Rare nasal tumor causes Cushing’s syndrome in man, 70, in US
Case highlights 'importance of coordinated, multidisciplinary care'
The case of a 70-year-old U.S. man with ectopic Cushing’s syndrome caused by an unusual type of nasal tumor, called an olfactory neuroblastoma, was described in a report that, according to its author, “demonstrates the necessity of collaboration among endocrinology, otolaryngology, neurosurgery, radiology, and oncology teams in managing rare ACTH-secreting tumors.”
The man’s condition was ultimately effectively managed with the cortisol-lowering medication ketoconazole before surgery and radiotherapy to address the tumor, the report noted.
“This case highlights the importance of timely diagnosis, comprehensive biochemical and radiologic assessment, and coordinated multidisciplinary management in [adrenocorticotropic hormone]-producing [olfactory neuroblastoma],” the researcher wrote.
Endocrinology deals with hormone-related conditions, while otolaryngologists are more commonly called ear, nose, and throat doctors. Neurosurgery or brain surgery is another medical specialty, as is oncology, or cancer treatment. Radiology uses imaging technology for the diagnosis and treatment of medical conditions.
Titled “Ectopic ACTH-Dependent Cushing Syndrome Secondary to Olfactory Neuroblastoma: A Rare Entity,” the case report was published in the journal AACE Endocrinology and Diabetes by a clinician in California.
Cushing’s syndrome refers to health conditions caused by high cortisol levels. It is most often caused by a tumor in the brain’s pituitary gland that releases too much adrenocorticotropic hormone (ACTH) to stimulate cortisol production; this is known as Cushing’s disease. Less commonly, the syndrome is caused by ACTH-producing tumors in other parts of the body, when it’s then known as ectopic Cushing’s syndrome.
Patient found to have multiple co-occurring medical conditions
This report detailed the case of a man starting his eighth decade with a relevant medical history of asthma, enlarged prostate, and high levels of fatty molecules in the blood. He sought treatment at a hospital with a six-week history of muscle weakness, abdominal fat, leg swelling, headaches, nosebleeds, and a 9.1 kg (20 pounds) weight gain.
During a physical exam, the man was found to have high blood pressure, fat buildup in the face and between the shoulder blades, stretch marks in the abdomen, and soft tissue swelling — all indicative of Cushing’s. Laboratory analyses revealed high cortisol levels in the blood, saliva, and urine, as well as high ACTH and blood sugar levels. His blood potassium levels were low and corrected with medication, and he also showed signs of kidney injury.
ACTH and cortisol blood levels failed to decrease in response to the low-dose dexamethasone test, consistent with ectopic ACTH-dependent Cushing’s.
Imaging CT studies of the head revealed the presence of a tumor in the ethmoid sinuses — the air-filled spaces located between the eyes, in the upper part of the nose — with no evidence of a pituitary tumor. The patient also had a significant enlargement of the adrenal glands, which sit atop the kidneys and produce cortisol.
Further analysis of the tumor tissue determined the diagnosis of olfactory neuroblastoma, a rare type of tumor originating in the upper part of the nasal cavity that’s responsible for the sense of smell.
Rare nasal tumor removed with minimally invasive procedure
The man was started on treatment with ketoconazole to reduce complications associated with high cortisol levels, then underwent surgery to remove the tumor via a minimally invasive procedure through the nose. Due to the suppression of ACTH and cortisol production after the surgery, he started treatment with hydrocortisone.
Within two weeks of the surgery, the patient experienced a normalization of blood pressure, blood ACTH levels, potassium, and sugar. To prevent disease recurrence, he was given radiotherapy.
He continued receiving hydrocortisone until his condition improved enough to be discharged home. He gradually tapered and discontinued hydrocortisone three months after the surgery, when ACTH, cortisol, and blood sugar levels had sustained normalization.
Early detection, complete surgical resection, and multimodal therapy, including radiotherapy, are associated with improved outcomes.
At this point, his Cushing’s symptoms were markedly reduced, and he had a normalization of the size of the adrenal glands. A follow-up CT scan confirmed the complete removal of the tumor.
According to the researcher, the prognosis of an olfactory neuroblastoma is influenced by several factors, including treatment strategy.
“Early detection, complete surgical resection, and multimodal therapy, including radiotherapy, are associated with improved outcomes,” the researcher wrote, calling them “critical components of care.”
“Lifelong follow-up with serial imaging and endocrine evaluation is essential to monitor for recurrence and late-onset adrenal insufficiency,” the researcher added.
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