Recent progress in non-clear cell renal cell carcinoma: biology and therapeutic strategies.

Non-clear cell renal cell carcinomas (nccRCC) comprise a heterogeneous group of rare malignancies, accounting for approximately 20% of all kidney cancers. Given the rarity of these diverse subsets of RCC, the treatment paradigm for nccRCC is often based on treatment strategies utilized in the management of clear cell renal cell carcinoma (ccRCC), which may not fully address the distinct molecular and genetic drivers unique to nccRCC tumors. However, recent advances in the molecular characterization of nccRCC have led to the identification of new therapeutic targets, resulting in the development of more targeted therapies for nccRCC treatment. Furthermore, the role of molecular characterization of renal tumors is emphasized in the 2022 World Health Organization reclassification of genitourinary tumors, given that delineation of renal tumor subtypes now also relies on genetic markers. In order to highlight this evolving treatment landscape, our review provides a comprehensive summary of recent progress related to the biology and management of nccRCC subtypes.