Rare adrenal tumor caused man’s Cushing’s, per case report

Ectopic Cushing's is curable if diagnosed, treated properly, researchers note

Marisa Wexler, MS avatar

by Marisa Wexler, MS |

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A rare adrenal tumor turned out to be the cause of a man’s Cushing’s syndrome, according to a case report from China.

The man had severe metabolic complications related to Cushing’s, but these resolved rapidly following surgical removal of the disease-driving tumor, called a pheochromocytoma.

Diagnosing and managing a pheochromocytoma is “challenging,” but the tumors are curable “if diagnosed successfully and managed intensively,” researchers wrote in “Ectopic adrenocorticotropic hormone-secreting pheochromocytoma with severe metabolic disturbances: A case report.” Their findings were published in the International Journal of Surgery Case Reports.

Cushing’s syndrome refers to any disorder that’s driven by high levels of the stress hormone cortisol. Cushing’s disease is a specific form of the syndrome caused by a tumor in the pituitary gland in the brain. The pituitary tumor produces and releases high amounts of a signaling molecule called adrenocorticotropic hormone (ACTH). That causes the adrenal glands, a pair of glands on top of the kidneys, to overproduce cortisol.

It’s possible for tumors in body parts other than the pituitary gland to produce ACTH and trigger Cushing’s syndrome. This form of the syndrome is referred to as ectopic Cushing’s syndrome and is much rarer than Cushing’s disease.

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Scans identify adrenal tumor in man with Cushing’s-like symptoms

Only a few documented cases have been reported on ectopic Cushing’s syndrome caused by an ACTH-producing pheochromocytoma.

Now, scientists in the Shandong Province in China described the case of a 46-year-old man who sought medical attention for complaints including vomiting, nausea, excessive sweating, headache, and panic attacks. He had high blood pressure and had been diagnosed with diabetes about a year before.

He’d also lost a substantial amount of weight over the past year, which is unusual in Cushing’s, as patients typically gain weight.

The man’s blood potassium levels fell while he was hospitalized. Low potassium, or hypokalemia, is a common sign of Cushing’s. And a battery of lab tests revealed that he had high levels of cortisol and ACTH, suggestive of Cushing’s disease.

There was no sign of a pituitary tumor. “Despite undergoing cranial MRI, no pituitary lesion was detected, thereby ruling out the possibility of Cushing’s disease,” the researchers wrote.

Imaging scans instead identified a mass on the patient’s right adrenal gland, suggesting that his condition might be caused by an ACTH-producing pheochromocytoma.

The patient underwent surgery to remove the tumor. Because low potassium levels can increase the risk of surgical complications, he was given high-dose potassium supplements administered directly into the bloodstream.

“The [surgical] management of patients with ACTH-secreting pheochromocytomas poses a significant challenge due to severe hypokalemia, and our patient’s potassium levels remained within the normal range through extensive central venous potassium supplementation,” the researchers wrote.

Following removal of the tumor, which was confirmed to be an ACTH-producing pheochromocytoma, the patient’s condition improved substantially. His blood pressure and blood sugar levels normalized, and he showed no signs of disease recurrence in follow-up visits.

“Proper preoperative recognition of complications … is the key to prevent the morbidity and mortality of an ACTH-producing pheochromocytoma,” the scientists wrote.