Overview
Acromegaly is a hormonal disorder caused by excessive production of growth hormone by the pituitary gland in adults. Unlike in children, where excessive growth hormone leads to gigantism and increased height, adults with acromegaly do not experience a significant change in their overall height. When this occurs, the bones in the body, particularly in the hands, feet, and face, undergo enlargement.
Due to the gradual progression of physical changes associated with acromegaly, diagnosing the condition can sometimes be challenging and may take considerable time. If left untreated, elevated levels of growth hormone can affect not only the bones but also other body parts, potentially leading to severe health complications, some of which may be life-threatening. However, with appropriate treatment, symptoms can be significantly improved, including the excessive growth of facial features, and the risk of complications can be reduced.
Symptoms
A common manifestation of acromegaly is the enlargement of hands and feet. This can be observed when previously well-fitting rings become tight or no longer fit at all, and when there is a gradual increase in shoe size over time.
Acromegaly can also result in gradual changes to the appearance of your face, such as a protruding lower jaw and brow bone, a widened space between teeth, thicker lips, and a larger nose.
Early signs of acromegaly may not become apparent for years because of its tendency to progress slowly. Sometimes, people can only detect bodily changes by contrasting older and more recent images.
In general, the signs and symptoms of acromegaly might vary from person to person and may include any of the following:
- The hands and feet appear to be bigger
- Increase in the size of the facial bones, nose, lips, and tongue
- Skin that is thickened, coarse and oily.
- Excessive sweating
- Excessive body smell
- Skin tags
- Weakness of the muscle or joint and tiredness
- Joint pain and restricted movement
- Headaches that may be severe or chronic
- Abnormalities in women’s menstrual cycles
- Male erectile dysfunction
- Decreased sexual desire
- A deeper, husky voice brought on by larger sinuses and vocal cords
- Excessive snoring as a result of an upper airway obstruction
- Problems with eyesight
Contact your doctor to schedule an examination if you experience the acromegaly-related signs and symptoms.
Acromegaly typically takes time to develop. Even your family members might not first recognize the slow physical changes that this condition causes. However, an early diagnosis is crucial so you can begin receiving the right care. If untreated, acromegaly can cause major health issues.
Causes
When the pituitary gland consistently and excessively produces growth hormone (GH), acromegaly develops.
At the base of your brain, behind the bridge of your nose, is a little gland called the pituitary gland. It makes GH as well as several other hormones. GH is crucial in controlling your physical development.
IGF-1, also known as insulin-like growth factor-I or IGF-I, is a hormone that your liver produces in response to the pituitary gland’s release of GH into your bloodstream. Your bones and other tissues grow as a result of IGF-I. Too much GH causes too much IGF-1, which can result in the signs, symptoms, and problems of acromegaly.
The most frequent factor resulting in excessive GH production in adults is a tumor:
- Pituitary tumors. Most cases of acromegaly are caused by the presence of benign tumors known as pituitary adenomas. These tumors play a significant role in the development of acromegaly symptoms due to their excessive production of growth hormone. The signs and symptoms experienced by individuals with acromegaly, such as headaches and vision problems, are primarily a result of the tumor exerting pressure on nearby areas of the brain.
- Nonpituitary tumors. In rare cases, acromegaly can be caused by tumors originating from organs such as the pancreas or lungs. These tumors have the ability to release growth hormone (GH) into the bloodstream. Alternatively, the some tumors can stimulate the pituitary gland to increase GH production by releasing a hormone called growth hormone-releasing hormone (GH-RH). This abnormal interaction between tumors and the hormonal system can lead to the development of acromegaly.
Diagnosis
Your physician will inquire about your health history and perform a physical examination. Then, he or she might suggest taking the following actions:
- IGF-1 measurement. Your doctor will take a blood sample from you after you’ve fasted for the night in order to determine your blood’s IGF-1 level. IGF-1 levels above normal point to acromegaly.
- Growth hormone suppression test. The most reliable way to confirm an acromegaly diagnosis is using this test. In this test, the amount of GH in your blood is assessed both before and after you consume a sugar-containing preparation (glucose). The glucose drink usually lowers the GH level in patients without acromegaly. Acromegaly, however, causes your GH level to frequently remain high.
