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Definition
Acromegaly is a rare disorder of excessive bone and soft tissue growth due to elevated levels of growth hormone. In young children, prior to the completed fusion and growth of bones, excessive growth hormone can cause a similar condition called gigantism.
Causes
In most cases, acromegaly is caused by over-secretion of growth hormone (GH) produced by a benign tumor of the pituitary gland. The pituitary gland is a small gland located at the base of the brain that produces many hormones. In a small number of cases, malignant tumors of other organs (pancreas, adrenal, lung) may be the source of excess GH.
Pituitary Gland
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Risk Factors
A risk factor is something that increases your chances of getting a disease or condition. Some rare cases of acromegaly are hereditary. The average age of diagnosis is 40-45 years old.
Symptoms
Symptoms usually develop very slowly over time. Acromegaly can cause serious complications and premature death if not treated.
In children, excess GH production causes elongation of the bones and associated soft tissue swelling. If not treated, children with this disorder can grow to a height of 7-8 feet.
Symptoms and complications in adults may include the following: Abnormally large growth and deformity of the: Hands (rings no longer fit)Feet (need a bigger size shoe)Face (protrusion of brow and lower jaw)Jaw (teeth do not line up correctly when the mouth is closed)LipsTongueCarpal tunnel syndrome Skin changes, such as: Thickened, oily, and sometimes darkened skin Severe acneExcessive sweating and offensive body order due to enlargement of the sweat glandsDeepening voice due to enlarged sinuses, vocal cords, and soft tissues of the throatFatigue and weakness in legs and armsSleep apneaArthritis and other joint problems especially in the jaw Hypothyroidism Enlargement of the liver, kidneys, spleen, heart, and/or other internal organs, which can lead to: DiabetesHigh blood pressureCardiovascular disease In women: Irregular menstrual cyclesGalactorrhea (abnormal production of breast milk) in about 50% of cases In men: Impotence in about 50% of cases DiagnosisThe doctor will ask about your symptoms and medical history, and perform a physical exam. The symptoms of acromegaly develop slowly over a number of years, so it is often not diagnosed until years after its onset.
Blood tests will be done to measure the level of insulin-like growth factor (IGF-I), currently the best screening test, growth hormone releasing hormone (GHRH), and other pituitary hormones. A glucose tolerance test may also be given to see if the GH level dropsit will not drop in cases of acromegaly. If these tests confirm acromegaly, the following may be done to locate the tumor that is causing the disorder: Head CT scan a type of x-ray that uses a computer to make pictures of the inside of the brain and surrounding structures MRI scan a test that uses magnetic waves to make pictures of the inside of the body, in this case the head Treatment The goals of treatment are to: Reduce production of GH to normal levelsStop and reverse the symptoms caused by oversecretion of GHCorrect other endocrine abnormalities (thyroid, adrenal, sex organs)Reduce the tumor sizeTreatment may include: SurgerySurgical removal of the pituitary tumor, or other tumor, that is believed to be causing acromegaly may be done. In most cases, this is the preferred treatment. RadiotherapyRadiotherapy is the use of highly focused external beams of radiation to shrink the tumor. It is used most often used when conventional surgery cannot be used or when medications have failed.
Medication Drugs may be given to reduce the level of GH secretion from the pituitary gland. These include: Cabergoline (Dostinex)given orallyPergolide (Permax)given orallyBromocriptine (Parlodel)may be given before surgery to shrink tumorOctreotide (Sandostatin)given by injections (may be the most effective medication for this condition)Pegvisomantgiven by injections if not responding to other forms of treatmentMedications must often be combined with other therapies to treat larger tumors affecting surrounding structures. PreventionThere are no guidelines for preventing acromegaly. Early diagnosis and treatment, however, will help prevent serious complications, some of which are irreversible. RESOURCES: National Institute of Diabetes & Digestive & Kidney Diseaseshttp://www.niddk.nih.gov Pituitary Network Associationhttp://www.pituitary.org Pituitary Tumor Network Associationhttp://neurosurgery.mgh.harvard.edu/ptna CANADIAN RESOURCES: BC Health Guidehttp://www.bchealthguide.org/ Health Canadahttp://www.hc-sc.gc.ca/index_e.html References: Abrams P, Alexopoulou O, Abs R, et al. Optimalization and cost management of lanreotide-Autogel therapy in acromegaly. Eur J Endocrinol. 2007;571-577.
Acromegaly treatment consensus workshop participants: guidelines for acromegaly management. J Clin Endocrinol Metab . 2002; 87:4054-4058. American Academy of Family Physicians website. Available at: http://www.aafp.org . Cook DM. AACE Acromegaly Guidelines Taskforce. American Association of Clinical Endocrinologists medical guidelines for clinical practice for the diagnosis and treatment of acromegaly. Endoc Pract . 2004;10:213-225. Glustina A, Barkan A, Casanueva FF, et al. Criteria for cure of acromegaly: a consensus statement. J Clin Endocrinol Metab . 2000;85:526-529. Melmed S. Medical progress: acromegaly. N Engl J Med . 2006;355:2558-2573. The Merck Manual of Medical Information . Simon and Schuster; 2000. National Institute of Diabetes & Digestive & Kidney Diseases website. Available at: http://www.niddk.nih.gov . Paisley AN, Trainer PJ. Medical treatment in acromegaly. Curr Opin Pharmacol . 2003;3:672-677. Trainer PJ, Drake WM, Katzneison L, et al. Treatment of acromegaly with the growth hormone-receptor antagonist pegvisomant. N Engl J Med . 2000; 342:1171-1177. Last reviewed March 2008 by David Juan, MDPlease be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.
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