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.
aDepartment of Medicine, Oregon Health and Science University, Portland, Oregon, USA
bSwedish Neuroscience Institute, Seattle, Washington, USA
Correspondence to Maria Fleseriu, MD, Division of Endocrinology, Diabetes and Clinical Nutrition, Oregon Health and Science University, BTE472, Portland, OR 97239, USA Tel: +1 503 494 9060; fax: +1 503 494 3375; e-mail: email@example.com