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New guideline for Cushing’s syndrome recommends tumor removal
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The Endocrine Society and the European Society for Endocrinology issued a new clinical practice guideline for managing Cushing’s syndrome, recommending tumor removal as a first-line treatment, according to research in The Journal of Clinical Endocrinology & Metabolism.
The guideline recommends best practices for treating the many causes of the hormonal syndrome, which is characterized by high levels of cortisol in the blood.
“It’s a confusing situation,” Lynnette K. Nieman, MD, chair of the guideline task force and a senior investigator with the NIH Eunice Kennedy Shriver National Institute of Child Health and Human Development, told Endocrine Today. “There are a number of different causes for [Cushing’s syndrome], and the number of treatments are many, and it’s confusing to people as to what is best to do.”
Lynette K. Nieman
The guideline recommends that first-line treatment for Cushing’s syndrome be the removal of the tumor as the best method to preserve pituitary function, unless surgery is not possible or will not address the excess cortisol.
“When you have Cushing’s syndrome, you suppress the normal function of the pituitary gland, so when you take out the tumor, you leave behind all the normal structure so that, eventually, the body can return back to normal function and regulation,” Nieman said. “If one does anything else, you distort the normal tissues.”
Patients who continue to have high levels of cortisol in the blood after surgery should undergo additional treatment, according to the guideline. Those who had an ACTH-producing tumor should receive lifelong screenings for high cortisol or tumor recurrences.
The guideline also noted that patients with Cushing’s syndrome should be treated for common comorbid conditions, including infections, cardiovascular disease, osteoporosis and psychiatric symptoms.
“People with hypercortisolism can die,” Neiman said. “When they die, it’s generally from an infectious cause, either general infectious diseases or unusual infections one would see in an immunocompromised person. They also tend to have more [blood] clots that may cause heart attacks or strokes. They also have high blood pressure oftentimes, and that contributes to morbidity and mortality as well.”
The guideline also stresses that clinicians must educate patients about treatment options and take their values and preferences into consideration.
“It’s important to educate the patient and their family about their disease and about the different kinds of treatment, and also to individualize the therapy to each patient,” Nieman said. – by Regina Schaffer
Disclosure: Nieman reports receiving a research grant from HRA Pharma and honoraria from UpToDate. Please see the complete guideline for the other researchers’ relevant financial disclosures.
Perspective
Back to TopMaria Fleseriu, MD, FACE
Nieman and her panel of international experts accomplished a big task in the recently published Endocrine Society guidelines for treatment of Cushing’s syndrome. Cushing's syndrome is a relatively rare disease but one associated with severe comorbidities and increased mortality. While diagnosis can be somewhat difficult, treatment of Cushing's syndrome of different etiologies might be even more complicated.
The guidelines used a GRADE approach, which rates the panel’s confidence in the risk estimates of favorable and unfavorable outcomes by classifying the quality of the evidence supporting each recommendation. Despite important advances in medical therapy over the last few years, the authors concluded that there is strong evidence that pituitary surgery, adrenal surgery or resection of the tumor causing ectopic Cushing’s should be first-line treatment.
Interestingly, the data are still not clear about which therapy is the best second-line choice in patients with persistent or recurrent disease. Repeat transsphenoidal surgery, medical therapy, radiotherapy and bilateral adrenalectomy are all possible options, and an individualized approach is clearly needed. Physicians’ engagement of the patient in shared decision making is crucial.
The authors also emphasize that patients without an established diagnosis of Cushing's syndrome will not benefit from initiating cortisol-lowering treatment. Another important recommendation involves testing for recurrence. The need to test for recurrence in all cases of ACTH-dependent hypercortisolism and long-term follow-up is advocated and late-night salivary and serum cortisol seem to be the best tests in achieving this goal.
This guideline highlights the big gaps in evidence-based knowledge in rare endocrine diseases, including Cushing's syndrome: numerous important recommendations are low-grade or even ungraded.
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