Editing Hypercortisolism (section)

== Management ==

* '''ACTH-independent disease: ipsilateral adrenalectomy'''
** '''Medications that block enzymes of steroid synthesis (mitotane, metyrapone, aminoglutethimide trilostane, ketoconazole, etomidate) are used for bridging a hypercortisolism patient to surgery or when surgical intervention is not possible.'''
* '''Cushing disease (ACTH-secreting pituitary adenoma): trans-sphenoid surgical resection'''
** '''Bilateral adrenalectomy is most often recommended when at least one attempt to treat the primary tumor has failed.''' It is also necessary in rare instances when hypercortisolism is life-threatening and swift definitive treatment is mandatory. '''Lifelong mineralocorticoid and glucocorticoid replacement is required in all patients.'''
*** Patients undergoing bilateral adrenalectomy are at risk (8-29%) for progressive growth of their pituitary adenoma, resulting in complications such as ocular chiasm compression, oculomotor deficiencies, and, rarely, a rise in intracranial pressure, resulting in the Nelson-Salassa syndrome (also known as Nelson syndrome), which is found in 8-29% of patients who have undergone bilateral adrenalectomy.
*** '''When counseling patients regarding bilateral adrenalectomy for ACTH-dependent Cushing syndrome, the urologist must also warn of the rare possibility of residual, functioning adrenal tissue remaining after the procedure'''
* '''Ectopic ACTH production: resection of the ACTH-producing tumor.'''
** Primary tumor resection is possible in only 10% of patients.
** '''For patients with unresectable primary tumors or whose primary ACTH-producing tissue cannot be identified, bilateral adrenalectomy with lifelong replacement therapy is an excellent therapeutic option'''