Controls presented an increased percentage of classical CD14++CD16− monocytes (p = 0.082), whereas CD14++CD16+ intermediate monocytes (p = 0.047) were higher in the CS group, both in the ACTH-dependent and ACTH-independent forms, with the highest atypical CD14+CD16++ monocytes in patients with ACTH-independent CS (p = 0.019). This evidence concerns the gene POMC and Cowden syndrome 1.