pT1a) and a RAVF near the tumor. Herein we report this case of left RCC with RAVF and multiple arterial malformation, which was successfully managed by evaluating preoperative risks with a genetic test, followed by TAE of the renal artery and open nephrectomy.