对马蹄肾患者进行肾细胞癌 (RCC) 手术面临着独特的解剖学挑战。 IVC 肿瘤血栓扩展和转移性疾病的存在进一步加剧了临床决策和技术挑战的复杂性。我们遇到了一名患有马蹄肾的 66 岁女性的独特病例，她在医院就诊时出现了 14 厘米的肿块。她的右肾 II 级肿瘤血栓延伸至下腔静脉、腹膜后淋巴结肿大和肺结节，活检证明与转移性透明细胞肾细胞癌 (cT3aN1M1) 一致。她预先接受了免疫检查点抑制（ICI）和靶向治疗的联合治疗。鉴于她对全身治疗的持久反应，我们选择对残余 ypT3aN0 疾病进行巩固性细胞减灭术肾切除术，此后她在放射学检查中一直保持无病状态。使用术中获得的分步说明性照片，我们详细介绍了我们的手术方法，并重点介绍了处理马蹄肾引起的晚期肾细胞癌类似病例的技术要点。我们介绍了一个马蹄肾引起的伴有 IVC 肿瘤血栓的转移性肾细胞癌的复杂病例。我们分享我们的临床和技术方法来克服她的病例固有的医疗和手术挑战。相对于 ICI 给药，减瘤性肾切除术的最佳作用和时机根据肿瘤和患者相关因素不断发展。事实上，我们赞成采用多学科方法来优化患者的治疗结果。

Performing surgery for renal cell carcinoma (RCC) in patients with horseshoe kidneys presents unique anatomic challenges. The complexities of clinical decision-making and technical challenges are further compounded by the presence of both IVC tumor thrombus extension and metastatic disease.We encountered the unique case of a 66-year-old woman with a horseshoe kidney who presented with a 14cm mass in her right kidney with level II tumor thrombus extension into the IVC, retroperitoneal lymphadenopathy, and pulmonary nodules that were biopsy-proven to be consistent with metastatic clear cell RCC (cT3aN1M1). She was treated with combination immune checkpoint inhibition (ICI) and targeted therapy upfront. Given her durable response to systemic therapy, we elected to perform a consolidative cytoreductive nephrectomy for residual ypT3aN0 disease, and she has since remained disease-free radiographically. Using step-by-step illustrative photographs obtained intraoperatively, we detail our surgical approach and highlight technical pearls to manage similar cases of advanced RCC arising from horseshoe kidneys.We present a complex case of metastatic RCC with IVC tumor thrombus arising from a horseshoe kidney. We share our clinical and technical approach to overcoming the medical and surgical challenges inherent to her case. The optimal role and timing of cytoreductive nephrectomy relative to ICI administration continue to evolve based on both tumor- and patient-related factors. Indeed, we favor a multidisciplinary approach to optimize patient outcomes.