Table 2: Correlations between gene mutations and clinical phenotype.

Syndrome Gene PCCs (%) Sympathetic PGL Parasympathetic PGL Bilateral/multifocal neoplasia Malignancy (%) MEN 2A RET ~50 Very rare Extremely rare + <3 MEN 2B RET ~50 Very rare Extremely rare + <3 VHL VHL 10–20 + Rare + 5 NF1 NF1 5 − − − 11 PGL1 SDHD + + + + ~5 PGL2 SDHAF2 − − + + Not known PGL3 SDHC − Rare + − Not known PGL4 SDHB Rare + Rare + ~40 PGL5 SDHA − + − Not known Not known TMEM127 mutation carriers TMEM127 100 − − + ~5 MAX mutation carriers MAX 100 + Extremely rare + ~10

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