Uncommon Adrenal Conditions: Tough to Identify, Tougher to Treat

MAY 05, 2017
Thomas Castles

There are several adrenal conditions that can be a big challenge for physicians to identify and treat – especially Cushing’s Syndrome, a rare condition that is the result of too much of the hormone cortisol in the blood usually caused by a tumor.

A trio of specialists presented their findings on Cushing’s Syndrome, adrenocortical carcinoma – a rare deadly disease in which malignant cells form in the outer layer of the adrenal gland – and adrenal function in critically ill patients at the American Association of Clinical Endocrinologists’ (ACE) 26th Annual Scientific and Clinical Congress in Austin, TX.

The presentation Innovations in Evaluation and Management of the Adrenal Diseases was led by Dr. Lynette Nieman, an active clinical investigator and chief of the endocrinology consultation service at the National Institutes of Health clinical center; Dr. Gary Hammer, an adrenal cancer specialist and director of the endocrine oncology program at the University of Michigan’s comprehensive cancer center; and Dr. Amir H. Hamrahian, professor of medicine and chief of the department of endocrinology at Cleveland clinic Abu Dhabi.

Cushing’s is a debilitating endocrine disorder characterized by excessive cortisol levels in the blood, according to Nieman, who underscored that the challenges inherent in not only diagnosing Cushing’s syndrome – these can include potential drug and hormone interference and false negatives or positives in testing and imaging studies – but also the importance of an individualized patient treatment plan to address the long-term effects and comorbidities associated with hypercortisolism.

Hammer discussed the distinction between more common benign tumors of the adrenal cortex and adrenocortical carcinoma (ACC), an ultra-rare endocrine malignancy that forms in the outer layer of the adrenal gland and has usually metastasized to other parts of the body by the time it is diagnosed, noting that most doctors, including endocrinologists, have never seen a case of adrenocortical carcinoma.

Because of this, the challenge, Hammer said, is figuring out when to be concerned, because the signs and symptoms induced by a tumor’s effect on adrenal production of key hormones include high blood pressure, weight gain and diabetes.

These “are among the most common symptoms in Western society,” the researchers said.

Hammer said he and his colleagues are collaborating with clinicians, researchers and institutions around the world to unravel the genetics of the disease with the goal of creating therapies that target the molecular effects of ACC.

Hamrahian went on to highlight the complex condition of adrenal insufficiency in critically ill patients, and recommended the use of random cortisol and free cortisol levels as the primary evaluation tool with these patients.

Read the AACE list of press releases.


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