If an adrenal growth is the underlying cause, then surgical removal of the abnormal adrenal gland is necessary.
Treatment options if the initial therapy is not effective
If the patient has Cushing's syndrome due to excess ACTH levels and the primary tumor has not been resected, there are several next step options. Repeat surgery can be considered to try again to remove the tumor. Surgical removal of both adrenal glands (bilateral adrenalectomy) can also be performed, as this will result in normalization of the effects of Cushing’s syndrome. This therapy will lead to required replacement of adrenal gland hormones, including cortisol and a medication that controls sodium and potassium balance.
Another option is the use of medical therapy. There are several medical therapies that work by reducing adrenal gland production of cortisol. These medications include ketoconazole, metyrapone, and aminoglutethamide.
There are several new medical agents recently available that may help further with medical control of Cushing’s syndrome. Korlym (mifepristone) is a cortisol blocker. This medication, given as a pill, blocks cortisol wherever it goes, and can lead to improvement in appearance and medical consequences in the majority of people.
Another new medication, Signifor (pasireotide) is a new form of somatostatin analog that works at the primary tumor and reduces ACTH in approximately one-third of patients.
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