Long-term Treatment With Metyrapone in a Man With Ectopic Cushing Syndrome

This is a unique case of ectopic adrenocorticotropic hormone (ACTH)-secreting mediastinal neuroendocrine tumor, presumably thymic in origin, with suspected lung metastasis in a 61-year-old man who was successfully managed with long-term metyrapone alone. He presented with severe hypokalemia and hypertension, complicated with psychosis and vertebral collapse. He survived through a complicated course of pulseless ventricular tachycardia arrest and a severe Cushing storm due to drug nonadherence. For 3 years since diagnosis, he remained stable on metyrapone, and was able to achieve biochemical eucortisolism, with normalization of ACTH and cortisol levels. In addition, his tumor was reduced in size and the suspicious lung metastasis regressed.