Urgent response advised for Cushing’s due to pancreatic tumors
Doctors discover tumors had spread to liver after initial tumor removal surgery
Two unusual cases of Cushing’s syndrome caused by pancreatic tumors that had spread to the liver were described in a recent report, highlighting the importance of assessing the presence of Cushing’s in people with pancreatic tumors who continue to show signs of the disorder.
The disorder, called ectopic ACTH syndrome (EAS), “is an emergency endocrine disease requiring an
urgent response,” researchers wrote. “Considering that all [pancreatic neuroendocrine tumors] have the potential to transform into ACTH-producing tumors during treatment, it is important to perform an endocrinological examination if the patient’s general condition deteriorate rapidly during [pancreatic neuroendocrine tumors] treatment.”
The report, “Two cases of pancreatic neuroendocrine tumors with ectopic ACTH syndrome during their disease course,” was published in the Clinical Journal of Gastroenterology.
Cushing’s may be caused by tumors outside pituitary gland in rare cases
Cushing’s syndrome is a broad umbrella term that encompasses any health condition where symptoms are driven by excessive levels of the hormone cortisol. Cushing’s disease is a specific form of the syndrome caused by a pituitary tumor that produces and releases a signaling molecule called adrenocorticotropic hormone (ACTH). High ACTH levels trigger the excessive production of cortisol by the adrenal glands.
In more rare cases, Cushing’s may be caused by other types of tumors found outside the pituitary gland that start to produce ACTH. This is sometimes referred to as ectopic ACTH syndrome.
In this report, scientists in Japan described the cases of two people — a 46-year-old man and a 55-year-old woman — who had EAS caused by pancreatic neuroendocrine tumors, or PanNETs, which are rare tumors that form in the hormone-producing cells of the pancreas.
“Adrenocorticotropic hormone (ACTH)-producing PanNETs are rare, functional tumors, and their clinical characteristics and outcomes have not been well reported,” the researchers wrote.
In both cases, patients were initially diagnosed with PanNETs, and had surgery to remove the pancreatic tumor. However, in both cases, the tumors were later found to have spread (metastasized) to the liver, requiring both patients to continue anti-cancer treatments for several years after their surgeries.
Patients started to show Cushing’s signs a few years after tumor surgery
A few years after the initial surgery, both patients started to show signs of Cushing’s syndrome, including sudden weight gain and a rounded “moon” face.
Lab tests in both patients showed elevated levels of both ACTH and cortisol, and examination of the tumors removed years before showed both were producing ACTH. Clinicians ultimately diagnosed both patients with EAS due to metastatic PanNETs.
This is an incredibly rare finding, the researchers noted, as there have been only two other documented cases of PanNETs causing EAS after the original tumor in the pancreas was removed.
Following this diagnosis, both patients were given the cortisol-lowering medication metyrapone (sold under the brand name Metopirone), along with other supportive therapies. Treatment was effective for lowering cortisol levels in both cases.
The first patient has now lived more than two years since the appearance of Cushing’s symptoms, and his condition remains largely stable. The general condition of the second patient, however, did not improve, and she eventually died a few months after EAS first developed.
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