Cushing’s Syndrome Treatments

Medications, Surgery, and Other Treatments for Cushing’s Syndrome

Written by | Reviewed by Daniel J. Toft MD, PhD

Treatment for Cushing’s syndrome depends on what symptoms you’re experiencing as well as the cause of Cushing’s syndrome.

Cushing’s syndrome is caused by an over-exposure to the hormone cortisol. This excessive hormone exposure can come from a tumor that’s over-producing either cortisol or adrenocorticotropic hormone (ACTH—which stimulates the body to make cortisol). It can also come from taking too many corticosteroid medications over a long period of time; corticosteroids mimic the effect of cortisol in the body.

The goal of treatment is to address the over-exposure. This article walks you through the most common treatments for Cushing’s syndrome.

Gradually decreasing corticosteroid medications: If your doctor has identified that the cause of your Cushing’s syndrome is corticosteroid medications, you may be able to manage your Cushing’s syndrome symptoms by reducing the overall amount of corticosteroids you take.

It’s common for some people with certain health conditions—such as arthritis and asthma—to take corticosteroids to help them manage their symptoms. In these cases, your doctor can prescribe non-corticosteroid medications, which will allow you to reduce—or eliminate—your use of corticosteroids.

It’s important to note that you shouldn’t stop taking corticosteroid medications on your own—suddenly stopping these medications could lead to a drop in cortisol levels—and you need a healthy amount of cortisol. When cortisol levels get too low, it can cause a variety of symptoms, such as muscle weakness, fatigue, weight loss, and low blood pressure, which may be life-threatening.

Instead, your doctor will gradually reduce your dose of corticosteroids to allow your body to resume normal production of cortisol.

If for some reason you cannot stop taking corticosteroids, your doctor will monitor your condition very carefully, frequently checking to make sure your blood glucose levels as well as your bone mass levels are normal. Elevated blood glucose levels and low bone density are signs of Cushing’s syndrome.

Surgery to remove a tumor: If it’s a tumor causing Cushing’s syndrome, your doctor may recommend surgery to remove the tumor. The 2 types of tumors that can cause Cushing’s are pituitary tumors (also called pituitary adenomas) and adrenal tumors. However, other tumors in the body (eg, in the lungs or pancreas) can cause Cushing’s syndrome, too.

Pituitary adenomas are benign (non-cancerous), and most adrenal tumors are as well. However, in rare cases, adrenal tumors can be malignant (cancerous). These tumors are called adrenocortical carcinomas, and it’s important to treat them right away.

Surgery for removing a pituitary tumor is a delicate process. It’s typically performed through the nostril, and your surgeon will use tiny specialized tools. The success, or cure, rate of this procedure is more than 80% when performed by a surgeon with extensive experience. If surgery fails or only produces a temporary cure, surgery can be repeated, often with good results.

If you have surgery to remove an adrenal tumor or tumor in your lungs or pancreas, your surgeon will typically remove it through a standard open surgery (through an incision in your stomach or back) or minimally invasive surgery in which small incisions are made and tiny tools are used.

In some cases of adrenal tumors, surgical removal of the adrenal glands may be necessary.

Radiation therapy for tumors: Sometimes your surgeon can’t remove the entire tumor. If that happens, he or she may recommend radiation therapy—a type of treatment that uses high-energy radiation to shrink tumors and/or destroy cancer cells.

Radiation therapy may also be prescribed if you’re not a candidate for surgery due to various reasons, such as location or size of the tumor. Radiation therapy for Cushing’s syndrome is typically given in small doses over a period of 6 weeks or by a technique called stereotactic radiosurgery or gamma-knife radiation.

Stereotactic radiosurgery is a more precise form of radiation. It targets the tumor without damaging healthy tissue.

With gamma-knife radiation, a large dose of radiation is sent to the tumor, and radiation exposure to the healthy surrounding tissues is minimized. Usually one treatment is needed with this type of radiation.

