Rare adrenal tumor caused woman’s ectopic Cushing’s syndrome
The woman had no typical signs of disease, which delayed her diagnosis
An adrenocorticotropic hormone (ACTH)-producing pheochromocytoma, a rare type of tumor in the adrenal gland, was the cause of Cushing’s syndrome in a 42-year-old woman, according to a case report in Mexico.
The woman had no typical signs of Cushing’s, which delayed her diagnosis and made her “case particularly difficult and unusual,” wrote the researchers. She underwent an adrenalectomy to remove the affected adrenal gland, which normalized her cortisol levels and eased her symptoms.
“The absence of [Cushing’s] features in this patient highlights the need to consider ectopic ACTH secretion in cases of adrenal masses,” the researchers wrote. The woman’s case was described in “Ectopic ACTH-secreting pheochromocytoma without typical signs of Cushing syndrome,” published in Oxford Medical Case Reports.
Cushing’s syndrome is caused by elevated levels of the hormone cortisol, called hypercortisolism. Cushing’s disease, one of the more common forms of the syndrome, is the result of a tumor in the pituitary gland that produces and releases high levels of ACTH, which causes the adrenal glands to produce too much cortisol.
Less often, the syndrome is caused by ACTH-producing tumors elsewhere in the body, called ectopic Cushing’s syndrome.
No typical signs of Cushing’s
In this case, the woman’s ectopic Cushing’s syndrome was caused by an ACTH-producing pheochromocytoma.
She had a history of diabetes, treatment-resistant high blood pressure, and a newly identified adrenal mass. When she was first seen, she showed a lack of energy and strength, and a CT scan confirmed a mass in her right adrenal gland. She had no typical signs of Cushing’s when she was admitted to the hospital.
Initial laboratory tests showed she had high white blood cell counts and high blood sugar levels, while her blood sodium and potassium levels were low. She also had high levels of cortisol in her blood, which remained high after a low dose dexamethasone suppression test, consistent with an ACTH-dependent form of Cushing’s syndrome.
Elevated levels of metanephrines, metabolites of certain hormones that help the body respond to stress, in the urine confirmed a pheochromocytoma.
The woman underwent minimally invasive surgery to remove the right adrenal gland containing the tumor. An analysis of the removed tissue confirmed the pheochromocytoma with a potential for aggressive behavior.
After surgery, her blood pressure stabilized, leading to blood pressure-lowering medications being discontinued. At a follow-up evaluation, the woman’s cortisol and metanephrine levels were normal. A significant reduction in cortisol levels was also seen after a subsequent dexamethasone suppression test. An imaging analysis indicated the tumor had been completely removed.
“Surgical resection is the definitive treatment for pheochromocytomas, particularly those secreting ACTH,” the researchers wrote. “Cortisol levels … returned to normal, demonstrating the effectiveness of adrenalectomy in resolving hypercortisolism.”
At her most recent follow-up, the woman remained without symptoms and had normal lab parameters, with no signs of disease recurrence.
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