VHL and nonpapillary renal cell carcinoma: The discovery that carcinogenesis is driven by HIF-2α has recently led to the development of a clinical HIF-2α antagonist (belzutifan) for the treatment of VHL-associated ccRCC, CNS-Hbl and pNET (Jonasch et al. 2021) and advanced sporadic ccRCC (Jonasch et al. 2024).