Despite this well-known inherited basis of PCCs and paragangliomas (PGLs), during the past decades somatic mutations with variable frequency in many genes, including EPAS1 (HIF2α), RET, VHL, RAS, NF1, ATRX, and CSDE1 recurrent somatic copy number alterations and several fusion genes, involving MAML3, BRAF, NGFR, and NF1, have been progressively identified [5–16]. Here, EPAS1 is linked to paraganglioma.