What are the symptoms of acromegaly? What causes acromegaly?

Acromegaly is a hormonal disorder that develops as a result of the pituitary gland producing too much growth hormone during an individual's adult years.

acromegaly

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What is acromegaly?

Excessive growth hormone leads to enlargement of the bones. In childhood, this leads to a condition called gigantism, resulting in increased height. However, in adults, there is no change in height; instead, the increase in bone size is limited to the individual’s hands, feet, and facial bones, a condition known as acromegaly.

Acromegaly is not a common condition, and because physical changes occur slowly over years, it may take a long time to notice the condition. Untreated high levels of growth hormone can affect not only the individual’s bones but also other parts of the body and can lead to serious, and in some cases life-threatening, health problems. Proper treatment for acromegaly can reduce the individual’s risk of developing complications and significantly improve symptoms, including the enlargement of bones in the limbs.

Why Does Acromegaly Occur?

Acromegaly occurs when the pituitary gland produces significantly more growth hormone than normal for an extended period. The pituitary gland, located beneath the brain and behind the nose, is a small gland responsible for producing various hormones, including growth hormone. Growth hormone plays a crucial role in regulating an individual’s physical growth.

When the pituitary gland releases growth hormone into the bloodstream, it triggers the production of a hormone called insulin-like growth factor 1 (IGF-1) in the individual’s liver. Insulin-like growth factor 1 is a hormone that promotes the physical growth of bones and other tissues in the body. Excessive production of growth hormone leads to an overproduction of insulin-like growth factor 1, which can result in the symptoms and complications of acromegaly.

The most common cause of excessive growth hormone production in adults is a tumor that develops in or affects the pituitary gland.

The majority of acromegaly cases are caused by a benign tumor called an adenoma that develops on the pituitary gland. The tumor produces an excess amount of growth hormone, leading to many of the symptoms and signs of acromegaly. Some symptoms of acromegaly, such as headaches and visual disturbances, result from the tumor exerting pressure on nearby brain tissues.

In some cases of acromegaly, tumors that develop in other areas of the body, such as the lungs or pancreas, can cause the disorder. These tumors may either spontaneously produce growth hormone or produce another hormone called a growth hormone-releasing hormone.

What are the potential complications of acromegaly?

Untreated acromegaly can lead to significant health problems. Symptoms and complications that may develop due to untreated acromegaly include high blood pressure (hypertension), high cholesterol, enlargement of the heart (cardiomyopathy), heart problems, osteoarthritis, type 2 diabetes, enlargement of the thyroid gland (goiter), precancerous growths in the intestinal lining (polyps), sleep apnea (pauses in breathing during sleep), carpal tunnel syndrome, increased risk of developing cancerous tumors, compression or fractures due to bone growth in the spine, and changes in vision or vision loss in later stages.

Early treatment of acromegaly can prevent the development or worsening of these complications. Untreated acromegaly and its complications can progressively worsen and lead to premature death.

What are the symptoms and types of acromegaly?

The most common symptom of acromegaly is enlargement in the hands and feet. For instance, an individual may notice that they no longer fit into rings they used to wear or that their shoe size has gradually increased over time.

Acromegaly can also lead to gradual changes in the shape of a person’s face, such as protrusion of the lower jaw and forehead bone, an enlarged nose, thicker lips, and widening gaps between teeth.

Because acromegaly tends to progress slowly, its early symptoms may not be apparent for many years. Sometimes individuals may only notice physical changes by comparing old photographs with more recent ones.

The symptoms and signs of acromegaly can vary from person to person. Common signs and symptoms of acromegaly include:

Growth in the bones of the hands and feet
Enlargement of facial bones, lips, nose, and tongue
Excessive sweating and body odor
Deepening of the voice due to enlarged vocal cords and sinuses
Hoarse voice
Thickened, patchy areas of skin
Joint stiffness and pain
Vision problems
Coarse, oily, thickened skin
Persistent or severe headaches
Severe snoring due to upper airway obstruction
Fatigue with joint or muscle weakness
Irregular menstrual cycles in women
Loss of interest in sex and erectile dysfunction in men

Individuals who notice the presence of symptoms and signs associated with acromegaly should see a doctor for examination to begin the treatment process before facing serious and severe complications. Acromegaly typically develops slowly. In some cases, even family members may not initially notice the gradual physical changes associated with this disorder. However, early diagnosis is important for individuals to start receiving appropriate care.

How is acromegaly diagnosed?

