Numerous missense mutations in the C-terminal prolyl-hydroxylation site in the oxygen-dependent degradation domain of HIF2α have been reported to cause polycythemia, pheochromocytoma, paraganglioma, somatostatinoma, hemangioma, and in one case hemangioblastoma (Taieb et al., 2016; Tarade et al., 2018). This evidence concerns the gene EPAS1 and hereditary pheochromocytoma-paraganglioma.