In this case series study of 68 patients with Cushing syndrome, ectopic adrenocorticotropic hormone–secreting carcinoid tumors with CS were associated with increased metastasis to lymph nodes, higher recurrence, and lower overall disease-free survival at 5 and 10years compared with quiescent bronchial carcinoid tumors, irrespective of histologic subtype, with no difference in outcome based on surgical approach. This evidence concerns the gene POMC and Cowden syndrome 1.