The first-in-class HIF2-α inhibitor—PT2385—and the more potent second-generation variant, PT2977, are both in phase 2 clinical trials [HIF-2 Alpha Inhibitor PT2385 in Treating Patients With Recurrent Glioblastoma (PT2385), NCT03216499; A Trial of PT2977 in Combination With Cabozantinib in Patients With Clear Cell Renal Cell Carcinoma (ccRCC), NCT03634540]. This evidence concerns the gene EPAS1 and nonpapillary renal cell carcinoma.