Acromegaly

Definition

Growth hormone (GH) controls the growth of soft tissue and bone. Elevated GH causes an excess of bone and soft tissue growth. In adults, this can cause a rare disorder called acromegaly. It can cause serious complications and early death if not treated.In young children, bone growth is still occurring. Excess GH can cause a similar condition called gigantism. Gigantism causes dramatic growth in children.

Causes

The pituitary gland is a small gland located at the base of the brain. It produces many hormones, including GH. In most cases, the elevation of GH is caused by a benign tumor of this gland. In a small number of cases, cancerous tumors of other organs, such as the pancreas, adrenal, or lung, may be the source of excess GH.
Pituitary Gland
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Risk Factors

Acromegaly is more common in people who are 40-45 years of age. Family history of acromegaly may rarely increase your risk of this condition.

Symptoms

Symptoms usually develop slowly over time.In children, the bones are longer and cause soft tissue swelling. If not treated, children can grow to a height of 7-8 feet.Symptoms and complications in adults may include:
  • Abnormally large growth and deformity of the:
    • Hands—rings no longer fit
    • Feet—need a bigger size shoe
    • Face—bulging of brow and lower jaw
    • Jaw—teeth do not line up correctly when the mouth is closed
    • Lips
    • Tongue
  • Skin changes, such as:
    • Thickened, oily, and sometimes darkened skin
    • Severe acne
    • Excessive sweating and unpleasant body order due to enlargement of the sweat glands
  • Deepening voice due to enlarged sinuses, vocal cords, and soft tissues of the throat
  • Difficulty sleeping due to a blocked airway
  • Swelling in the neck
  • Fatigue and weakness in the legs and arms
  • Joint pain, especially in the jaw
  • In women:
    • Irregular menstrual cycles
    • Abnormal production of breast milk
  • In men—inability to get or sustain an erection

Diagnosis

You will be asked about your symptoms and medical history. A physical exam will be done. Acromegaly is often not diagnosed until years after it begins.Your bodily fluids may be tested. This can be done with blood tests.Images may be taken of your bodily structures. This can be done with:

Treatment

The goals of treatment are to:
  • Reduce production of GH to normal levels
  • Stop and reverse the symptoms caused by excess GH
  • Correct other endocrine abnormalities, such as thyroid, adrenal, and sex organs
  • Reduce tumor size
Treatment may include:

Surgery

The tumor that is believed to be causing acromegaly may be removed. In most cases, this is the preferred treatment. However, drug treatment is increasing in popularity.

Radiation Therapy

In adults, external beams of radiation are used to shrink the tumor. It is most often used when surgery cannot be used or when medications have failed.

Medication

Medications may be given to: reduce the level of GH. These include:
  • Reduce the level of GH
  • Shrink the tumor before surgery

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Prevention

There are no current guidelines to prevent acromegaly. Early treatment will help to prevent serious complications.

RESOURCES

National Institute of Diabetes & Digestive & Kidney Diseases
http://www.niddk.nih.gov

Pituitary Network Association
http://www.pituitary.org

CANADIAN RESOURCES

Canadian Society of Endocrinology and Metabolism
http://www.endo-metab.ca

Health Canada
http://www.hc-sc.gc.ca

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. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed. Updated May 9, 2014. Accessed June 4, 2014.

Acromegaly treatment consensus workshop participants: guidelines for acromegaly management. J Clin Endocrinol Metab. 2002; 87:4054-4058.

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, et al. Criteria for cure of acromegaly: a consensus statement. J Clin Endocrinol Metab. 2000;85:526-529.

Katznelson L, Atkinson JL, et al.American association of clinical endocrinologists medical guidelines for clinicalpractice for the diagnosis and treatment of acromegaly - 2011 update: executive summary. Endocr Pract. 2011;17(4):636-646.

Melmed S. Medical progress: acromegaly. N Engl J Med. 2006;355:2558-2573.

Paisley AN, Trainer PJ. Medical treatment in acromegaly. Curr Opin Pharmacol. 2003;3:672-677.

Sherlock M, Woods C, Sheppard MC. Medical therapy in acromegaly. Nat Rev Endocrinol. 2011;7(5):291-300.

Trainer PJ, Drake WM, et al. Treatment of acromegaly with the growth hormone-receptor antagonist pegvisomant. N Engl J Med. 2000; 342:1171-1177.

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