Acromegaly

Overview

Acromegaly is a rare condition in adults that causes some bones, organs and other tissue to grow bigger. A small gland in the brain called the pituitary gland drives these changes by making too much growth hormone. This usually happens due to a tumor of the pituitary gland. The tumor isn't cancer.

When the body has too much growth hormone, bones get bigger. In childhood, this leads to increased height as part of a condition called gigantism. In adults with acromegaly, a change in height doesn't happen. Instead, bones in the hands, feet and face become bigger.

These changes happen slowly over many years. So people with acromegaly and their loved ones may take a long time to notice the symptoms. And healthcare professionals may have a hard time finding and treating the condition early on.

Without treatment, acromegaly can lead to other serious and sometimes life-threatening health conditions called complications. But treatments such as surgery, medicine and radiation can lower the risk of complications. Treatment also can improve many acromegaly symptoms.

Symptoms

Acromegaly symptoms can change the way some body parts look. Changes can include:

• Thick ears and lips.
• A broad nose.
• Enlarged hands and feet.
• A jutting brow or jaw.
• Gaps between teeth.
• An enlarged tongue.
• An expanded rib cage that may cause the chest to have a round shape.

Skin changes can include:

• Acne.
• Harmless skin growths called skin tags.
• Coarse, oily and thickened skin.
• Swelling in the tissue under the skin.

Most often, people with acromegaly don't have every possible body change. And because the changes come on slowly, they may take years to notice. But over time, rings may no longer fit fingers like they used to. Or shoe size may get bigger. Sometimes, people notice the changes only by comparing old photos with newer ones.

Other acromegaly symptoms can include:

• Vision troubles, including loss of side vision.
• More sweating and body odor than is typical.
• Extreme tiredness.
• Headaches.
• Joint pain.
• Deeper voice.

When to see a doctor

Get a healthcare checkup if you think you have symptoms of acromegaly. The condition usually develops slowly. Even family members may take a long time to notice the physical changes that happen. But it's important for a healthcare professional to find the condition as early as possible. Treatment can help prevent serious health conditions that can happen along with acromegaly.

Causes

The most common cause of acromegaly is a tumor in the pituitary gland. The tumor is called an adenoma. It isn't cancer. But it makes too much growth hormone over a long amount of time.

Too much growth hormone causes many symptoms of acromegaly. Some of the symptoms, such as headaches and impaired vision, are due to the tumor pressing on nearby brain tissues.

Rarely, tumors in other parts of the body cause acromegaly. These include tumors of the lung or pancreas. Sometimes these tumors release growth hormone. Or they make a hormone called growth hormone-releasing hormone. This signals the pituitary gland to make more growth hormone.

The pituitary gland is located at the base of the brain, behind the bridge of the nose. It makes growth hormone and other hormones. Growth hormone plays an important role in controlling physical growth.

The pituitary gland releases growth hormone into the bloodstream. This triggers the liver to make a hormone called insulin-like growth factor-1, also called IGF-1. IGF-1 is really what causes bones and other tissues to grow. Too much growth hormone leads to too much IGF-1. And that can cause acromegaly symptoms and complications.

Risk factors

People who have a rare genetic condition called multiple endocrine neoplasia, type 1 have a higher risk of acromegaly. This condition also is called MEN 1.

In MEN 1, the parathyroid glands, pancreas and pituitary gland may grow tumors and release extra hormones. Extra parathyroid hormone can cause thin bones and kidney stones. A pancreas tumor may make the hormone insulin and cause low blood sugar. If the pituitary tumor makes extra growth hormone, acromegaly results. Very rarely, acromegaly can run in families.

Complications

Without treatment, acromegaly can lead to other health conditions called complications. These complications can include the following.

Conditions of the heart and blood vessels such as:

• High blood pressure.
• Higher risk of narrow arteries, which may lead to a heart attack or stroke.
• A disease of the heart muscle called cardiomyopathy.

Cancer and conditions that can lead to cancer:

• Higher risk of some cancers.
• Growths called polyps on the lining of the colon. Without treatment, these growths can lead to colon cancer.

Sexual and reproductive health conditions such as:

• Missed periods or irregular vaginal bleeding.
• Trouble getting or keeping an erection, also called erectile dysfunction.
• Less sexual desire.

