POMC and Cowden syndrome 1: Considering second-line tests for the diagnosis of ACTH-dependent CS, in 13 out of 14 cases the response of ACTH or cortisol to CRH test was absent, 11 out of 13 patients did not achieve sufficient cortisol suppression after 8-mg DST and their urinary cortisol to cortisone ratio was increased in 8 out of 11 patients.