In addition to the well-known cancer syndromes caused by the RET, VHL or NF1 genes that include pheochromocytomas, and the SDH genes (SDHA, SDHB, SDHC, SDHD, SDHAF2) that are primarily associated with PPGL, recently identified suspected or confirmed PPGL-associated genes include HRAS, EPAS1 [HIF2A], FH, MDH2, IDH1, IDH2, DLST, SLC25A11, GOT2, SUCGL2, TMEM127 and MAX. The gene discussed is EPAS1; the disease is hereditary pheochromocytoma-paraganglioma.