Abstract
Patients with non-clear cell renal cell carcinoma (nccRCC) comprise approximately 25% of all patients with primary renal cell cancer. The standard systemic treatment for nccRCC is not yet established because patients with this diagnosis have been excluded from most clinical trials and nccRCC are a very heterogeneous category that include many clinically and molecularly distinct diseases.
The most common aberration is an activating mutation or amplification of MET, which is found in many papillary renal cell carcinomas. A comparative phase II study of tyrosine kinase inhibitors showed that cabozantinib is currently the most effective nccRCC monotherapy.
Many other studies are underway, and the future is probably in the combination of immunotherapy with targeted treatment.