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Acromegaly diagnosis treatment, guideline issued by The Endocrine Society
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A new clinical practice guideline developed by The Endocrine Society and published the Journal of Clinical Endocrinology & Metabolism addresses evaluation and management of acromegaly.
Recommendations for appropriate biochemical assessment of the rare condition and management during pregnancy are laid out, by strength, and a therapeutic algorithm that includes medical monotherapy or combination therapy offered.
“Overabundance of growth hormone and insulin-like growth factor-1 (IGF-1) can reduce life span and lower quality of life for people with acromegaly, so it is crucial to identify a treatment plan to control hormone levels and the tumor itself,” Laurence Katznelson, MD, of Stanford University School of Medicine, chair of the task force that authored the guideline, said in a statement from the society. “The condition often requires individualized treatment because signs and symptoms can vary from patient to patient.”
Katznelson, selected by the Endocrine Society Clinical Guideline Subcommittee, along with five experts in the field and a methodologist, developed the evidence-based guideline, co-sponsored by the European Society of Endocrinology.
The recommendations are broken down into seven categories including: diagnosis; presentation and management of comorbidities and mortality risk; goals of management; surgery; medical therapy; radiotherapy/stereotactic radiotherapy; special circumstances.
Diagnosis recommendations
Under diagnosis, the task force recommends measuring IGF-1 levels in patients who have facial features or large extremities associated with acromegaly.
“Biochemical screening is recommended for all patients presenting with clinical features of acromegaly,” according to the guideline. “Measuring an IGF-1 level is recommended as the initial screen for acromegaly because it is a marker of integrated (growth hormone) GH secretion.”
Further, the task force recommends against relying on the use of random GH levels to diagnose the condition and for confirming diagnosis by assessing lack of GH suppression following hyperglycemia during oral glucose load.
“Although an elevated random GH level is suggestive of acromegaly, single random GH measurements are not usually recommended due to inherent episodic GH secretion from both normal and adenomatous pituitaries,” according to the guideline.
Measuring IGF-1 levels in patients who have a mass on the pituitary gland is important in ruling out acromegaly, the Society’s guideline highlights.
Managing acromegaly
The task force recommends assessing for hypopituitarism and replacing hormone deficits in patients with acromegaly.
“Hypopituitarism may be due to tumor compression or a result of surgical or radiation treatment,” according to the guideline. “Adequate replacement of central adrenal, gonadal, and thyroid insufficiency is recommended.”
Among the surgery recommendations are imaging studies, MRI preferably, at least 12-weeks after a procedure to remove a pituitary tumor in order to visualize residual tumor and adjacent structures.
“This serves as the new baseline image for follow-up assessment,” according to the guideline. “In patients with preoperative visual field defects, repeat visual field testing should be performed.”
The task force also recommends annual GH/IGF-1 reassessment following medication withdrawal to monitor radiation therapy (RT) effectiveness.
In patients who develop gigantism, the guideline recommends clinicians follow standard approaches to normalizing GH and IGF-1 hyper-secretion.
“Management approaches including treatment combinations of more than one surgical procedure, combined medical treatments, and RT may all be required,” the members wrote. “Surgery is the first line of therapy, although adjuvant therapy is often required.”
Confidence in the guidelines
The task force reviewed primary evidence and commissioned two additional systematic reviews to develop the guideline. The Grading of Recommendations, Assessment, Development and Evaluation system was utilized to describe both the strength of recommendations and the quality of evidence. Consensus was obtained through one group meeting, several conference calls and e-mail communications.
“The Task Force has confidence that persons who receive care according to the strong recommendations will derive, on average, more good than harm,” according to the guideline. “Weak recommendations require more careful consideration of the person’s circumstances, values, and preferences to determine the best course of action.”
Disclosures: Task force members report no relevant disclosures; please see guideline for a full list of their financial disclosures.
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