Journal Article

Management of Cushing disease

Swearingen, Brooke, Biller, Beverly M. K., Tritos, Nicholas A.

2011

Overview

Transsphenoidal surgery leads to remission in 70–90% of patients with Cushing disease. However, the risk of recurrence could reach 20–25% at 10 years after surgery. This Review summarizes data on the efficacy and safety of established therapies for patients with Cushing disease and highlights agents that are being investigated as possible future therapies for patients with this condition. 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. Key Points Transsphenoidal surgery is advisable for most patients with newly diagnosed Cushing disease and often leads to rapid and sustained remission of hypercortisolism with low morbidity in most cases Pituitary reoperation might be considered in patients with persistent or recurrent Cushing disease, but the remission rate is lower in comparison to initial surgery Pituitary gland radiation therapy might lead to remission in many patients with persistent or recurrent Cushing disease, but takes a considerable time and a lifelong risk of hypopituitarism exists Medical therapy can be used in patients who are awaiting the therapeutic effects of pituitary gland radiation therapy or in very ill patients in preparation for surgery Bilateral adrenalectomy could be considered in patients with persistent or recurrent Cushing disease to control hypercortisolism, but will induce adrenal insufficiency and carries the risk of Nelson syndrome

• Publisher: Nature Publishing Group UK,Nature Publishing Group
• Subject: 692/699/2743/2742/1738 / 692/700/565/485 / 692/700/565/545 / Adrenalectomy / Care and treatment / Cushing Syndrome / Endocrinology / Health aspects / Humans / Medical education / Medicine / Medicine & Public Health / Patients / Pituitary ACTH Hypersecretion - complications / Pituitary ACTH Hypersecretion - drug therapy / Pituitary ACTH Hypersecretion - radiotherapy / Pituitary ACTH Hypersecretion - surgery / Pituitary gland / Radiation therapy / Radiotherapy / Remission (Medicine) / review-article / Risk factors / Surgeons / Surgery