Mutations in components of the succinate dehydrogenase complex in paraganglioma and pheochromocytoma; mutations in fumarate hydratase (FH) in renal cell carcinoma; and mutations in isocitrate dehydrogenase-1 and −2 (IDH1 and IDH2) that occur in a significant fraction of gliomas, acute myeloid leukemias and other forms of cancer. The gene discussed is IDH1; the disease is central nervous system cancer.