Inactivating mutations in TCA cycle enzymes fumarate hydratase (FH) and succinate dehydrogenase (SDH) are driver mutations in a subset of human cancers and they mediate epigenetic reprogramming.83 SDH mutations are present in gastrointestinal stromal tumors (GISTs), renal cell carcinoma, paraganglioma and pheochromocytoma. This evidence concerns the gene SDHB and renal cell carcinoma.