Rare adrenal tumor found as cause of woman’s Cushing’s syndrome

Symptoms seen to ease after surgery to remove gland: Case report

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by Andrea Lobo |

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A woman who developed Cushing’s syndrome caused by an adenoma — a benign tumor in the adrenal gland — saw her symptoms ease after she had surgery to remove the gland, scientists in Nepal reported.

In rare cases, adrenal adenomas are associated with high levels of metanephrine, a byproduct of certain hormones made by the adrenal glands. This more frequently is seen in the presence of a pheochromocytoma, a rare adrenal gland tumor that can coexist with adrenal adenomas in some cases.

According to researchers, this occurrence constitutes “an extremely rare case of adrenal adenoma associated with raised metanephrine,” without the presence of a  pheochromocytoma.

Surgery “could resolve” the associated “biochemical and clinical features” of Cushing’s syndrome in patients in such cases, the team noted.

The case was described in a report, “Adrenal adenoma associated with raised metanephrine level: A case report,” published in the International Journal of Surgery Case Reports.

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Cushing’s syndrome comprises a group of conditions driven by excessive levels of the stress hormone cortisol. In Cushing’s disease, one of its most common forms, a tumor in the brain’s pituitary gland triggers the excessive production of a hormone called adrenocorticotropic hormone (ACTH), causing the adrenal glands to overproduce cortisol.

In this report, researchers described the case of a woman who developed Cushing’s syndrome due to an adrenal tumor that was associated with some rare features.

The 30-year-old woman was admitted to the hospital with increased weight gain, weakness in the lower limbs, and uncontrolled blood pressure and blood sugar levels, despite being on several medications to control these conditions.

A physical examination showed the woman had a rounded face indicative of Cushing’s, abdominal stretch marks, and an abnormal curvature of the spine. She also had purple skin patches on the limbs and hands, and swelling in the ankles and feet.

The woman had a six-year history of diabetes, a 2.5-year history of high blood pressure, and hypothyroidism, or insufficient thyroid hormone production. For the prior two years, she had been treated by a psychiatrist for palpitations, decreased sleep, irritability, and aggressive behavior.

Laboratory tests showed the woman had high hemoglobin A1c (HbA1c), an indicator of diabetes. The levels of cortisol in the urine over the course of 24 hours also were elevated. Conversely, her blood ACTH levels were low.

A dexamethasone suppression test, a diagnostic assessment used to evaluate cortisol production, failed to lower its levels, consistent with the presence of Cushing’s.

Testing also showed high metanephrine levels in the urine. Metanephrine results from the breakdown of catecholamines, such as norepinephrine and epinephrine, which function both as hormones to regulate several functions in the body and as nerve cell messengers. Catecholamines are produced by the adrenal glands.

A CT scan then revealed a mass in the woman’s right adrenal gland that extended to the liver, with a size of 3.4 x 2.2 cm. She underwent surgery to remove the right adrenal gland, a procedure called adrenalectomy.

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Tissue analysis shows adrenal tumor is benign

Based on clinical, laboratory, and imaging data, she was initially thought to have Cushing’s syndrome and a pheochromocytoma, which is usually associated with high metanephrine levels.

However, a tumor tissue analysis showed the tumor was benign and lacked the typical features of a pheochromocytoma. Instead, the patient was found to have an adrenal adenoma.

Researchers noted that it’s “extremely rare” to have adrenal adenoma associated with Cushing’s syndrome and elevated metanephrine with no evidence of pheochromocytoma.

After surgery, the woman’s metanephrine and cortisol levels dropped, and her Cushing’s symptoms were seen to ease. After her condition stabilized, she was discharged and placed on regular monitoring.

At the latest follow-up, seven months after surgery, her metanephrine and cortisol levels remained within a normal range.

Researchers noted this case highlights that Cushing’s syndrome due to an adrenal adenoma “can be associated with biochemical and clinical features suggestive of [a pheochromocytoma] which could resolve after adrenalectomy.”