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Primary therapy of Cushing's disease: Transsphenoidal surgery and pituitary irradiation

Lynnette K Nieman, MD
Section Editor
André Lacroix, MD
Deputy Editor
Kathryn A Martin, MD


Cushing's disease is caused by pituitary corticotropin (ACTH)-secreting tumors. These tumors are almost always benign and are usually microadenomas (ie, <10 mm in diameter). In 30 to 40 percent, the microadenoma is so small that it is not detectable by magnetic resonance imaging (MRI), while in 10 to 15 percent a macroadenoma is present. Treatment is aimed first at the anterior pituitary gland.

The progressive stages of treatment that may be required to cure a patient of Cushing's disease are shown in the figure (algorithm 1). Primary therapy consists of transsphenoidal surgery or pituitary irradiation. Patients who fail this first approach can be treated either by repeat transsphenoidal surgery, medical therapy, radiotherapy, or, as a final resort, surgical or medical adrenalectomy. An overview of transsphenoidal surgery and pituitary irradiation for the primary therapy of Cushing's disease will be reviewed here. An overview of the management of Cushing's syndrome, as well as medical therapy and surgical adrenalectomy for Cushing's are reviewed separately. (See "Overview of the treatment of Cushing's syndrome" and "Medical therapy of hypercortisolism (Cushing's syndrome)" and "Persistent or recurrent Cushing's disease: Surgical adrenalectomy".)


Transsphenoidal microadenomectomy is currently the treatment of choice for Cushing's disease. When successful, the patient is cured and is eventually left with normal hypothalamic-pituitary-adrenal function [1]. Surgical techniques and results of transsphenoidal surgery for pituitary adenomas are also reviewed in a separate topic. (See "Transsphenoidal surgery for pituitary adenomas and other sellar masses", section on 'Corticotroph adenomas'.)

Magnetic resonance imaging (MRI) is performed preoperatively to try to locate the pituitary tumor. However, surgery should be performed even if a tumor is not visualized.

Approach — The operative approach varies. Traditionally, the procedure involved transsphenoidal exploration through either a sublabial or endonasal approach, followed by use of a high-powered microscope that allowed for binocular vision. More recently, use of an endoscope, which does not provide binocular vision, has been advocated but has not been definitively shown to be superior [2]. (See "Transsphenoidal surgery for pituitary adenomas and other sellar masses", section on 'Comparison of microscopic versus endoscopic techniques'.)

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Literature review current through: Nov 2017. | This topic last updated: Nov 17, 2017.
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