Update on Hypercortisolism Therapy

Abstract

Treating Cushing's syndrome is very challenging and should be tailored to the patient. Surgery is considered the gold standard treatment for both pituitary adrenocorticotropic hormone (ACTH)-secreting adenomas, ectopic ACTH-secreting tumors and adrenal tumors, as the chance to be successful is high, especially for adrenal tumors, when performed in specialized centers by expert surgeons. Pituitary radiotherapy represents a second-line treatment in patients not cured with surgery, or when medical treatment is not suitable/efficacious, although the rate of cure is largely variable and achieved only in the long term, and is associated with the risk of developing secondary hypopituitarism. Several drugs, acting at the pituitary, adrenal or peripheral tissue level, are available. Medical treatment is indicated as second-line therapy for patients unsuccessfully treated with pituitary surgery, or in those awaiting radiotherapy to become effective, or prior to adrenalectomy, and as the first-line approach to prepare patients for surgery, especially those with severe comorbidities, or in those not suitable/refusing surgery. The success rate of medical therapy is variable, depending on the cause and severity of hypercortisolism, and is often associated with important side effects.