Case study suggests need for tailored Cushing’s treatment
Woman in remission developed another form of syndrome
A U.S. case study describes woman in remission from Cushing’s disease who later developed a different form of Cushing’s syndrome, a rare occurrence that researchers said suggests the need for recognizing that this may happen and adopting individualized treatment approaches.
The woman was initially diagnosed with Cushing’s disease, a form of Cushing’s syndrome caused by a tumor in the brain’s pituitary gland. Six years after undergoing surgery to remove the pituitary tumor, the 44-year-old woman again started showing symptoms of Cushing’s, which was found to be caused by a tumor in one of her adrenal glands.
“Our case emphasizes the importance of recognizing the rare occurrence of successfully treated [Cushing’s disease] followed by the recurrence of [Cushing’s syndrome] from a different gland, and the adoption of management strategies tailored to each individual patient’s preferences,” the researchers wrote.
The study, “A Second Look at Cushing Disease: Hypercortisolism Recurrence From Another Gland,” was published in JCEM Case Reports.
Cushing’s syndrome is driven by hypercorticolism, or an excess of cortisol in the body. Hypercorticolism can lead to a variety of symptoms, including weight gain and changes to the hair and skin.
Cushing’s treatment and causes
A variety of underlying problems can cause Cushing’s syndrome. These include tumors, which are usually benign. Tumors that form in the pituitary gland cause the subtype of Cushing’s syndrome called Cushing’s disease. Pituitary tumors produce and release high amounts of adrenocorticotropic hormone (ACTH), which increases cortisol production in the adrenal glands.
Cushing’s may also be caused by tumors that form in the adrenal glands themselves, which are located on top of the kidneys.
If Cushing’s is being caused by a tumor, removing the tumor is usually the preferred method of Cushing’s treatment. While these tumors may recur, it is uncommon for a person to have multiple subtypes of Cushing’s.
This is what happened to the woman in the case study, described by a pair of researchers in Phoenix.
She had a history of multiple tumor removal surgeries. In 2009, she underwent surgery to remove her left adrenal gland due to an adrenal tumor. Two years later, she developed Cushing’s disease due to a pituitary tumor, which was surgically removed as well, resolving her symptoms. After that, she had surgery to remove a cancerous tumor in her thyroid gland.
Six years after undergoing surgery for Cushing’s disease, the woman began experiencing Cushing’s symptoms again. These included weight gain, facial roundness and redness, hair loss, and easy bruising. Her clinicians found her urine cortisol levels were high, and an MRI scan revealed an abnormality in her pituitary gland.
“These findings, in conjunction with the patient’s clinical symptoms, raised concerns of recurrence of [Cushing’s syndrome],” the researchers wrote.
Follow-up testing showed that she had low morning ACTH levels in her blood, but her cortisol levels were normal. An MRI scan of her abdomen then revealed a tumor in her right adrenal gland. Over the course of a year, her clinicians continued to monitor her ACTH and cortisol levels, ultimately arriving at the diagnosis of adrenal Cushing’s syndrome.
While removing the affected adrenal gland would have addressed the problem, this would leave her with no adrenal function and would require her to be on lifelong hormone replacement therapy, something she wanted to avoid.
“The patient’s hesitation to undergo a second adrenalectomy [surgery to remove the adrenal gland] demonstrates the importance of personalized medicine in individualizing the treatment plan for our patient,” the researchers wrote.
Medical management options also presented potential difficulties. The woman was concerned about some therapies that could cause side effects affecting her liver function. After consulting with her clinicians, she decided to start treatment with Isturisa (osilodrostat).
Over the following seven years, the size of the woman’s adrenal tumor remained stable. Some of her symptoms, including swelling and lack of energy, also began to ease. A possible side effect of Isturisa is adrenal insufficiency (very low cortisol levels), so the clinical team trained her on what to do when and if those symptoms arose.
Although the case was unusual, it may be instructive, according to the researchers.
“Physicians should be aware of the rare occurrence of two different etiologies of [Cushing’s syndrome] in the same patient and should consider its possibility in patients with recurrent hypercortisolism,” they wrote.
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