IDH2 and hereditary leiomyomatosis and renal cell cancer: Oncometabolites hinder DNA repair Recently, two studies by Sulkowsk et al. suggested that IDH, FH, or SDH mutation-induced accumulation of 2-HG, fumarate, or succinate suppresses the homologous recombination (HR) DNA repair pathway in gliomas and AML with mutant IDH, hereditary leiomyomatosis and renal cell cancer (HLRCC), and succinate dehydrogenase-related hereditary paraganglioma and pheochromocytoma (SDH PGL/PCC) (Sulkowski et al., 2017; Sulkowski et al., 2018).