This theory would explain the observed 30% partial response rate of Belzutifan in VHL-associated hemangioblastomas [42], while this drug and PT2385, a first-generation HIF-2α inhibitor [57], demonstrated clinical efficacy in VHL patients with renal cell carcinoma (RCC) [40,41,62]. This evidence concerns the gene EPAS1 and hemangioblastoma.