Inactivating mutations in SDH subunits have been identified in both genetic and sporadic cancer types, including familial paraganglioma/pheochromocytoma (PGL/PCC) (115), renal carcinoma, thyroid cancer, neuroblastoma, gastrointestinal stromal tumor, ovarian cancer and testicular seminoma (116), whereas FH loss-of-function hallmarks hereditary leiomyomatosis and renal cell cancer (HLRCC) and skin and uterine leiomyomas (22). Here, FH is linked to hereditary leiomyomatosis and renal cell cancer.