Abstract: Non-clear cell renal cell carcinomas(nccRCC) represent a heterogeneous group of diseases. Once metastatic, nccRCC histologies are generally characterized by resistance to traditional systemic therapies, and survival rates are low. Recent research showed that some therapies can treat nccRCC effectively.This article reviews the current therapies approved for nccRCC, such as such as vascular endothelial growth factor receptor tyrosine kinase inhibitors, mTOR inhibitors, MET inhibitors, cytotoxic drugs and immune checkpoint inhibitors, so to ultimately, enhanced the understanding of the optimal strategies for each subtype in nccRCC.

Key words: non-clear cell renal cell carcinoma, metastatic carcinoma, target therapy, chemotherapy , Immunotherapy