Oncol Res Treat. 2019;42(3):128-135. doi: 10.1159/000495366. Epub 2019 Feb 23.
Non-clear cell renal cell carcinomas (nccRCC) are rare diseases with heterogeneous histopathologically and genetically defined entities. The clinical data on optimal systemic treatments of nccRCC is rather limited. In this review, the current World Health Organization (WHO) classification of nccRCC based on histopathologic and genetic findings is reported. Regarding systemic treatment options, the most commonly used agents are mTOR inhibitors like everolimus or temsirolimus, or tyrosine kinase inhibitors like sunitinib. 2 small randomized clinical trials with nccRCC comparing sunitinib with everolimus revealed a trend towards a better progression-free survival (PFS) and overall survival (OS) in favor of sunitinib. In RCC with predominant sarcomatoid features, both chemotherapy and targeted agents are reported without any preference for outcome. For subsequent lines of therapy, some case reports describe promising effects of PD-1 or PD-L1 inhibitors in nccRCC including sarcomatoid subtype and Bellini duct carcinoma. Currently, nccRCCs are treated similarly to clear cell RCC or, whenever possible, within clinical trials. Clinical trials with immune checkpoint inhibitors are ongoing.
非透明细胞肾细胞癌（nccRCC）是一种罕见疾病，具有组织病理学和遗传学定义的异质性实体。nccRCC 的最佳系统治疗的临床数据相当有限。在这篇综述中，报告了基于组织病理学和遗传学发现的当前世界卫生组织（WHO）nccRCC 分类。关于系统治疗选择，最常用的药物是 mTOR 抑制剂，如依维莫司或替西罗莫司，或酪氨酸激酶抑制剂，如舒尼替尼。两项比较舒尼替尼与依维莫司的 nccRCC 的小型随机临床试验显示，舒尼替尼在无进展生存期（PFS）和总生存期（OS）方面具有更好的趋势。在以肉瘤样特征为主的 RCC 中，报道了化疗和靶向药物均无任何优势。对于后续治疗线，一些病例报告描述了 PD-1 或 PD-L1 抑制剂在 nccRCC 中的有希望的效果，包括肉瘤样亚型和 Bellini 导管癌。目前，nccRCC 的治疗与透明细胞 RCC 相似，或者在可能的情况下在临床试验中进行。免疫检查点抑制剂的临床试验正在进行中。
