Knowing Your Cushing’s Disease Care is in the Right Hands
Superior Treatment for Patients with Cushing’s Disease
As one of the most comprehensive pituitary disorder programs in the United States, the Pacific Pituitary Disorders Center at Pacific Neuroscience Institute (PNI) offers world-class expert care. Among the top ranked neurology and neurosurgery programs in the nation, our center’s compassionate multidisciplinary specialists provide advanced, personalized treatment while focusing on our patients’ quality of life.
Affiliated with award-winning Providence hospitals Saint John’s Health Center and Little Company of Mary, PNI neurosurgeons lead the way in advancing safer, more effective keyhole and minimally invasive endoscopic pituitary tumor removal approaches.
If you, a family member, or friend have a new diagnosis, require a second opinion, or have a pituitary tumor or related hormonal disorders recurrence, our expert physicians can help you understand your condition and determine an optimal treatment plan.
Think Pituitay. Think PNI.
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For information about pituitary disorder treatment please complete the form below. We will respond to you within 12-24 hours. To speak with someone right away contact us at 213-214-2526.
Symptoms
Because excess cortisol has so many effects on different parts of the body and brain, symptoms of Cushing’s disease can present in many different and initially subtle ways. The major types of changes are in body habitus, blood glucose levels, blood pressure, bone and skin integrity, immune system and ability to fight infection, as well as sexual function and psychological state. Unfortunately, the diagnosis of Cushing’s disease is often long delayed.
Body changes including weight gain in the face (moon face), above the collar bone (supraclavicular) and on the back of the neck (buffalo hump) are commonly seen in patients with Cushing’s disease. Skin changes may include easy bruising, with purplish stretch marks (stria) and red cheeks (plethora) as well as excess hair growth (hirsutism) on the face and body. The high cortisol levels also cause weakness, fatigue and muscle wasting. Women may develop menstrual disorders including amenorrhea (absence of menses) and both women and men may develop decreased libido.
Additional serious consequences may include hypertension, diabetes mellitus, osteoporosis and depression. Patients are often diagnosed with Cushing’s disease after several years of symptoms which might include progressive weight gain, new onset hypertension or diabetes and mood changes. Comparison of old and recent photographs will often demonstrate changes in appearance. An Endocrinologist should always supervise the evaluation for Cushing’s disease.
Diagnosis
Hormonal Diagnosis
The first step in diagnosing Cushing’s disease is to confirm excessive cortisol secretion which is done by performing a 24-hour urinary free cortisol collection, midnight salivary cortisol test and/or a low-dose dexamethasone suppression test. If blood ACTH levels are elevated or normal, then the source is from either an ACTH-secreting pituitary adenoma or an ectopic (elsewhere in the body) ACTH-producing tumor. A high-dose dexamethasone suppression test and/or petrosal sinus sampling are tests used to distinguish between ectopic ACTH production and pituitary ACTH production (Cushing’s disease). Petrosal sinus sampling should never be performed before the diagnosis of ACTH-dependent Cushing’s syndrome is established.
Imaging
Once the diagnosis of Cushing’s syndrome is confirmed by hormonal testing, a pituitary MRI can detect an adenoma in 70-80% of cases. Dynamic post-gadolinium MRI is a recent technique that helps diagnose small adenomas that may not be seen on a conventional pituitary MRI. CT scans of the adrenal glands are very useful for determining the presence or absence of an adrenal tumor causing Cushing’s syndrome.
Treatment
- Endoscopic Endonasal Surgery
Surgical removal is the primary means to achieve long term remission in Cushing’s disease; at Pituitary Centers of Excellence, remission rates range from 80-90% for microadenomas and 30-70% for invasive adenomas or macroadenomas; for non-visible adenomas, the surgical success rate ranges from 40-60%. Because of improved visualization, the endoscopic endonasal approach is rapidly becoming the preferred method for removal of most pituitary adenomas, including ACTH-secreting adenomas. Long-term follow-up with 24 hour urinary free cortisol levels every 6 months is essential to monitor for tumor recurrence which can occur in 5 – 10% of patients. - Medical Therapy
In patients who fail to have remission of their Cushing’s disease or syndrome state after surgery, “adrenal-directed” medications can be used. These are initially effective but have some side effects, and the overall long-term control of Cushing’s disease with these drugs is rather poor. - Two Relatively New FDA-Approved Medical Therapies
For patients with persistent Cushing’s disease after surgery, include Signifor (pasireotide) and Korlym (mifepristone). Both medications have shown moderate success in clinical trials. Pasireotide is an injectable somatostatin receptor ligand with strong affinity for somatostatin receptor 5. In clinical trials, pasireotide reduced urinary free cortisol (UFC) levels in a majority of Cushing’s disease patients. The main side effect is hyperglycemia. Mifepristone, is a glucocorticoid receptor blocker that can result in improvements in symptoms of Cushing’s and blood glucose control. The main side effects are adrenal insufficiency, low blood potassium levels and vaginal bleeding in women. - Bilateral Adrenalectomy
Removal of the adrenal glands was at one time a preferred method for treating Cushing’s disease and lowering cortisol levels. Currently however, this treatment is reserved for patients who have failed prior endonasal transsphenoidal surgery. Although bilateral adrenalectomy has a high success rate in reversing hypercortisolism ranging from 90% to 100%, there is significant risk (25%) of developing Nelson’s syndrome with an aggressive ACTH-secreting adenoma. Prior radiosurgery or radiotherapy to the pituitary gland may reduce the risk and delay onset of Nelson’s syndrome. The average interval between bilateral adrenalectomy and development of Nelson’s syndrome is approximately 5 to 10 years but may be as short as 6 months. Given the long-term risk of Nelson’s syndrome after bilateral adrenalectomy this treatment is generally considered a 2nd or 3rd line therapy.
Our Physicians
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Patient Experience
Pituitary Disorders Center
Dennis’ Story – Pituitary Adenoma
Experience an actual account of Pituitary Adenoma.
Ajay’s Story – Pituitary Adenoma
Experience an actual account of Pituitary Adenoma.
Pituitary Disorders Center
Dennis’ Story | Pituitary Adenoma
