肾细胞癌（RCC）是最致命的泌尿生殖系统癌症之一，死亡率最高，并且在其整个发展过程中可能未被发现。 RCC 可以是散发性的或遗传性的。探索肾细胞癌潜在的遗传异常对于了解非遗传性肾癌的起源具有重要意义。 RCC 的治疗经历了几个世纪的发展，从细胞因子时代到靶向治疗再到免疫治疗。手术治疗是主要的治疗方式，特别是对于器官局限性疾病。此外，当与介入放射科医生一起偶然发现小的肾脏肿块时，泌尿肿瘤学界重点关注保留肾单位的手术方法和消融手术。除了批准用于肾细胞癌治疗的新联合疗法外，还进行了多项试验来研究某些药物的潜在益处。这可能会为转移性肾细胞癌 (mRCC) 患者带来持久的缓解和更长的生存获益。几种已批准的药物通过靶向 VEGF 信号传导和 mTOR 降低了 RCC 患者的死亡率。这篇综述更好地解释了 RCC 进展中涉及的信号通路、肿瘤代谢物和 RCC 中可用于诊断的重要生物标志物。此外，它还概述了 RCC 致癌的特征，以帮助对抗治疗耐药性，以及有关当前管理和未来治疗方案的详细信息。未来，将提供多模式和综合护理，随着我们对这种疾病的了解越来越多，新的治疗方案也会出现。© 2023。作者获得 Springer Science Business Media, LLC（Springer Nature 的一部分）的独家许可。

Renal cell carcinoma (RCC) is one of the most lethal genitourinary cancers, with the highest mortality rate, and may remain undetected throughout its development. RCC can be sporadic or hereditary. Exploring the underlying genetic abnormalities in RCC will have important implications for understanding the origins of nonhereditary renal cancers. The treatment of RCC has evolved over centuries from the era of cytokines to targeted therapy to immunotherapy. A surgical cure is the primary treatment modality, especially for organ-confined diseases. Furthermore, the urologic oncology community focuses on nephron-sparing surgical approaches and ablative procedures when small renal masses are detected incidentally in conjunction with interventional radiologists. In addition to new combination therapies approved for RCC treatment, several trials have been conducted to investigate the potential benefits of certain drugs. This may lead to durable responses and more extended survival benefits for patients with metastatic RCC (mRCC). Several approved drugs have reduced the mortality rate of patients with RCC by targeting VEGF signaling and mTOR. This review better explains the signaling pathways involved in the RCC progression, oncometabolites, and essential biomarkers in RCC that can be used for its diagnosis. Further, it provides an overview of the characteristics of RCC carcinogenesis to assist in combating treatment resistance, as well as details about the current management and future therapeutic options. In the future, multimodal and integrated care will be available, with new treatment options emerging as we learn more about the disease.© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.