Other serious conditions including:

• The most common type of arthritis, called osteoarthritis.
• Type 2 diabetes.
• An irregular growth of the thyroid gland, called a goiter.
• A condition called sleep apnea in which breathing stops and starts many times during sleep.
• A condition called carpal tunnel syndrome that causes numbness, tingling, and weakness in the hand and arm.
• Spinal cord compression or fractures.
• Vision changes or vision loss.

Early treatment of acromegaly can prevent these complications or keep them from becoming worse. Without treatment, acromegaly and its complications can lead to early death.

Diagnosis

Diagnosis involves the steps that your healthcare professional takes to find out if you have acromegaly. Your healthcare professional asks about your health history and does a physical exam. You also may need the following tests:

• IGF-1 measurement. This blood test measures the level of IGF-1 in your blood. A high IGF-1 level can mean that the level of growth hormone also is high. This can be a clue for acromegaly.
• Growth hormone suppression test. This blood test measures your growth hormone level before and after you drink a type of sugar water called glucose. In people who don't have acromegaly, the glucose drink typically causes the growth hormone level to fall. But if you have acromegaly, your growth hormone level tends to stay high.
• Imaging tests. Magnetic resonance imaging (MRI) can help pinpoint the location and size of a tumor in your pituitary gland. If no pituitary tumors are seen, you may need more imaging tests to look for other types of tumors. Your healthcare professional also may recommend X-rays of the hands and feet. These can help check for bone growth.

Treatment

Acromegaly treatment aims to improve symptoms and treat or prevent complications. The goal is to lower growth hormone and IGF-1 back to their proper levels and keep them there.

To help lower your growth hormone (GH) and IGF-1 levels, treatment options often include:

• Surgery to remove the tumor that's causing symptoms. Most often, this is the first treatment for acromegaly that's caused by a pituitary gland tumor.
• Medicine to help lower hormone levels. This is usually an option if surgery doesn't bring down growth hormone to the right level.
• Radiation to shrink the size of the tumor. Often, this a treatment choice if surgery isn't an option. It's also an option if surgery can't remove the whole tumor or if medicine doesn't help enough.

Some people need a mix of these treatments. Your treatment plan depends on factors such as:

• The location and size of your tumor.
• How serious your symptoms are.
• Your age and overall health.

It's common for some changes in physical features to improve with treatment. For example, swelling of soft tissue often goes down. And the skin often becomes less oily and coarse. But enlarged bones don't return to the size that they used to be.

If you also have other health conditions due to acromegaly, you may need other treatments to manage them.

Surgery or other procedures

Surgeons can remove most pituitary tumors using a method called transsphenoidal surgery. A surgeon works through the nose to remove the tumor from the pituitary gland. If the tumor causing symptoms isn't located on the pituitary gland, the surgeon recommends another type of surgery to remove the tumor.

Removing the tumor often returns growth hormone to the right level, especially if the tumor is small. If the tumor was putting pressure on the tissues around the pituitary gland, removing the tumor also helps relieve headaches and vision changes.

Sometimes, surgeons can't remove the whole tumor. When this happens, the level of growth hormone may still be too high after surgery. Another surgery, medicines or radiation treatments may be needed.

Medicines

Medicine can help lower hormone levels or block the hormones' effects. Your healthcare professional may recommend one or more of the following:

• Medicines called somatostatin analogues that cause the body to make less growth hormone. In the body, a brain hormone called somatostatin limits the production of growth hormone. The medicines octreotide (Mycapssa, Sandostatin) and lanreotide (Somatuline Depot) are lab-made versions of somatostatin. Taking one of these medicines signals the pituitary gland to make less growth hormone. That also helps lower IGF-1. These medicines also might make a pituitary tumor smaller. You take the medicines by mouth or receive a monthly shot.
• Medicines called dopamine agonists that lower hormone levels. The medicines cabergoline and bromocriptine (Cycloset, Parlodel) may help lower levels of GH and IGF-1 in some people. These medicines also may help make a tumor smaller. You take dopamine agonists by mouth, and the doses often are high. That can raise the risk of side effects. Side effects can include upset stomach, vomiting, stuffy nose, tiredness, dizziness, sleep problems and mood changes.
• Medicine called a growth hormone antagonist that blocks the action of growth hormone. The medicine pegvisomant (Somavert) blocks the effect of growth hormone on the body's tissues and results in lower IGF-1 levels. Pegvisomant doesn't lower the level of growth hormone or shrink tumor size. It may affect the liver, so your healthcare professional monitors your liver health with blood tests while you're on pegvisomant. The medicine is given as a daily shot and may be used along with other medicines.

