While there is less clinical evidence of HIF2α driving hemangioma, relative to PPGL, liver hemangiomas associated with loss-of-pVHL mouse models can be rescued by co-deletion of HIF2α or HIFβ, which provides further evidence for the gatekeeping role of HIF2α in hemangioma and hemangioblastoma (Rankin et al., 2008; Rankin et al., 2005). Here, EPAS1 is linked to hemangioblastoma.