Advances in non-clear cell renal cell carcinoma management: From heterogeneous biology to treatment options

Non-clear cell renal cell carcinoma (nccRCC), which makes up nearly one quarter of all RCC subtypes, commonly impacts younger patients, and is often metastatic at presentation, typically has a worse prognosis in the metastatic setting compared to clear-cell RCC, with overall survival durations that are ~10 months shorter. Most clinical trials have either excluded or only included small numbers of patients with nccRCC, creating a dearth of data on response rates and survival outcomes. Researchers reviewed the use and outcomes of cytotoxic chemotherapy, various combination modalities of tyrosine kinase inhibitors and immune checkpoint inhibitors, and targeted agents, and discussed active ongoing clinical trials for patients with nccRCC and future directions in the treatment of this rare disease.