The Endocrine Society has released a Clinical Practice Guideline on the diagnosis and treatment of acromegaly, according to a press release.
The condition, which is caused by excess growth hormone (GH) in the blood, is very rare, with an estimated three cases occurring among 1 million people each year. Acromegaly usually occurs in middle-aged men and women, and in children, the excess GH causes gigantism.
A noncancerous pituitary tumor is usually the culprit behind acromegaly. The excess GH is manufactured by the tumor, which also stimulates the overproduction of insulin-like growth factor-1 (IGF-1).
Because of the overabundance of both GH and the growth-promoting IGF-1, a person with acromegaly usually has large hands and feet, thick lips, coarse facial features, a jutting forehead and jaw, and widely spaced teeth. Patients with the condition also are at risk for diabetes, hypertension, cardiovascular disease and sleep apnea.
The guideline recommends surgery to remove tumors from the pituitary gland as primary therapy, which will immediately lower GH levels if successful. Five-year disease recurrence rates range from 2% to 8%, the press release states.
If symptoms persist after surgery, however, the guidelines recommend medical therapy. Additionally, radiation therapy is recommended if tumor tissue remains after surgery or when medication is ineffective or inappropriate.
The release also states that other recommendations include:
- Measurement of IGF-1 in people with facial features or large extremities associated with acromegaly.
- Measurement of IGF-1 levels to rule out acromegaly in those with masses on the pituitary gland.
- Confirmation of diagnosis by seeing if GH is still produced after an oral glucose tolerance test.
- Examination for damage caused by the pituitary tumor, which could lead to hypopituitarism in people diagnosed with acromegaly.
- Imaging studies conducted 12 weeks after surgery to visualize any residual tumor tissue.
- Annual GH and IGF-1 reassessments after withdrawal from medications in patients receiving radiation therapy.
- Surgery as primary therapy in people who develop gigantism as children followed by medication or radiation based on condition and response to therapy.
“Overabundance of growth hormone and 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,” said Laurence Katznelson, MD, of the Stanford University School of Medicine in California, and chair of the task force that authored the guideline.
“The condition often requires individualized treatment because signs and symptoms can vary from patient to patient.”
The guideline was co-sponsored by the European Society of Endocrinology.