Heterozygous mutations in the fumarate hydratase (FH) cause FH tumor predisposition syndrome, an autosomal dominant condition with increased risk for uterine and cutaneous leiomyomata, renal tumors, and a lower risk of pheochromocytomas and paragangliomas (Kamihara et al. 1993). Here, FH is linked to hereditary pheochromocytoma-paraganglioma.