Acromegaly is a serious condition that occurs when the body produces too much growth hormone (GH), the hormone that controls growth. When your body has an excess in GH, it leads to irregular growth of bones, cartilage, muscles, organs, and other tissues. This can cause serious disease and even premature death.
Acromegaly is caused by an overproduction of the hormones that control growth.
These hormones include growth hormone, growth hormone-releasing hormone (GHRH), and insulin-like growth factor 1 (IGF-1). In most cases, a benign tumor is applying pressure to your pituitary gland, causing it to produce and distribute these hormones unnecessarily.
In rare cases, a tumor elsewhere in your body, such as the lungs, adrenal glands, or pancreas, can lead to an overproduction of growth hormone, resulting in symptoms of Acromegaly. Non-pituitary tumors found in other areas of the body like the abdomen and chest can also cause Acromegaly in extremely rare cases. The pituitary gland responds to a stimulus to produce GH. For example, some hypothalamic tumors may secrete growth hormone-releasing hormone (GHRH), which tells the body to produce GH.
Sometimes, Acromegaly can be caused by an increased growth hormone production from outside the pituitary gland, such as from neuroendocrine tumors like small cell lung cancer or carcinoid tumors.
No, in over 99% of patients, adenomas are NOT cancerous. Although these types of tumors are benign, they can create problems because of their size. Problems associated with pituitary tumors can lead to decreased vision, loss of normal pituitary function (hypopituitarism) and/or cause headaches due to excessive hormone production.
It is common for the jaw, forehead, and other parts of the face to enlarge. This is a visible and noticeable change to your appearance, but since it happens slowly over time, many people, including your primary care doctor, may not notice without comparing old photographs to your current state. Often, a friend or relative you haven’t seen in a few years or a new consulting doctor notices the change.
- Arthritis, back pain, spine curvature (kyphosis) – due to enlargement of your bones and cartilage in joints
- Swelling of your face, lips, and tongue
- Breathing problems during sleep – due to narrowing of your airway resulting from swelling
- Tiredness during the day – due to poor sleep
- Your skin thickens
- You experience overly oily skin or acne
- Increased sweating
- Tingling or numbness in your fingers or toes – due to entrapment of nerves by enlarging muscles
- Change in bite or chewing, or spreading out of teeth – due to enlargement of your jaw and other facial bones
- Large numbers of skin tags
- Vision loss, partial or complete
Pituitary tumors also can damage the gland itself, disrupting your hormone production. The resulting hormone imbalances are responsible for symptoms such as impotence, low sex drive, and changes in your menstrual cycle.
Acromegaly can cause enlargement of your organs such as your heart, thyroid gland, liver, and kidneys. Untreated, acromegaly is linked to early heart disease, high blood pressure, heart rhythm disorders, diabetes, and colonic polyps, a precursor of colon cancer.
People with acromegaly have almost twice the chance of dying prematurely as the general population. Successful treatment, however, will restore near-normal health in most individuals.
acromegaly that affects the heart or blood pressure or causes diabetes may have the following symptoms, although they may not occur in everyone with this rare disease.
- Increased thirst or urination
- Shortness of breath
- Chest pain
- Palpitations or rapid heart beat
- Poor exercise tolerance
Blood tests are used to help confirm your diagnosis. Measurement of IGF-1 level is considered the most reliable test for acromegaly. Another test measures levels of IGF-binding protein-3 (IGFBP-3), a protein that interacts with IGF-1. This test is usually used to help confirm the diagnosis of acromegaly.
Some health care providers measure how suppressible diabetic glucose levels. Blood glucose (sugar) suppresses growth hormone. The level of growth hormone in your blood is measured after you drink a sugary drink. The resulting high blood sugar level suppresses the growth hormone level in healthy people but not in people who have too much growth hormone.
A CT scan or MRI of the head is done to look for a pituitary tumor.
Treatments for acromegaly do work in a significant proportion of people. Unfortunately, however, the disorder is rarely caught early enough to prevent permanent damage or even premature death.
