EPAS1 and hereditary pheochromocytoma-paraganglioma: Abnormal HIF2α stabilization, through HIF2α gain-of-function or VHL loss-of-function mutations [17], has been found in pheochromocytomas and paragangliomas [18–20], as well as loss of VHL in clear cell renal carcinoma (for review see [21, 22]).