To diagnose acromegaly, the doctor first conducts a physical examination and gathers information about the patient’s medical history. During this examination process, the individual should discuss all observed symptoms with the doctor and share information about any other conditions or illnesses they may have. For the diagnosis of acromegaly, the doctor may initially request a blood test.

For the diagnosis of acromegaly, the doctor may initially request a blood test. After fasting overnight, a blood sample is taken to measure the level of insulin-like growth factor 1 hormone in the blood. A high level of insulin-like growth factor 1 observed after the test indicates the possibility of acromegaly.

The most reliable method to confirm the diagnosis of acromegaly is the growth hormone suppression test. During this test, the levels of growth hormone in the individual’s blood are measured before and after drinking a sugary liquid. In individuals without acromegaly, the sugary drink typically causes a decrease in growth hormone levels. However, in individuals with acromegaly, there tends to be a tendency for growth hormone levels to remain high.

The doctor may recommend imaging tests such as magnetic resonance imaging (MRI) to determine the location and size of a tumor in the pituitary gland, which can help confirm the diagnosis of acromegaly. If a pituitary tumor is not seen, the doctor may request other imaging tests to look for tumors elsewhere in the body where the pituitary gland is not located.

How is acromegaly treated?

Treatment for acromegaly varies for each individual. The treatment plan will likely depend on factors such as the location and size of the tumor, the severity of symptoms, the individual’s age, and overall health. Treatment options for acromegaly typically include surgery to remove or reduce the tumor causing the symptoms, radiation therapy, and medications to help normalize hormone levels.

Doctors may recommend additional therapies to help manage complications experienced by individuals due to acromegaly-related complications.

Doctors can remove most pituitary tumors through a method called transsphenoidal surgery. During this procedure, the surgeon works through the individual’s nostrils to remove the tumor from the pituitary gland. If the tumor causing the symptoms is not located in the pituitary gland, the doctor may recommend a different type of surgical intervention to remove the tumor.

The removal of the tumor, especially in cases where the tumor is small, typically restores growth hormone levels to normal values. Removing the tumor also helps alleviate symptoms such as headaches and changes in vision if the tumor compresses surrounding tissues in the pituitary gland.

In some cases, the tumor may not be completely removed during surgery. In such cases, the individual may still have high levels of growth hormone after surgery. In this situation, the doctor may recommend additional surgery, medication, or radiation therapy options.

Your doctor may recommend various medications or combinations of medications to help normalize your hormone levels. Medications that reduce growth hormone production are used for this purpose. A brain hormone called somatostatin works against growth hormone production and suppresses it. A synthetic version of this hormone is available. Using one of these medications signals the individual’s pituitary gland to produce less growth hormone. These medications can also shrink the size of a pituitary tumor. These medications are typically injected into the hip muscles (gluteal muscles) once a month by a healthcare professional.

Medications that lower hormone levels may help lower levels of growth hormone and insulin-like growth factor 1 hormone in some individuals. These medications can also help reduce the size of the tumor present in the individual. In the treatment process of acromegaly, these medications generally need to be taken in high doses, which can increase the risk of side effects. Common side effects of medications that lower hormone levels include dizziness, nausea, vomiting, nasal congestion, sleep problems, mood changes, and fatigue.

Medications that directly block the effects of growth hormone inhibit the hormone’s direct effects on body tissues. These medications can be very beneficial for individuals in whom other treatment modalities have not been successful. These types of medications, given as a daily injection, can help lower insulin-like growth factor 1 hormone levels and alleviate symptoms. However, these types of medications generally cannot permanently lower growth hormone levels or shrink tumor size.

In cases where the tumor is only partially or not removed during surgery, the doctor may recommend radiation therapy, also known as radiotherapy. Radiation therapy destroys the remaining tumor cells and gradually reduces growth hormone levels. It may take years for this treatment to significantly improve acromegaly symptoms.

Radiation therapy not only lowers growth hormone but also other pituitary hormone levels. Therefore, individuals undergoing radiation therapy should have regular follow-up appointments with their doctors to ensure that their pituitary glands are functioning properly and hormone levels are controlled. This follow-up care may continue permanently in some cases. Types of radiation therapy include conventional radiation therapy and stereotactic radiosurgery.

Conventional radiation therapy is typically administered every weekday for four to six weeks. The full effects of conventional radiation therapy may not be evident until ten years after treatment.

Stereotactic radiosurgery delivers high doses of radiation to target tissues while using 3D imaging technologies to limit damage to surrounding tissues. This type of treatment can often be given in a single dose. These types of radiation therapies can return growth hormone levels to normal within five to 10 years.