Cushing disease is caused by a corticotroph
tumor of the pituitary gland. Patients with
Cushing disease are usually treated with transsphenoidal surgery, as this approach leads to remission in 70-90% of cases and is associated with low morbidity when performed by experienced pituitary gland surgeons. Nonetheless, among patients in postoperative remission, the risk of recurrence of
Cushing disease could reach 20-25%
at 10 years after surgery. Patients with persistent or recurrent
Cushing disease might, therefore, benefit from a second pituitary operation (which leads to remission in 50-70% of cases),
radiation therapy to the pituitary gland or bilateral
adrenalectomy. Remission after
radiation therapy occurs in ∼85% of patients with
Cushing disease after a considerable latency period. Interim medical
therapy is generally advisable after patients receive
radiation therapy because of the long latency period. Bilateral
adrenalectomy might be considered in patients who do not improve following transsphenoidal surgery, particularly patients who are very ill and require rapid control of
hypercortisolism, or those wishing to avoid the risk of
hypopituitarism associated with
radiation therapy. Adrenalectomized patients require lifelong adrenal
hormone replacement and are at risk of
Nelson syndrome. The development of medical
therapies with improved efficacy might influence the management of this challenging condition.