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). This evidence concerns the gene FH and paraganglioma.