Medications for Cushing’s syndrome: If surgery and/or radiation aren’t effective, medications can be used to regulate cortisol production in the body. However, for people who have severe Cushing’s syndrome symptoms, sometimes medications are used before surgery and radiation treatment. This can help control excessive cortisol production and reduce risks during surgery.

Examples of medications your doctor may prescribe for Cushing’s syndrome are: aminoglutethimide (eg, Cytadren), ketoconazole (eg, Nizoral), metyrapone (eg, Metopirone), and mitotane (eg, Lysodren). Your doctor will let you know what medication—or combination of medications—is right for you.

You may also need to take medication after surgery to remove a pituitary tumor or adrenal tumor. Your doctor will most likely prescribe a cortisol replacement medication. This medication helps provide the proper amount of cortisol in your body. An example of this type of medication is hydrocortisone (a synthetic form of cortisol).

Experiencing the full effects of the medication can take up to a year or longer. But in most cases and under your doctor’s careful supervision, you can slowly reduce your use of cortisol replacement medications because your body will be able to produce normal cortisol levels again on its own. However, in some cases, people who have surgery to remove a tumor that causes Cushing’s syndrome won’t regain normal adrenal function, and they’ll typically need lifelong replacement therapy.2

Treating Cushing’s Syndrome Conclusion
You may need one treatment or a combination of these treatments to effectively treat your Cushing’s syndrome. Your doctor will let you know what treatments for Cushing’s syndrome you’ll need.

From https://www.endocrineweb.com/conditions/cushings-syndrome/cushings-syndrome-treatments

Bilateral testicular tumors resulting in recurrent Cushing’s syndrome after bilateral adrenalectomy

Corresponding author: Troy Puar, MRCP (UK), Department of Medicine, Div. of Endocrinology, Radboud University Medical Centre, 6500 HB Nijmegen, The Netherlands. Phone: +31 243614599, Fax: +31 243618809, e-mail: Troy_puar@cgh.com.sg
Received: July 14, 2016
Accepted: November 29, 2016
First Published Online: November 30, 2016

Recurrence of hypercortisolism in patients after bilateral adrenalectomy for Cushing’s disease is extremely rare.

We present a rare case of a 27-year-old man who previously underwent bilateral adrenalectomy for Cushing’s disease with complete clinical resolution. Cushingoid features recurred 12 years later, along with bilateral testicular enlargement. Hormonal tests confirmed ACTH-dependent Cushing’s. Surgical resection of the testicular tumors led to clinical and biochemical remission.

We report bilateral testicular tumors occurring in a patient with recurrent Cushing’s disease 12 years after bilateral adrenalectomy. Using mRNA expression analysis and steroid metabolome profiling, the tumors demonstrated both adrenocortical and gonadal steroidogenic properties, similar to testicular adrenal rest tumors found in patients with congenital adrenal hyperplasia. This suggests the presence of pluripotent cells even in patients without CAH.

– See more at: http://press.endocrine.org/doi/abs/10.1210/jc.2016-2702#sthash.F4lfWg9j.dpuf

“How can you leave her like this?”

A mother has revealed the anguish her family suffered after her daughter (16), who is in need of brain surgery, was turned away from Beaumont Hospital.

The National Centre for Neurosurgery had no beds or theatre access for nine patients with malignant brain tumours last Friday.
One of the people who was turned away was 16-year-old Chloe Holian from Donegal.

Her mother Caitriona explained to the Anton Savage Show on TodayFM that the road to treatment has been fraught with setbacks.

“I can’t stress how happy I am with the neurosurgeon and his team are there but it seems our consultant’s hands are tied, what am I supposed to do?” she said.

Chloe was diagnosed in July with a recurrence of Cushing’s syndrome, a metabolic disorder which is caused by abnormally high levels of the hormone cortisol in the blood stream.

After being promised treatment in July and then August, the Letterkenny girl was finally admitted on Thursday and was fasting for a procedure on Friday morning when she was told it was cancelled.

