[Non-clear cell renal cell carcinoma]

Non-clear cell renal cell carcinomas (nccRCC) account for approximately 20-25% of renal cell carcinomas. Approximately 20 different histologically and/or molecularly defined entities are subsumed under nccRCC. Many entities only have a share of 1% or less. Due to the rarity of the subpopulations, there are currently no larger randomized studies and there is a some uncertainty regarding the optimal treatment recommendations. The previous treatment recommendations for localized and advanced nccRCC are presented. Data from the various systemic therapies are presented. For nccRCC, there are no different entity-related therapy guidelines due to a lack of data and especially randomized studies. The tumors should be treated in the same way as clear cell renal cell carcinomas. Monotherapies with a tyrosine kinase inhibitor (TKI; in chromophobe RCC also with an mTOR inhibitor) or with combination therapy ICI/ICI or TKI/ICI (ICI: immune checkpoint inhibitor) can be used, depending on the risk factors and individual decision. Randomized trials are urgently needed.