Researchers Report Rare Case of Cushing’s Caused by Bilateral Adrenal Tumors

Cases of adrenocorticotropic hormone (ACTH)-independent Cushing’s syndrome are often caused by unilateral tumors in the adrenal glands, but Indian researchers have now reported a rare case where the condition was caused by tumors in both adrenal glands.

Fewer than 40 cases of bilateral tumors have been reported so far, but an accurate diagnosis is critical for adequate and prompt treatment. Sampling the veins draining the adrenal glands may be a good way to diagnose the condition, researchers said.

The study, “Bilateral adrenocortical adenomas causing adrenocorticotropic hormone-independent Cushing’s syndrome: A case report and review of the literature,” was published in the World Journal of Clinical Cases.

Cushing’s syndrome, a condition characterized by excess cortisol in circulation, can be divided into two main forms, depending on ACTH status. Some patients have tumors that increase the amount of ACTH in the body, and this hormone will act on the adrenal glands to produce cortisol in excess. Others have tumors in the adrenal glands, which produce excess cortisol by themselves, without requiring ACTH activation. This is known as ACTH-independent Cushing’s syndrome.

Among the latter, the disease is mostly caused by unilateral tumors — in one adrenal gland only —  with cases of bilateral tumors being extremely rare in this population.

Now, researchers reported the case of a 31-year-old Indian woman who developed ACTH-independent Cushing’s syndrome because of tumors in both adrenal glands.

The patient complained of weight gain, red face, moon face, bruising, and menstrual irregularity for the past two years. She recently had been diagnosed with high blood pressure and had started treatment the month prior to the presentation.

A physical examination confirmed obesity in her torso, moon face, buffalo hump, thin skin, excessive hair growth, acne, swollen legs and feet, and skin striae on her abdomen, arms, and legs.

Laboratory examinations showed that the woman had an impaired tolerance to glucose, excess insulin, and elevated cortisol in both the blood and urine. Consistent with features of Cushing’s syndrome, cortisol levels had no circadian rhythm and were non-responsive to a dexamethasone test, which in normal circumstances lowers cortisol production.

Because ACTH levels were within normal levels, researchers suspected an adrenal tumor, which led them to conduct imaging scans.

An abdominal computed tomography (CT) scan showed adrenal adenomas in both adrenal glands (right: 3.1 cm × 2.0 cm × 1.9 cm; left: 2.2 cm × 1.9 cm × 2.1 cm). A magnetic resonance imaging (MRI) scan showed that the pituitary gland (which normally produces ACTH) was normal.

To determine whether both adrenal tumors were producing cortisol, researchers sampled the adrenal veins and compared their cortisol levels to those of peripheral veins. They found that the left adrenal gland was producing higher amounts of cortisol, thought the right adrenal gland was also producing cortisol in excess.

“Our case indicates that adrenal vein [blood] sampling might be useful for obtaining differential diagnoses” in cases of Cushing’s syndrome, researchers stated. Also, they may help design a surgical plan that makes much more sense.”

The tumors were surgically removed — first the left, and three months later the right — which alleviated many of her symptoms. She also started prednisolone treatment, which helped resolve many disease symptoms.

“Bilateral cortisol-secreting tumors are a rare cause of Cushing’s syndrome,” researchers said. So when patients present bilateral adrenal lesions, “it is crucial to make a definitive diagnosis before operation since various treatments are prescribed for different causes,” they said.

The team recommends that in such cases the two tumors should not be removed at the same time, as this approach may cause adrenal insufficiency and the need for glucocorticoid replacement therapy.

From https://cushingsdiseasenews.com/2019/06/27/rare-case-of-cs-due-to-bilateral-tumors-in-the-adrenal-glands/

Through The Art Of Makeup, People With Rare Pituitary Disorders Now Have Unique Resources To Help Address Common Physical And Emotional Changes

Did you know that applying contour powder on certain areas of your face, like the outer rim of the jaw, along the hairline or along the hollows of the cheek, can help make enlarged features less noticeable? Seems like a basic makeup tip, right? Well, to a person with a pituitary disorder that dramatically changes one’s facial features, this type of information may make a major difference – not only physically, but also psychologically.

This is why Novartis has teamed up with Kevyn Aucoin Beauty (KAB) to bring The Highlights Project to those living with acromegaly and Cushing’s disease. The program offers a variety of virtual tools such as makeup suggestions from professional artists and video demonstrations. These resources address common concerns expressed by patients while also educating about these rare, but serious pituitary disorders.

Kevyn Aucoin, founder of KAB and famed Hollywood makeup legend, was diagnosed with acromegaly in 2001 at the age of 40 and passed away less than 12 months later. Aucoin believed in the transformative nature of makeup and saw it as a reflection of both inner and outer beauty. In this spirit, The Highlights Project features a series of makeup tutorials, tips and inspirations designed to help enhance the self-image of pituitary patients. The program also includes the perspective of a psychotherapist who specializes in helping patients with acromegaly and Cushing’s disease.

Like so many other people with acromegaly and Cushing’s disease, Kevyn went undiagnosed for years and faced both emotional and physical challenges as a result of his condition. “Through Kevyn Aucoin Beauty’s partnership with Novartis on The Highlights Project, we hope that we can inspire others living with these pituitary disorders to see their own beauty and view makeup as Kevyn did, not as a mask, but as a tool for discovery,” said Desiree Tordecilla, Executive Vice President, Kevyn Aucoin Beauty.

Acromegaly and Cushing’s disease are pituitary disorders caused by the presence of a noncancerous tumor on the pituitary gland. The symptoms often include highly visual physical changes in the body. For people with acromegaly, enlarged facial features, jaw and brow protrusions, thickening of the skin and skin tags are common. People with Cushing’s disease frequently experience uncontrollable weight gain, facial fullness and redness, a buffalo hump, acne and oily skin. Beyond the external physical changes, these conditions often also cause serious health complications such as cardiovascular issues, fatigue, muscle weakness and cognitive changes. Those living with uncontrolled acromegaly and Cushing’s disease are also at an increased risk of death. Due to the rare nature of these diseases, receiving an accurate diagnosis can be difficult and may take several years – therefore, education and awareness is critical.

The Highlights Project aims to provide support and help those with acromegaly and Cushing’s disease manage the physical manifestations and psychosocial challenges often associated with these conditions.

“As someone who was self-conscious about how unfeminine my facial features appeared, I was amazed by the impact the simple makeup tricks I picked up from The Highlights Project had on my self-esteem,” said Shannon Goodson, who was diagnosed with acromegaly in 2008. “Staying positive, educating yourself about the condition and monitoring hormone levels to ensure the disease is under control are the first steps to empowering yourself and understanding that you are so much more than your diagnosis.”

The mission of The Highlights Project is to help put a face to the challenges those with acromegaly and Cushing’s disease may encounter and serve as a vessel for learning. Novartis is committed to helping to transform the care of rare pituitary conditions and bringing meaningful solutions to patients. To help support acromegaly and Cushing’s disease patients, and learn more about The Highlights Project, visit TheHighlightsProject.com. For more information about these diseases, visit AcromegalyInfo.com and CushingsDisease.com.
Read more from Journal Sentinel: http://www.jsonline.com/sponsoredarticles/health-wellness/through-the-art-of-makeup-people-with-rare-pituitary-disorders-now-have-unique-resources-to-help-address-common-physical-and-emotional-changes8087390808-251841151.html#ixzz2wtDUV9iF

 

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