The germline or somatic alteration in nuclear mitochondrial complex II genes encoding SDH subunits (SDHB, C, and D) were found associated with pheochromocytomas and paragangliomas (Gimm et al., 2000; van Nederveen et al., 2007; Bardella et al., 2011). Here, SDHB is linked to hereditary pheochromocytoma-paraganglioma.