Mutations (germline) in SDH subunits have been linked to familial paraganglioma syndromes, pheochromocytomas (PGL/PCC), renal cell carcinomas (RCC), and gastrointestinal stromal tumors (GISTs) [14], both as predisposing and prognostic factors [13]. The gene discussed is SDHB; the disease is gastrointestinal stromal tumor.