Blood
Ectopic Cushing’s syndrome tied to repeat blood clots in the lungs
The unusual case of a woman with Cushing’s syndrome caused by an adrenal tumor who experienced recurring episodes of blood clots in the lungs was described in a recent report.
The study, “Adrenocorticotropic hormone-secreting phaeochromocytoma as a cause of treatment-resistant hypertension and recurrent pulmonary emboli,” was published in The Journal of the Royal College of Physicians of Edinburgh.
Cushing’s syndrome is a broad term that encompasses any medical disorder driven by abnormally high levels of the stress hormone cortisol. In its most common form, Cushing’s disease, a tumor in the brain’s pituitary gland leads to the excessive production of the signaling molecule adrenocorticotropic hormone (ACTH), which prompts the adrenal glands sitting atop the kidneys to increase cortisol production.
It’s rarer for tumors found outside the pituitary gland to release ACTH. However, this can happen and lead a form of the syndrome known as ectopic Cushing’s syndrome.
Ectopic Cushing’s syndrome traced to tumor in woman’s left adrenal gland
Scientists in Sweden reported the unusual case of a 50-year-old woman with ectopic Cushing’s caused by a rare adrenal tumor.
She had been living with hypertension since her early 30s, but her high blood pressure had stopped being controlled by treatments. She reported notable weight gain, easy bruising, and muscle weakness — all hallmark symptoms of Cushing’s.
She complained of chest pain and shortness of breath, and a scan of her lungs revealed the presence of a pulmonary embolism — a blood clot — in the upper part of her right lung. She was treated with anticoagulants, medicines that help prevent the formation of blood clots, for six months. But a few months after she stopped them, the chest pain returned and scans revealed more pulmonary embolisms.
She was started on long-term treatment with anticoagulants.
Researchers noted that abnormal blood clots that form in veins and interfere with blood flow, referred to as venous thromboembolism or VTE, occur in up to 8% of Cushing’s patients. Pulmonary embolisms are a type of venous thromboembolism that can be life-threatening.
“An important lesson [of this report] is the increased risk of VTE [in Cushing’s patients],” the researchers wrote.
Clinicians initially thought the woman had Cushing’s disease, so they conducted petrosal sinus sampling — an invasive diagnostic test for disease that measures ACTH levels in the veins draining fluids from the pituitary gland. Results did not show elevated levels of ACTH, however.
Clinicians next performed a similar test, sampling blood from veins near the adrenal glands. This test revealed substantially high ACTH levels in the left adrenal gland vein. Adrenal gland scans also showed signs of an abnormal growth.
The patient underwent surgery to remove her left adrenal gland, and this led to a rapid and dramatic reduction in her cortisol and ACTH levels. Analysis of the removed gland confirmed it housed a type of tumor called pheochromocytoma that was releasing ACTH, confirming the diagnosis of ectopic Cushing’s syndrome caused by an adrenal tumor.
After having the tumor removed, her symptoms eased notably: her blood pressure was better controlled, and though she was still experiencing some muscle weakness, researchers reported that she was gaining in strength with continual physical therapy.
“Patients with ACTH-secreting phaeochromocytoma may present primarily with Cushing’s syndrome, which can be cured with unilateral adrenalectomy [surgical removal of an adrenal gland],” the scientists wrote, noting that “these patients have an increased risk of VTE, which requires careful monitoring.”
