Second-line treatment for Cushing's disease when initial pituitary surgery is unsuccessful

Abstract

Purpose of review: Adenectomy via transsphenoidal surgery is considered the treatment of choice for Cushing's disease. It is successful in about 80% of patients in the hands of an experienced surgeon. When transsphenoidal surgery fails or is contraindicated, a second-line treatment must be chosen. The review focuses on second-line treatment options.

Recent findings: Repeat pituitary surgery results in the cure of Cushing's disease in about 50% of cases. Bilateral adrenalectomy results in resolution of hypercortisolemia in almost all patients, but leaves the patient glucocorticoid and mineralocorticoid deficient. Nelson's syndrome, depending on the definition, occurs in up to 35% of these patients. Irradiation of the residual pituitary tumor typically takes several years before the full effect is realized; it can cause panhypopituitarism. Finally, pharmacologic treatment of persistent hypercortisolemia can be effective, but is often associated with untoward side effects. These side effects are a powerful deterrent to its use. Several new pharmacologic agents are being studied and show some promise.

Summary: Each of the second-line treatments for Cushing's disease currently available can be effective at treating hypercortisolism, but each has significant limitations. New pharmacologic agents may soon offer some very exciting treatment options.