Acromegaly is disorder in the pituitary glands caused by a noncancerous tumor and results in the swelling or enlargement of the face, hands, and feet.
What is acromegaly?
In general, acromegaly is the overgrowth of tissues around the body caused by an excess of growth hormone and Insulin-like Growth Factor (IGF-1). In almost all cases, this overgrowth is caused by the pituitary gland's overproduction of these hormones. A resulting, often benign, tumor, called an adenoma, is likely to be present in the pituitary gland.
Noticeable symptoms include a distinct appearance characterized by prominent facial features and extremely large hands and feet. Other symptoms may include cardiovascular disease, sleep apnea, diabetes, joint disease, carpal tunnel syndrome, skin thickening, and a deepening of the voice. If a pituitary tumor is present, you may also experience headaches, vision loss, loss of libido and, depending if you are a man or a woman, erectile dysfunction or menstrual dysfunction.
Treatments include surgery or radiation to eradicate tumors, medications to control symptoms, and monitoring for additional complications and associated conditions.
You should visit your primary care physician. Acromegaly is usually treated by surgical removal of the abnormal growth in the pituitary gland.
Acromegaly symptoms
Symptoms generally develop over the period of years, and the average time from onset to diagnosis is about 12 years. Symptoms can be related to the appearance, internal growth and swelling, the development of other diseases, and the pituitary tumor.
Appearance-related symptoms
Symptoms of acromegaly that may be outwardly apparent include:
- Enlargement of the jaw, hands, and feet: Over a period of months to years, the excess growth hormone most commonly leads to growth of these tissues. People with acromegaly may notice this as an increase in their glove or shoe size over time, though the changes are generally too slow to notice by looking in the mirror.
- Coarsening of facial features: The nose, facial bones, and jaw may all grow and cause the person's appearance to change significantly over time.
- Skin thickening: The skin of many people with acromegaly will become noticeably thicker.
- Deepening of the voice: Due to changes in facial structures and the tissues of the voice box, a person's voice may become deeper.
Symptoms caused by internal growth and swelling
Swelling and excess growth can also be found within the body, affecting a number of different systems.
- Colon polyps and fibroids: People with acromegaly are at higher risk of other benign tumors, including colon polyps (which can become cancerous) and uterine fibroids.
- Joint disease: Cartilage and joint tissues enlarge, leading to joint and back pain.
- Carpal tunnel syndrome: Swelling of tissues near the wrist can cause the tingling and pain of carpal tunnel syndrome via compression of the median nerve.
- Organ enlargement: Just as the tissues on the outside of the body become larger, so do the tissues inside the body.
- Sleep apnea: Nearly half of people with acromegaly will develop sleep apnea due to obstruction of the airway by changes in bone structure, an enlarging tongue, and tissue growth in the mouth and throat.
Other diseases
Other diseases may develop due to acromegaly, including:
- Cardiovascular disease: People with acromegaly may develop cardiovascular diseases like hypertension, left ventricular hypertrophy, valvular disease, cardiomyopathy or heart failure.
- Diabetes: Growth hormone stimulates production of IGF-1, which has many effects on the metabolic system.
Tumor-related symptoms
People with acromegaly may also experience symptoms due to compression of brain structures by the pituitary tumor. These symptoms include:
- Vision loss: The pituitary gland sits right next to optic nerves, which connect your eyes to your brain. Compression of the nerves by the tumor can damage this connection and cause people to lose parts of their vision.
- Headache: A large mass in the brain can cause headache due to compression of other structures.
- Menstrual dysfunction, erectile dysfunction or loss of libido: The pituitary gland secretes many other hormones. A large pituitary tumor may compress the rest of the gland and lead to lower levels of these other hormones. This can lead to altered sexual function including menstrual dysfunction, erectile dysfunction, and loss of libido.
Acromegaly causes
Acromegaly is primarily caused by an excess of growth hormone in the body. This hormone is required in normal amounts to make the body grow. It is produced within the pituitary gland, which you may remember from your grade school discussions of puberty. This gland is located at the base of the brain behind the eyes.
Pituitary tumor
In most cases of acromegaly, a tumor forms in the pituitary gland called an adenoma. This tumor is almost always benign; however, it produces large quantities of growth hormone much larger than the average adult needs, which results in the following.