- Imaging. To determine the precise location and size of a tumor on your pituitary gland, your doctor could advise an imaging test, such as magnetic resonance imaging (MRI). Your doctor might schedule additional imaging exams to look for nonpituitary cancers if no pituitary abnormalities are visible.
Treatment
The treatment approach for acromegaly is tailored to each individual patient, taking into consideration factors such as the location, size, and characteristics of the tumor, the severity of symptoms, age, and overall health. The primary treatment options typically involve surgical intervention or radiation therapy to remove or reduce the size of the tumor responsible for the symptoms. Additionally, medications may be prescribed to help normalize hormone levels, specifically to lower growth hormone (GH) and insulin-like growth factor-1 (IGF-1) levels.
Depending on the specific health issues related to acromegaly, your doctor may also recommend additional therapies or interventions to help manage the challenges associated with the condition. The goal of treatment is to alleviate symptoms, reduce the tumor size, normalize hormone levels, and improve overall health and quality of life. It is important to consult with your healthcare provider to determine the most appropriate treatment plan for your specific situation.
Surgery
Transsphenoidal surgery is a surgical technique commonly employed to remove the majority of pituitary tumors. During this procedure, the surgeon accesses the pituitary gland by going through the nose. In cases where the tumor causing the symptoms is not located on the pituitary gland, your doctor may recommend a different type of surgery to remove the tumor.
Following tumor removal, especially if the tumor is small, GH levels typically return to normal. This surgical intervention can also help alleviate headaches and vision disturbances caused by the tumor’s pressure on surrounding tissues near the pituitary gland.
In some instances, it may not be possible for the surgeon to remove the entire tumor. If this is the case, even after surgery, GH levels may remain elevated. In such situations, your doctor may suggest additional surgery, medication, or radiation therapy as potential treatment options. The aim is to further manage and reduce GH levels.
Medications
To assist your hormone levels return to normal, your doctor may advise one of the following medications, or a combination of medications:
- Drugs that reduce growth hormone production (somatostatin analogues). Somatostatin, a brain hormone, inhibits the body’s ability to produce GH. Synthetic somatostatin is available as the medications octreotide and lanreotide. One of these medications tells the pituitary gland to make less growth hormone, and it might even shrink a pituitary tumor. Usually, a medical expert will inject these medications once a month into the gluteal muscles in your buttocks.
- Drugs to lower hormone levels (dopamine agonists). Some persons may benefit from a reduction in GH and IGF-1 levels after taking the oral drugs cabergoline and bromocriptine. These medicines might also aid in reducing tumor size. These drugs typically need to be taken in large doses to treat acromegaly, which raises the possibility of side effects. These medications frequently cause nausea, vomiting, stuffy noses, fatigue, dizziness, sleep issues, and mood swings.
- Drug to block the action of GH (growth hormone antagonist). A drug called pegvisomant stops the effects of GH on the body’s tissues. Pegvisomant may be quite helpful for folks who haven’t had significant success with other treatments. The daily injection of this medication can help lower IGF-1 levels and ease symptoms, but it has no effect on GH levels or tumor size.
Radiation
In cases where the surgeon was unable to fully remove the tumor during surgery, your doctor may recommend radiation therapy. Radiation therapy aims to eliminate any remaining tumor cells and gradually reduce GH levels. It is important to note that significant improvement in the symptoms of acromegaly may take several years after initiating radiation therapy.
During radiation therapy, not only GH but also other pituitary hormones may be affected, leading to their decreased production. Regular follow-up appointments with your doctor will likely be necessary to monitor the functionality of your pituitary gland and assess hormone levels. This ongoing care may be required for the rest of your life to ensure proper management of the condition.
Various radiation therapy forms include:
- Conventional radiation therapy. Over the course of four to six weeks, this kind of radiation therapy is typically administered every working day. For ten or more years following treatment, conventional radiation therapy may not have its full impact.
- Stereotactic radiosurgery. In stereotactic radiosurgery, a high radiation dose is given to the tumor cells while the radiation exposure to healthy surrounding tissues is kept to a minimum. Usually, it can be given in a single dose. Within five to ten years, this kind of radiation may return GH levels to normal.
