Indeed, it was shown that mutations in SDHA account for around 30% of all KIT/PDGFRA-WT GIST (10), while being responsible for <1% of all pheochromocytoma and paraganglioma cases (PPGL), as opposed for example to SDHB that accounts for about 10% of all PPGL (22, 23). Here, SDHB is linked to gastrointestinal stromal tumor.