Recurrent CS is associated with positive pituitary uptake of 68Ga-DOTA-TATE. Although in these cases it would not be possible to distinguish pathological from physiological uptake, positive 68Ga-DOTA-TATE is useful as it indicates the presence of functioning pituitary tissue. The absence of pituitary uptake in patients with recurrent CS suggests an ectopic ACTH source. The gene discussed is POMC; the disease is Cowden syndrome 1.