“When we got down they told us that they decided to put off the surgery for a couple of days,” said Caitriona.

She was told that the doctors wanted to perform a dexamethasone suppression test first to confirm that Chloe was, in fact, suffering from Cushing’s – despite previous diagnosis revealing that she was.

However, she soon found out that the test couldn’t be performed.

“At 11am someone in scrubs came around to say it wasn’t fair but he had to tell us she won’t be doing the surgery… and she wouldn’t be getting the major test either,” said Caitriona.

She said he was very empathetic of their situation.

“I felt sorry for him having to tell us that news… I asked him ‘how can you leave her like this?’

“He promised that he was going to organise this test himself. It was quite difficult as you need four people in the surgery to do this test, you need the radiographer, neurosurgeon, endocrinologist and anesthetist.”

Unfortunately, an anesthetist was not available for the test.

Caitriona said that Chloe was quite upset at the news. One of the side-effects of her condition is excessive weight gain and the student has gained six stone since last September.

“She had psyched herself up for the surgery,” explained her mother.

“Everybody was around her encouraging her, they threw a party for her before she went because it was a big thing. Chloe has no confidence because she’s put on an extra six stone. She was looking forward to getting her old self back, she just wanted to go and do this operation and get it over and done with.

“For anybody to have a little bit of a weight gain they can be conscious of it but if you’re 16-years-old and you’ve gained six stone and you can’t explain it…”

Caitriona said the family were forced to pack their bags and return to Donegal but, as of today, they have still not received a rescheduled appointment.

The mother-of-three is struggling to juggle home life with trips to Dublin but she said the family’s life is on hold until the tumour is removed.

This is the second time that Chloe has developed Cushing’s, in 2009 she was sent to London for surgery as treatment was not yet available in Ireland.

Patients lives are being threatened by delays, according to the head of the country’s national brain surgery centre. Clinical Director Mohsen Javadpour says people are at risk of dying while they’re waiting for treatment.

From http://www.independent.ie/life/how-can-you-leave-her-like-this-mothers-anguish-as-daughter-16-in-need-of-brain-surgery-is-turned-away-from-beaumont-35029557.html

Elevated late-night salivary cortisol may indicate recurrent Cushing’s disease

Carroll TB, et al. Endocr Pract. 2016;doi:10.4158/EP161380.OR.

 

Elevated late-night salivary cortisol may serve as an early biochemical marker of recurrent Cushing’s disease, and prompt intervention may result in clinical benefits for people with Cushing’s disease, according to recent study findings.

According to the researchers, late-night salivary cortisol level is more sensitive for detecting Cushing’s disease recurrence compared with urinary free cortisol or a dexamethasone suppression test.

Ty B. Carroll, MD, assistant professor at the Medical College of Wisconsin Endocrinology Center and Clinics in Menomonee Falls, and colleagues evaluated 15 patients (14 women; mean age, 49.1 years) with postsurgical recurrent Cushing’s disease (mean time to recurrence, 3.3 years) after initial remission to determine the performance of urinary free cortisol and late-night salivary cortisol measurements for detecting recurrent Cushing’s disease.

Participants were identified as having Cushing’s disease between 2008 and 2013; there was no standard for follow-up, but after remission confirmation participants were followed at least every 6 months after surgery for 2 years and then annually thereafter. Late-night salivary cortisol was the primary biochemical test to screen for recurrence, and follow-up tests with a dexamethasone suppression test, urinary free cortisol or other tests were performed if late-night salivary results were abnormal or if suspicion of recurrence was high.

Of the cohort, 80% had normal urinary free cortisol (< 45 µg/24 hours) at recurrence. Primary transphenoidal adenoma resection was performed in all participants. Evidence of pituitary adenoma on MRI at the time of recurrence was present in seven of 12 participants with normal urinary free cortisol and two of three participants with abnormal urinary free cortisol. Normal renal function was present in all participants, and 14 underwent testing with late-night salivary cortisol, dexamethasone suppression test and urinary free cortisol.

