Updated results from an open label phase 2 study investigating belzutifan (MK-6482), an oral HIF-2α inhibitor, for VHL disease-associated ccRCC (NCT03401788) has revealed an impressive ORR of 49.2%, with 88.5% of patients still in the study at 20.2 months follow-up [47]. This evidence concerns the gene EPAS1 and nonpapillary renal cell carcinoma.