Therapies

Radiation therapy destroys any leftover tumor cells after surgery to remove the tumor. It also slowly lowers the level of growth hormone. It may take months or years for radiation to improve acromegaly symptoms in ways that you notice.

Radiation often lowers levels of other pituitary hormones too — not just growth hormone. If you get radiation, you'll likely need regular follow-up healthcare visits. These visits let your healthcare professional check your hormone levels and make sure that your pituitary gland is working right. Follow-up care may last for the rest of your life.

Types of radiation therapy include:

• Stereotactic radiosurgery. Stereotactic radiosurgery uses 3D imaging to deliver a beam of high dose of radiation to the tumor cells. This treatment also limits the amount of radiation to the healthy tissue that surrounds the tumor. Most often, stereotactic radiosurgery is given in a single dose. This type of radiation may bring growth hormone back to the right level within 5 to 10 years. The most common technique that healthcare professionals use to give this type of radiation is called Gamma Knife. It doesn't involve the use of a knife.
• Proton beam radiation. This type of radiation uses tiny particles called protons that point to the tumor. It may cause less damage to the pituitary gland and the tissue that surrounds it than does conventional radiosurgery.
• Conventional radiation therapy. This type of radiation involves receiving small doses of radiation over 4 to 6 weeks. The risk of damage to the pituitary gland is higher than it is with proton beam radiation and stereotactic radiosurgery. You may not see the full effect of conventional radiation therapy for 10 or more years after treatment.

Preparing for an appointment

You'll likely first see your primary healthcare professional. Or you may be referred right away to a doctor called an endocrinologist who finds and treats hormone conditions.

It's good to prepare for your appointment. Here's some information to help you get ready and to know what to expect from your healthcare professional.

What you can do

• Be aware of any restrictions before the appointment. When you make the appointment, ask if there's anything you need to do to prepare for tests. For instance, you may be asked to stop eating for a certain number of hours before a test. This is called fasting.
• Write down your symptoms. Keep track of anything that causes you discomfort or concern. This may include headaches, vision changes or discomfort in your hands. Write down all of your symptoms, even if they don't seem related to the reason for which you made the appointment.
• Write down key personal information, including any changes in your sex life or menstrual cycle.
• Make a list of all medicines, vitamins and supplements you're taking.
• Take along old pictures that your healthcare professional can use to compare against your appearance today. Your healthcare professional likely will be interested in photos from 10 years ago through the present.
• Take along a family member or friend if you can. This person may remember something that you miss or forget.
• Write down questions to ask your healthcare professional.

Making a list of questions helps you make the most of your time with your healthcare professional. For acromegaly, some basic questions to ask include:

• What's the most likely cause of my symptoms? Are there other possible causes?
• What tests do I need? And should I see a specialist?
• What treatments are available for this condition? Which approach do you recommend?
• How long will I need treatment before my symptoms improve? Could treatment help me look and feel as I did before I developed symptoms of acromegaly?
• Will I have long-term complications from this condition?
• I have other health conditions. How can I best manage the conditions together?
• Are there brochures or other printed material I can take with me? What websites do you recommend?

Feel free to ask any other questions you have.

What to expect from your doctor

Your healthcare professional is likely to ask you questions such as:

• What are your symptoms, and when did they start?
• Have you noticed any changes in how you look?
• How much would you say your features have changed over time? Do you have old photos I can use for comparison?
• Have you noticed changes in your sex life, or sleep? Do you have headaches or joint pain, vision changes, or more sweating than usual?
• Does anything seem to make your symptoms better or worse?
• Do your old shoes and rings still fit? If not, how much has their fit changed over time?
• Have you had a colon cancer screening test?