Patients with ectopic Cushing’s syndrome face challenges in their healthcare management if they live in developing countries, according to a study of cases in Colombia that describes challenges unique to the tropics.
“This study may lead to the formation of groups of excellence for the management of [Cushing’s syndrome] and impact the survival and quality of life of those affected with this uncommon disease,” researchers wrote.
Abnormally high amounts of the hormone cortisol in the bloodstream cause Cushing’s syndrome. This excess cortisol can stem from chronic corticosteroid use (exogenous Cushing’s) or from small tumors (endogenous Cushing’s) found mostly in the pituitary gland.
These tumors trigger the overproduction of adrenocorticotrophic hormone (ACTH). ACTH then signals the adrenal glands, which sit atop the kidneys, to secrete excess cortisol (hypercortisolism).
Tumors arising outside the pituitary gland that still provoke excess ACTH lead to the rarer ectopic Cushing’s syndrome (ECS), which accounts for roughly 5%–10% of endogenous Cushing’s overall.
Researchers from the University of Antioquía, in Medellín, Colombia, however, pointed out that Cushing syndrome’s prevalence in Colombia is unknown and although limited data from similar countries has been reported, it remains unclear if trends found in those countries can be applied to others.
The team sought to better understand the clinical features of patients with ECS from Colombia, as well as their resources in the healthcare system that could ultimately “provide the best management for these patients.”
From four referral centers, the investigators found 14 ECS cases, comprising eight females and six males, averaging 54.4 years in age.
Most patients (eight) had tumors in their thorax, or upper torso, roughly between the neck and abdomen. Four of these were lung carcinoids, three were small cell lung cancers, and one patient had a metastatic thymoma, which is a tumor that began in the thymus but spread elsewhere.
One patient had a medullary thyroid carcinoma in her neck, and another patient’s tumor was labeled “occult,” meaning that its origin could not be found.
The most common signs and symptoms were muscle weakness (11 patients) and moon face (nine). Others included abdominal fat, a discoloration of the skin known as ecchymosis, dark patches of skin (hyperpigmentation), and build-up of fat between the shoulders (buffalo hump) and above the collarbones (supraclavicular fat pads).
The most common comorbidities, or conditions appearing alongside ECS, were high blood pressure (13 patients), diabetes (12), abnormal amounts of fats in the bloodstream (four), and congestive heart failure (three), among others.
The researchers identified some comorbidities that may be unique to patients in tropical zones. Several patients in this series, for instance, experienced malaria, tuberculosis, and other tropical infections.
Two patients entered septic shock associated with febrile neutropenia (fever with a low count of neutrophils, a type of white blood cell) following their first cycle of chemotherapy. Four patients had osteoporosis and one had fractures in his lower vertebrae that were not associated with metastatic disease.
The patients’ median average level for basal cortisol was 30.5 micrograms per decilitre (mcg/dL). For 24-hour urinary free cortisol (UFC), it was 2,600 micrograms per 24 hours (mcg/24 h) in the 12 patients with data available. For ACTH, the median average level was 91 picograms per millilitre (pg/mL).
The site of a patient’s primary, or originating, tumor could be found in 13 individuals, and seven underwent surgery to remove tumors. No post-surgery UFC measurements were available to determine if the surgery corrected the excess cortisol levels. Surgery corrected hypercortisolism in four patients.
Eight patients with advanced disease died from tumor progression or complications related to chemotherapy. Their median survival time was 27 months.
Inoperable tumors associated with the highest mortality risk and tumors of the upper torso, called intrathoracic tumors, showed the most aggressive behavior.
Surgery to remove the primary tumor appeared to be the most effective ECS treatment. The researchers noted that access to some of the more sophisticated technology to locate tumors is limited in their region, which presented a challenge.
“Our series shows the necessity in developing countries of an adequate biochemical evaluation, localization studies, and an expert’s center with endocrinologists and surgeons for ECS treatment,” the researchers wrote.
“ECS is a rare endocrine disease associated with a high rate of complications and mortality,” they wrote. “This report may serve as a reference for studies at the national or regional level and may lead to a more careful identification of co-morbidities as well as a more cost-effective approach.”
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