Mutations of the Krebs-cycle genes that encode fumarate hydratase (FH) and succinate dehydrogenase (SDH) are associated with cancer predisposition syndromes, including HLRCC and SDH PGL/PCC, both characterized by the propensity to develop aggressive tumors, including RCC [1, 2]. This evidence concerns the gene SDHB and hereditary leiomyomatosis and renal cell cancer.