POMC and Cowden syndrome 1: The Endocrine Society guidelines report a prevalence of hirsutism of 48% in CS [10], while in the ERCUSYN European Registry hirsutism was found in 93% of women with ectopic ACTH CS and its prevalence was significantly higher compared with that in the other groups of CS (63% in the CD and 37% in patients with adrenal CS) [17].