Germline mutations, resulting in a decrease in or loss of expression of succinate dehydrogenase (SDH), fumarate hydratase (FH), and isocitrate dehydrogenase have been reported in inherited paragangliomas, gastrointestinal stromal tumors, pheochromocytomas, myomas, SDH, papillary renal cell cancer (FH), and gliomas (46). The gene discussed is FH; the disease is gastrointestinal stromal tumor.