CYP11B1 and Cowden syndrome 1: Aminoglutethimide, an inhibitor of cholesterol side-chain cleavage, 11-beta-hydroxylase (CYP11B1) and 18-hydroxylase (CYP11B2) [35], and trilostane, an inhibitor of 17-beta-hydroxysteroid dehydrogenase, 3-beta-hydroxysteroid dehydrogenase (3-beta-HSD) and 17-alpha-hydroxylase/17,20-lyase (CYP17) [36], have been used in the past but only occasionally in more recent times in the treatment of CS, because of their unsatisfactory pharmacological profile.