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Acromegaly

Acromegaly (also known as gigantism) is the condition when too much human growth hormone is secreted in the pituitary gland. This often comes from a pituitary adenoma in the pituitary gland and this leads to excessive bone growth.

In adolescence, when the growth plates are not closed yet, the bones can grow to the point where the untreated person looks like a giant (= “gigantic”, hence the condition is called “gigantism”).

Symptoms

However, in the older teenager or in adults with closed growth plates, bones still grow, but in a different pattern. Now acromegaly (A= facial appearance, B=hand of patient above, normal hand below) presents with a more prominent chin and the mandible grows to the point where there are bite problems. The larynx cartilage enlarges and a deep husky voice develops. The ribs grow and lead to a barrel like chest. The skin grows thick and darkens. Body hair turn thick and course. The tongue becomes large and furrowed. Joint problems occur from necrotic and erosive changes that lead to premature degeneration. When the tumor gets very large visual problems including blindness can develop, but usually the tumor is detected early enough so that this can be prevented.

Diagnostic tests

 Acromegaly

Acromegaly

Blood tests show very high GH and IGF-1 values. X-rays show an enlarged pocket where the pituitary gland is located (=”sella turcica”). CT or MRI scans depict the adenoma within the anterior lobe of the pituitary gland. A neurosurgical and endocrinological consultation in a university center should be sought to check out the pituitary gland function and to confirm that the diagnosis is indeed a benign adenoma of the anterior pituitary gland. Sometimes there can be an “ectopic source” of GHRH from a tumor in the lungs or in the pancreas (Ref. 4, p.74).

Treatment of acromegaly

Every case is different. However, with a pituitary adenoma a neurosurgeon is usually asked to do a resection of the tumor through the nose and a sinus cavity. This pituitary adenoma surgery is called the transsphenoidal resection of the pituitary adenoma. This procedure is also called transphenoidal hypophysectomy transphenoidal. The operative sample is sent to the pathologist for analysis, but mostly the report is a benign adenoma consisting of growth hormone producing cells. Occasionally the tumor can technically not be removed with surgery. An alternative is the radiotherapy approach where the pituitary gland is irradiated. This, however, has more potential side effects as scarring can occur in adjacent areas such as in the vital hypothalamus. It is important that the parent of the youngster and the patient discuss all the details of the therapy and the possible side effects before the treatment is given.

 

References:

1. B. Sears: “The age-free zone”. Regan Books, Harper Collins, 2000.

2. R.A. Vogel: Clin Cardiol 20(1997): 426-432.

3. The Merck Manual, 7th edition, by M. H. Beers et al., Whitehouse Station, N.J., 1999. Chapter 8: Thyroid disorders.

4. The Merck Manual, 7th edition, by M. H. Beers et al., Whitehouse Station, N.J., 1999. Chapter 7:Pituitary disorders.

5. J Levron et al.: Fertil Steril 2000 Nov;74(5):925-929.

6. AJ Patwardhan et. al.: Neurology 2000 Jun 27;54(12):2218-2223.

7. ME Flett et al.: Br J Surg 1999 Oct;86(10):1280-1283.

8. The Merck Manual, 7th edition, by M. H. Beers et al., Whitehouse Station, N.J., 1999. Chapter 261: Congenital anomalies.

9. AC Hackney : Curr Pharm Des 2001 Mar;7(4):261-273.

10. JA Tash et al. : Urology 2000 Oct 1;56(4):669.

11. D Prandstraller et al.: Pediatr Cardiol 1999 Mar-Apr;20(2):108-112.

12. B. Sears: “Zone perfect meals in minutes”. Regan Books, Harper Collins, 1997.

13. J Bain: Can Fam Physician 2001 Jan;47:91-97.

14. Ferri: Ferri’s Clinical Advisor: Instant Diagnosis and Treatment, 2004 ed., Copyright © 2004 Mosby, Inc.

15. Rakel: Conn’s Current Therapy 2004, 56th ed., Copyright © 2004 Elsevier

Last modified: October 13, 2017

Disclaimer
This outline is only a teaching aid to patients and should stimulate you to ask the right questions when seeing your doctor. However, the responsibility of treatment stays in the hands of your doctor and you.