Table 2: Correlations between gene mutations and clinical phenotype.

SyndromeGenePCCs (%)Sympathetic PGLParasympathetic PGLBilateral/multifocal neoplasiaMalignancy (%)

MEN 2ARET~50Very rareExtremely rare+<3
MEN 2BRET~50Very rareExtremely rare+<3
VHLVHL10–20+Rare+5
NF1NF1511
PGL1SDHD++++~5
PGL2SDHAF2++Not known
PGL3SDHCRare+Not known
PGL4SDHBRare+Rare +~40
PGL5SDHA+Not knownNot known
TMEM127 mutation carriersTMEM127100 + ~5
MAX mutation carriersMAX100+Extremely rare + ~10

PCCs: pheochromocytomas; PGL: paragangliomas; +: present; −: absent.