SDHB and adrenal gland pheochromocytoma: On encountering a patient with PCC/PGL and a rare missense variant in SDHx, evidence of pathogenicity could be inferred from (1) the very observation in an individual with the relevant rare PCC/PGL phenotype of a rare variant in an associated gene (PP2 in the ACMG/AMP framework), (2) location of that variant within a sub-region of the gene particularly associated with pathogenicity (PM1), and (3) subphenotypic features particularly associated with PVs in the SDHx genes, eg, invasive disease or loss of SDHB staining on immunohistochemistry (IHC) (PP4).