Bone changes of acromegaly are permanent. Many of the soft-tissue changes, such as swelling, enlarged tongue, thickened skin, acne, carpal tunnel syndrome, goiter, and sexual problems are reversible with treatment.
Complications such as high blood pressure, diabetes, and high triglycerides start to reverse when your levels of GH and IGF-1 are normalized. Correction of these complications can prevent more serious illness, such as stroke, heart disease, and death.
There are several treatment options in acromegaly. Whichever treatments are used, the goal is to relieve and reverse the symptoms of the disease. This is done in two ways: by normalizing your GH and IGF-1 production and by reducing the effects of your pituitary tumor on surrounding tissues. A secondary goal is to avoid damaging normal pituitary tissue. The treatments that are used for a specific individual depend on the cause of your acromegaly. For the large majority of cases caused by pituitary adenomas, treatments include surgery, drug therapy, and radiation therapy.
By removing your tumor, you will stop the overproduction of GH and other growth-stimulating hormones once the pressure has stopped being applied to your pituitary gland. The tumor is removed through an incision inside the nose. The operation is called transsphenoidal hypophysectomy. While technically not “brain surgery,” this is a very delicate operation calling for an experienced surgeon.
If your acromegaly goes into remission after surgery, there is usually no need for further treatment. (Some people, however, require lifelong hormone replacement after pituitary surgery.) GH and IGF-1 levels after surgery will indicate whether or not you need to continue to manage your symptoms with other treatments such as drugs and radiation. If these levels have not returned to normal, it is likely that you will require additional treatment.
Growth hormone receptor blockers represent the newest category of drugs for the treatment of acromegaly. These drugs block the places on cells where growth hormone “docks.” If the excess growth hormone in the blood can’t dock on a cell, it can’t cause abnormal growth of the cell.
Pegvisomant, the only U.S. Food and Drug Administration-approved treatment for acromegaly normalizes IGF-1 level in over 90% of people.
Somatostatin analogs act like a natural hormone, which stops the secretion of growth hormone. Octreotide, the most common drug in this group, works in most people who take it. You must inject yourself with this medication either three times per day under the skin or once per month in the muscle.
Dopamine agonists stop the secretion of growth hormone by stimulating dopamine (a chemical in the brain) activity.
Cabergoline continues to gain popularity due to the fact that its oral route administration and lower price tag than hormone receptor blockers or somatostatin analogs.
Drug therapy is available to treat the symptoms of acromegaly and shrink tumors in preparation for surgery. Some drugs block the production of growth hormones while others prevent GH from stimulating the production of IGF-1.
The final treatment option for symptoms of acromegaly that persist after surgery and drug therapy is radiation.
Radiation is often the first treatment for people who cannot undergo surgery because of other medical conditions or the size and/or the location of their tumor.
A majority of people who undergo radiation therapy permanently lose function of their pituitary gland. These people must take hormone replacement for the rest of their lives. Drug or radiation therapy usually goes on for years. Radiation therapy can take anywhere from 18 months to several years.
Surgery can remove your hormone-secreting tumor completely or partially. Even after surgery, you may require additional therapies.
Drug therapy, an effective way to treat the symptoms of acromegaly before and after surgery, can shrink tumors in preparation for surgery. Some drugs block the production of growth hormones, others prevent growth hormone from stimulating the production of IGF-1, and via dopamine, stimulation stops GH production in people with this rare disease.
Drug and/or radiation therapy typically goes on for several years. Even after successful therapy, you may want to see your health care provider regularly to check your GH and IGF-1 levels.
To send acromegaly into remission, care providers prescribe a combination of treatments since no one series of therapies works best for everyone and
Radiation serves as the final treatment option for the treatment of the symptoms that persist after surgery and drug therapy.
Radiation can be the first treatment for patients who cannot undergo surgery, due to the size and/or the location of their tumor and offers a solution for patients with other medical conditions that interfere with the surgery or the size and/or the location of their tumor.
The majority of people who undergo radiation therapy permanently lose function of their pituitary gland. These people must take hormone replacement for the rest of their lives. Drug or radiation therapy usually goes on for years. Radiation therapy can take anywhere from 18 months to several years.
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