Rare nasal tumor caused ectopic Cushing’s syndrome in man, 47

Surgery reduced cortisol and ACTH levels in case report from Saudi Arabia

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by Andrea Lobo |

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A rare type of nasal tumor, called olfactory neuroblastoma, caused ectopic Cushing’s syndrome in a 47-year-old man, as described in a recent case report.

Surgical treatment led to a reduction of the patient’s blood cortisol and adrenocorticotropic hormone (ACTH) levels, which were elevated as a result of the tumor.

“This report contributes valuable insights shedding light on the unique [olfactory neuroblastoma] manifestation causing ectopic ACTH syndrome,” the researchers wrote.

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The report, “Olfactory Neuroblastoma Causing Cushing’s Syndrome Due to the Ectopic Adrenocorticotropic Hormone (ACTH) Secretion: A Case Report,” was published in the journal Cureus.

Cushing’s syndrome is an umbrella term for disorders driven by high levels of the hormone cortisol, or hypercortisolism. One of its most common forms, Cushing’s disease, is caused by a tumor in the brain’s pituitary gland. These tumors produce and release high amounts of ACTH, which triggers the overproduction of cortisol by the adrenal glands, located atop the kidneys.

In rare cases, Cushing’s syndrome is caused by ACTH-producing tumors found in other parts of the body. Such cases are known as ectopic Cushing’s syndrome.

The cAase report

Here, researchers from Saudi Arabia described the case of a 47-year-old Bangladeshi man with a history of an olfactory neuroblastoma, a rare type of nasal tumor, that had been surgically removed twice and treated with radiotherapy. He also had poorly controlled diabetes and high blood pressure.

He presented to the emergency room with weakness in the legs, loss of of smell and taste, low food intake, and swelling under the lower jaw. The patient had a typical Cushingoid appearance, with a moon face, fat buildup between the shoulders and in the abdomen, purple stretch marks in the abdomen, and skin darkening.

Laboratory tests revealed his blood potassium levels were low while blood sugar levels were high. His cortisol and ACTH levels in the blood were also elevated. A high-dose dexamethasone suppression test failed to reduce both ACTH and cortisol levels, consistent with the possibility of ACTH-dependent ectopic Cushing’s syndrome.

A CT scan revealed the patient had enlarged lymph nodes below the lower jaw, as well as thyroid nodules that were found to be benign. An MRI scan indicated the olfactory neuroblastoma had returned. A biopsy then confirmed there was ectopic ACTH production in the tumor.

After having the tumor surgically removed, the patient’s cortisol and ACTH levels decreased. His blood potassium levels also normalized, resulting in an easing of leg weakness. He received treatment with hydrocortisone for four days.

Three weeks after surgery, his lymph node metastases were removed. The patient opted out of chemotherapy and planned to be transferred to his home country for further management.

According to researchers, this report emphasizes that although rare, ectopic ACTH production may occur in people with olfactory neuroblastoma and that healthcare professionals who deal with these tumors should be aware of this potential complication.

“The ongoing monitoring of the patient’s clinical features will further enrich the understanding of the course of this uncommon phenomenon,” they wrote.