A 71-year-old woman with ectopic Cushing’s syndrome saw her clinical symptoms subside after undergoing surgery to remove several nodules from her left lung.
One nodule removed was an adrenocorticotropic hormone (ACTH)-producing carcinoid tumor, while another was due to a lung infection caused by the fungus. Cryptococcus. Her infection was associated with immunodeficiency due to elevated ACTH production and subsequently elevated cortisol levels.
“This case provides a valuable lesson that when ACTH-producing tumors are suspected and there are multiple nodules in the lungs, infectious lesions may occur along with carcinoid tumors,” the researchers wrote.
The case report,Ectopic adrenocorticotropic hormone-secreting carcinoid with solitary cryptococcosis in the lungs”, published in the magazine cancer reports.
Cushing’s syndrome is characterized by symptoms associated with hypercortisolismor high cortisol levels, possibly due to tumors leading to excessive ACTH production. Cushing’s disease, a common form of this syndrome, is caused by benign tumors in the brain’s pituitary gland that trigger ACTH production. Rising levels of this hormone signal the adrenal glands, located above the kidneys, to overproduce cortisol.
ACTH-producing lung tumors are a rare cause of ectopic Cushing’s syndrome.
In rare cases, Cushing’s disease is caused by an ACTH-producing carcinoid tumor in the lungs. These and other cases of Cushing’s that are not due to pituitary tumors are called ectopic Cushing’s syndrome.
These patients may be susceptible to infections, as excessive production of ACTH and cortisol can reduce the effectiveness of the immune system. Small cell lung carcinoma accounts for the majority of ectopic cases (40-50%) and can be difficult to diagnoseparticularly when combined with certain infections.
Scientists in Japan have detailed the case of an elderly woman with ectopic Cushing’s syndrome caused by ACTH-producing lung tumors, who also had a fungal lung infection. infections by cryptococcus they are commonly seen in immunocompromised individuals, and the fungus is often transmitted through bird droppings. This patient had no birds or animals.
The woman had been treated for diabetes for 30 years. However, despite diet and insulin therapy, their blood sugar levels, measured by the percentage of glycosylated hemoglobin, that is, the hemoglobin that contains glucose or blood sugar, were in the range of 7 .9% to 8.5% (normal range is less than 5.7%). As such, she was considered to have refractory or difficult-to-control diabetes.
He was admitted to the hospital for diabetic dietary and lifestyle counseling in April 2019. While there, he developed recurrent pneumonia and bronchitis, and exhibited typical Cushing’s symptoms, including a “moon face” (swelling that the face becoming round, full, or puffy), obesity, and red lines on the skin.
Laboratory tests indicated Cushing’s syndrome but were inconclusive, and brain imaging showed no abnormalities in the pituitary gland. The patient refused to submit to a sinus blood sample, a Cushing’s diagnostic test which measures ACTH levels in the blood of the veins that drain the pituitary gland, due to its invasiveness.
High-resolution CT scans showed two nodules in her left lung, suggesting the presence of ectopic Cushing’s syndrome. The nodules, one in the apex region of the lung (top) and the other in the lingual region (bottom), were surgically removed eight months after hospital admission. Immediately after surgery, she was started on hydrocortisone (50 mg/day) as a precaution for cortisol replenishment in adrenal insufficiency.
ACTH-producing tumors increase the likelihood of infection
Tissue examination confirmed that the lingual nodule was a typical ACTH-producing carcinoid tumor, while the nodule in the vertex region was the result of a fungal lung infection.
Four days after surgery, her blood levels of ACTH and cortisol were normal, and hydrocortisone was gradually reduced. She was discharged 15 days after surgery, with an indication to take a lower dose of hydrocortisone (20 mg/day). Thereafter, her urine cortisol levels were within a normal range, and her diabetes and Cushing’s symptoms decreased.
The efficacy of surgery in treating her Cushing’s syndrome was confirmed in tests six months after surgery, and the woman remained on hydrocortisone at 15 mg/day. In July, 18 months after surgery, she showed no evidence of tumor recurrence, no Cushing’s symptoms, and no elevated ACTH levels.
He maintained insulin therapy to control his diabetes, with glycosylated hemoglobin levels now around 7%. Two years after the surgery, she stopped taking hydrocortisone.
“East [is a] very instructive case in terms of the difficulty of diagnosing ACTH-producing tumors, the possibility of infection complicating immunodeficiency caused by ACTH-producing tumors, and the surgical approach,” the scientists wrote, adding that, as in this case, when a carcinoid lung “with ectopic ACTH production”, at the same time another “pulmonary infection nodule” may be present given the possibility of immune system deficiency.
“Fortunately, there are no serious perioperative infections [those around the time of surgery] occurred in this case, but ACTH-producing tumors can lead to an increased risk of infection…so care should be taken,” they wrote.