Rare ACTH-producing nasal tumor caused ectopic Cushing’s in woman
Case report: Following surgery, she remained symptom-free after 3 years
A rare type of nasal tumor called olfactory neuroblastoma caused ectopic Cushing’s syndrome in a 56-year-old woman, according to a recent case report.
The condition was effectively managed with the cortisol-lowering medication metyrapone before being followed by surgery and radiotherapy. After starting metyrapone, the woman developed an opportunistic lung infection, which required treatment before she could undergo surgery.
The case was described in the report “Ectopic adrenocorticotropic hormone syndrome due to olfactory neuroblastoma: A case report and literature review,” published in Endocrinología, Diabetes y Nutrición (English edition).
ACTH-producing tumors cause ectopic Cushing’s syndrome less frequently
Cushing’s syndrome refers to health conditions marked by elevated levels of the hormone cortisol, or hypercortisolism. Cushing’s disease is commonly caused by a tumor in the brain’s pituitary gland. The tumor releases excessive levels of adrenocorticotropic hormone (ACTH) to stimulate cortisol production.
Less frequently, the syndrome is caused by ACTH-producing tumors found in other parts of the body. This is called ectopic Cushing’s syndrome.
In this report, the researchers described the case of a woman with no relevant medical history except high levels of fatty molecules in the blood controlled with medication. She arrived at the hospital with a three-month history of leg swelling, excessive thirst, production of high volumes of urine, and an 8 kg (about 17.6 pounds) weight loss.
During a physical exam, she was found to have mild hirsutism, or the excessive growth of body hair, slight darkening of the skin of the face, and significant leg and thigh swelling. Laboratory analyses revealed she had high levels of cortisol in the blood, saliva, and urine, as well as high ACTH and elevated blood sugar levels.
Her blood potassium levels were low, so she began treatment with potassium supplementation and spironolactone. She also received insulin to regulate her blood sugar levels.
ACTH and cortisol blood levels failed to decrease in response to low- and high-dose dexamethasone tests, consistent with ectopic ACTH-dependent Cushing’s. Imaging studies, including CT and MRI scans of the head, revealed the presence of a tumor in the olfactory bulb, with invasion of the nasal and left eye cavity.
Researchers reviewed previous cases of Cushing’s caused by a nasal tumor
At this point, the woman mentioned she was unable to perceive smell and had experienced nosebleeds of unknown origin for several months. Further analysis of the tumor tissue determined that her symptoms were caused by an olfactory neuroblastoma, a rare type of nasal tumor originating in the upper part of the nasal cavity that’s responsible for the sense of smell.
She initiated treatment with metyrapone, which controlled her blood levels of potassium, eased swelling, and reduced blood and urine cortisol levels. After seven days of treatment, the woman developed a fever and had lower than normal oxygen blood levels and increased inflammation markers associated with a fungal lung infection.
Following the successful treatment of the infection, she underwent surgery to remove the tumor and radiotherapy. Due to the suppression of cortisol production, she initiated treatment with hydrocortisone.
At a three-year follow-up, the woman remained without symptoms, with cortisol levels within the normal range and no evidence of disease recurrence in imaging scans.
The researchers also conducted a literature review of 30 previously published cases of ectopic Cushing’s caused by olfactory neuroblastoma. Patients had a mean age of 46.5 years and were mainly middle-aged men.
These tumors often presented with nasal obstruction and nose bleeds, and in most patients (56.7%), increased ACTH levels were present before the tumor was detected. Among patients who started on optimal medical therapy, one developed an opportunistic infection, while three began treatment for diagnosed infections simultaneously with treatment for Cushing’s. After treatment, the disease recurred in 15.6% of the patients.
Noting that tumors like the one this woman had are “a very rare cause of [ectopic ACTH-dependent Cushing’s] with poor prognosis and a relapsing course,” the team said that “in the presence of severe hypercortisolism, [preventive treatment] for common opportunistic agents must be considered.”
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