Patients with CS from ectopic ACTH production were older (p=0.031), had lower phosphate concentration at time of diagnosis (p=0.004), were more likely to have hypophosphatemia (p=0.023), had higher xULN of 24h UFC (p<0.001) and had lower potassium concentrations (p<0.001) than patients with CS from ACTH producing pituitary adenomas. Here, POMC is linked to Cowden syndrome 1.