Non-clear-cell renal cell carcinomas (nccRCC) represent around 25% of all renal cancers and are a very heterogeneous group of tumours in terms of both biological features and prognosis. Papillary renal cell carcinomas (pRCC) are the most frequent subtype with 15% to 20% of all kidney cancers. Improved biological knowledge of these tumours has led to better identification of each subtype. Among pRCC, some exhibit mutations of the MET oncogene and others mutations of the gene coding for fumarate hydratase. The management of nccRCC, in particular the pRCC subtype, has evolved considerably in recent times, spearheaded by the advent of targeted therapies including anti-angiogenics but also new immunotherapy agents. Several studies have in the last few years prompted a new standard of care for these nccRCC. We propose to present throughout this article the latest available efficacy data on different compounds assessed in the treatment of the most frequent nccRCC, including the pRCC, chromophobe carcinoma, collecting duct carcinoma, MiT family translocation renal cell carcinoma and renal medullary carcinoma subtypes.
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