VHL and hereditary pheochromocytoma-paraganglioma: It also has potential implications for the phenotype of “pseudohypoxic” pheochromocytoma, which typically manifests upregulation of genes in the HIF pathway and is associated with mutations in HIF-2α, PHD2, PHD1, VHL, and specific subunits of tricarboxylic acid cycle enzymes succinate dehydrogenase SDHx.