Of participants with normal urinary free cortisol at recurrence, nine had an abnormal dexamethasone suppression test (cortisol 1.8 µg/dL), and all had at least one elevated late-night salivary cortisol measurement (> 4.3 nmol/L). Mean late-night salivary cortisol was 10.2 nmol/L, and mean urinary free cortisol was 19.9 µg/24 hours.

Therapy for recurrent Cushing’s disease was administered in 11 of the 12 participants with abnormal urinary free cortisol. Adrenocorticotropic hormone (ACTH)-staining pituitary adenoma was confirmed in three participants who underwent repeat transphenoidal adenoma resection. Pharmacotherapy was administered to seven participants with normal urinary free cortisol, and two additional participants underwent bilateral adrenalectomy.

Abnormal dexamethasone suppression test was found in two participants with elevated urinary free cortisol at the time of recurrence, and two participants had confirmed abnormal late-night salivary cortisol. All three participants with elevated urinary free cortisol at the time of recurrence underwent therapy.

“This study has shown potential clinical benefit of either surgical or medical therapy in recurrent [Cushing’s disease] patients with elevations of [late-night salivary cortisol] and normal [urinary free cortisol],” the researchers wrote. “We believe that the outcomes observed in this retrospective case series suggest that the risk/benefit ratio of early treatment needs to undergo a more rigorous prospective evaluation utilizing [late-night salivary cortisol] elevation as an early biochemical marker of recurrent [Cushing’s disease].” – by Amber Cox

Disclosure: Carroll reports being a consultant for Corcept Therapeutics. Please see the full study for a list of all other authors’ relevant financial disclosures.

From http://www.healio.com/endocrinology/adrenal/news/online/%7B9ea4e4ed-6428-49b8-9b2a-11462cb21349%7D/elevated-late-night-salivary-cortisol-may-indicate-recurrent-cushings-disease

Role of radiosurgery in management of pituitary adenoma-The BNI experience

Screenshot 2016-05-27 13.12.55

 

S Meah, E Youssef, W White

Summary: Researchers conducted this study to determine the efficacy of stereotactic radiosurgery with CyberKnife for the treatment of recurrent pituitary adenoma. They concluded that stereotactic radiosurgery for recurrent/residual pituitary adenomas using CyberKnife appears to be relatively safe and effective when compared to conventional radiotherapy.

Methods:

  • Included in this retrospective study were patients who underwent cyberKnife radiosurgery for recurrent or residual pituitary adenoma at Barrow Neurological Institute (n=48).
  • Patients were followed for an average of 44 months.
  • Thirty-three patients had non-functioning adenomas, 10 had acromegaly, and 5 had Cushing’s disease.
  • Researchers analyzed the change in tumor size, hormonal function, and complication of therapy for each patient.

Results:

  • Tumor resection operation was performed through either the transsphenoidal or transcranial approach before CyberkKnife treatment.
  • All patients had either recurrence or residual mass in the cavernous sinus before CyberKnife treatment.
  • The total irradiation dose ranged from 2100-4000 cGy in an average of 3-5 fractions.
  • Slightly more than half of the patients (n=26, 54.2%) had smaller tumors at follow-up, while 22 (45.8%) had stable tumors.
  • Visual acuity remained unchanged post-treatment.
  • One patient developed radiation-induced temporal lobe necrosis.
  • Four patients (8.3%) required hormonal replacement due to panhypopituitarism.
  • Of the 15 patients with functioning adenoma, hormonal function improved in 12.
  • Treatment failed in 1 patient with acromegaly, 2 patients with Cushing’s disease, and 1 patient with non-functioning adenoma.

From http://www.mdlinx.com/endocrinology/conference-abstract.cfm/ZZFEBE5A85394340E188330278A399E6CF/57978/?utm_source=confcoveragenl&utm_medium=newsletter&utm_content=abstract-list&utm_campaign=abstract-AACE2016&nonus=0

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