POMC and Cowden syndrome 1: Among all patients with ACTH-dependent CS (n = 26 with values at last visit), the median (min, max) change from baseline to last observed visit was 3.7 (-15.6, 20.0) pmol/L [16.8 (-70.9, 90.9) pg/mL] (p = 0.003) for plasma ACTH; 4.0 (-1650.5, 1404.8) nmol/d (1.4 [-598.0, 509.0] μg/24 h) (p = 0.873) for 24-h UFC; -0.33 (-30.6, 47.7) nmol/L (-0.01 [-1.1, 1.7] μg/dL) (p = 0.815) for LNSC; and 22.0 (-127.0, 339.0) nmol/L (0.8 [-4.6, 12.3] μg/dL) (p = 0.239) for serum cortisol.