These data on overall HIF-2α protein expression in PPGL as one tumor entity, in combination with our results on the dependence of cytoplasmic HIF-2α in the different tumor subtypes, suggest an important tumor-driving role of HIF-2α in these tumors. sPGLs and neuroblastomas arise along the sympathetic chain ganglia and share a common ancestor cell in the trunk neural crest. This evidence concerns the gene EPAS1 and neoplasm.