Treatment for Cushing's syndrome depends on the cause.
If long-term use of steroid medicine is the cause of Cushing's syndrome:
- Your doctor will help you lower your dose or gradually stop taking it. It may take a while for the symptoms to go away.
- Do not stop taking steroid medicine on your own. That can be very dangerous. Your doctor will help you change your medicine or lower the dose slowly.
If a pituitary tumor is the cause:
- Surgery to remove the tumor offers the best chance for recovery. This surgery requires great skill and should be done at a major medical center where doctors specialize in pituitary surgery.
- If surgery to remove the tumor isn't possible or hasn't worked, you may have other options to consider such as medicine, radiation, or surgery to remove the adrenal glands. You and your doctor can talk about the pros and cons of each option.
If an adrenal tumor is the cause:
- Surgery to remove the tumor is usually done if the tumor is not cancer (benign). If the tumor is cancer, the whole gland is removed.
- Medicine may be tried if surgery isn't an option.
If a tumor of the lungs or another organ is the cause, the tumor will be removed or treated with radiation or medicines.
Cushing's Disease and Negative MRI
Patients with Cushing’s disease often present with a “negative” MRI study, showing no evidence of tumor. Inferior petrosal sinus sampling is mandatory to confirm that the source of ACTH production is the pituitary gland.
The first step is these cases is to make sure the MRI study is of high-quality, as frequently patients do not obtain the best possible imaging. At Stanford Pituitary Center we have developed with our expert neuroradiologists the best possible imaging protocol to maximize chances of microadenoma identification; this includes a 1-mm fine-cut T1 sequences with and without contrast in all 3 spatial planes, dynamic sequences, and a flair post-contrast sequence.
If imaging is still not conclusive, intraoperative exploration is required, following the technique described by Professor Oldfield that Dr. Juan Fernandez-Miranda learnt from him during his tenure at University of Virginia (2007-2008). This starts with wide surgical exposure to carefully explore all surfaces of the gland, including those in contact with the medial wall of the cavernous sinus, as microadenomas may cause mild bulging in the surface of the gland. Next, vertical incisions separated by 2-mm are performed to gently explore the inside of the gland, starting at the site of highest suspicion. Finally, when no adenoma is identified, and selective removal is not possible, a partial hypophysectomy is performed by removing a quarter of the gland on each side and at the bottom of the gland and preserving the central core of the gland. This technique maximizes chances of microadenoma removal while minimizing risk of pituitary dysfunction.
Treatment for Ectopic Cushing’s Syndrome
If the evaluation suggests the presence of ectopic Cushing's syndrome, then the treatment is directed to the source. For example, if a carcinoid tumor in the lung is detected, then there are several options for therapy, including surgical excision.
Treatment for Adrenal Growth
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.