Cushing Disease Is Not Necessarily a Progressive and Fatal Disease

Patients with Cushing disease are thought to have progressive and unremitting symptoms if left untreated. We report a patient with documented Cushing disease that was untreated for 26 years. A 50-year-old woman presented with a medical history consistent with longstanding Cushing syndrome. A dexamethasone suppression test done 26 years before our evaluation confirmed Cushing syndrome. She was lost to follow up and remained untreated over a 26-year span. During this period, she had episodes of fatigue, weight gain, menstrual irregularity, and hypertension. These episodes were punctuated with periods of transient cessation of her symptoms. She presented to us after worsening of symptoms. Cushing syndrome was suspected and confirmed by elevated urinary-free cortisol and 17-hydroxycorticosteroid. Pituitary magnetic resonance imaging revealed a 4-mm adenoma. A pituitary tumor was identified at transphenoidal surgery, and the patient became hypocortisolemic postoperatively. Cushing disease has generally been thought to be a progressive and unremitting disease. This case illustrates that, in contrast to our current thinking, Cushing disease is not necessarily a progressive disease.