- High IGF-1 levels: The growth hormone stimulates the production of another hormone, Insulin-like Growth Factor-1 (IGF-1) to high levels.
- Excess growth: This hormone then acts on tissues around the body, causing them to grow and giving persons with acromegaly their characteristic appearance. The bones, however, do not grow larger as they refuse to grow further after the completion of puberty.
- Further negative effects: These hormones also have significant negative metabolic effects on the individual affected.
Who is most often affected
Acromegaly is a very rare disease. It affects just three to four people per million each year. Most commonly, it occurs in otherwise healthy individuals during their 40s.
Treatment options and prevention for acromegaly
All therapy for acromegaly is aimed at preventing the symptoms and complications described above. This is done primarily by attempting to get the growth hormone and IGF-1 levels back to normal through medications, radiation, or surgery. With early and appropriately managed therapy, many of the symptoms can be reversed (including the changes in appearance) and early death can be avoided.
Medications
Multiple classes of medications exist which can be used to chronically manage acromegaly. These medications all work by either reducing the quantity of growth hormone or IGF-1 or by reducing their action throughout the body.
- Types: These medications include dopamine agonists, somatostatin receptor agonists, and pegvisomant. These medications are either in injection or pill form.
- Prognosis: The downside of these medications is that they have side effects and must be taken for life.
Radiation
Like many tumors, pituitary tumors causing acromegaly have been shown to respond to radiation therapy.
- Efficacy: Radiation appears to be less effective at reducing growth hormone down to normal levels, though it is very good at stopping the growth of the mass.
- Prognosis: The decline in growth hormone is very slow and those affected will often present with other hormone deficiencies later on.
Surgery
In some cases, surgery can act as a cure for acromegaly. Experienced surgeons can remove the tumor by operating through the nose to get access to the base of the brain and the pituitary.
- Efficacy: Removal of the mass is much more effective at curing acromegaly when it is caught early and the tumor is still small.
- Prognosis: Surgery may have side effects due to the sensitive nature of the pituitary gland and the surrounding structures.
When to seek further consultation for acromegaly
Acromegaly is a serious medical condition. All persons with acromegaly should have regular, scheduled follow-up with their primary care physician, as well as an endocrinologist.
If you have been diagnosed with acromegaly
Some studies have reported an average lifespan reduction of ten years for people with acromegaly, mostly due to complications of the disease on other organs and side effects of mismanaged treatment. However, with regular treatment and monitoring, people with acromegaly have been shown to have much better outcomes they can see noticeable improvements in their appearance and a lessening of symptoms.
In general, regular visits with your physician will consist of testing of your hormone levels, checking for progression or regression of symptoms, medication management, and screening for complications of acromegaly.
Questions your doctor may ask to determine acromegaly
- Do you have trouble sleeping?
- Have you been feeling more tired than usual, lethargic or fatigued despite sleeping a normal amount?
- How long has your current headache been going on?
- Any fever today or during the last week?
- How severe is your headache?
Self-diagnose with our free Buoy Assistant if you answer yes on any of these questions.
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References
- R. Dineen, P.M. Stewart, M. Sherlock; Acromegaly, QJM: An International Journal of Medicine, Volume 110, Issue 7, 1 July 2017, Pages 411420, QJM: An International Journal of Medicine Link
- Acromegaly. UCLA Pituitary Tumor Program. UCLA Health Link.
- Chanson P, Salenave S. Acromegaly. Orphanet Journal of Rare Diseases. 2008;3:17. Orphanet Journal of Rare Diseases Link
- National Institute of Diabetes and Digestive and Kidney Diseases. Published April 1, 2012. NIDDK Link.
- Chanson P, Medical Treatment of Acromegaly with Dopamine Agonists or Somatostatin Analogs. Neuroendocrinology. 2016;103:50-58. Neuroendocrinology Link
- Neggers S, J, C, M, M, Muhammad A, van der Lely A, J, Pegvisomant Treatment in Acromegaly. Neuroendocrinology. 2016;103:59-65. doi:10.1159/000381644. Neuroendocrinology Link
- Minniti G, Scaringi C, Enrici RM. Radiation Techniques for Acromegaly. Radiation Oncology (London, England). 2011;6:167. Radiation Oncology Link