Belzutifan, a potent and selective small-molecule inhibitor of HIF2α analog of PT2385, has proven to be of remarkable therapeutic benefit for patients with ccRCC and the Pacak-Zhuang syndrome caused by loss-of-function mutations in VHL or gain-of-function mutations in EPAS1, respectively [25,26]. Here, EPAS1 is linked to nonpapillary renal cell carcinoma.