Overview of Acromegaly Treatment

Acromegaly is a rare and chronic hormonal disorder caused by the excessive production of growth hormone (GH) by the pituitary gland, a small gland located at the base of the brain. This overproduction of GH leads to the abnormal and uncontrolled growth of bones and tissues throughout the body.

One of the hallmark characteristics of acromegaly is the enlargement of certain body parts, such as the hands, feet, and facial features. This results in a distinctive and often pronounced physical appearance. Additionally, individuals with acromegaly may experience various health complications, including joint pain, cardiovascular issues, diabetes, and organ enlargement.

The most common cause of acromegaly is the presence of a noncancerous tumor called a pituitary adenoma within the pituitary gland. These tumors secrete excess GH, disrupting the body's hormonal balance.

Without proper diagnosis and treatment, acromegaly can lead to significant health problems and reduced life expectancy. Early detection and appropriate medical interventions, such as surgery, medication, or radiation therapy, are crucial for managing the condition. In this article, we will take a look into the treatment options available for acromegaly and answer common questions you may have.

The pituitary gland resides within a bony cavity formed by the sphenoid bone, situated at the base of the skull, behind the eyes. In cases of pituitary adenomas, these tumors can exert pressure on the optic nerves, leading to visual disturbances or even vision loss.

These potentially devastating symptoms can significantly impact a patient's activities of daily living, ability to safely operate a motor vehicle, and overall quality of life. Consequently, the significance of addressing this issue through treatment becomes evident.

Transsphenoidal surgery is an operative technique specifically designed to access the base of the skull through the nasal passage. This approach is frequently employed to remove tumors located near the pituitary gland, the vital organ responsible for regulating hormones.

The procedure involves inserting a small camera (endoscope) and instruments through the nasal passage, followed by the careful removal of the sphenoid bone beneath the brain, at the skull base, to access and remove the tumor. This minimally invasive method leaves no obvious scar, mitigates the direct risks of exposing brain tissue, and facilitates an efficient recovery process.

After undergoing transsphenoidal surgery, your post-operative care will involve monitoring either in the intensive care unit (ICU) or a standard hospital ward. In the immediate period following the procedure, you may experience nasal congestion, which can make regular breathing somewhat challenging.

Breathing through your mouth may feel more comfortable during this initial period. Additionally, you might notice nasal drainage tinged with blood, occasional episodes of nausea, or headaches, all of which typically resolve naturally or are managed with prescribed medications.

Typically, the hospital stay lasts for a duration of 1 to 2 days. Upon returning home, it is essential to follow the post-operative instructions provided by your surgeon diligently.

You will likely have a follow-up appointment scheduled with your neurosurgeon within 1 to 2 weeks after the surgery to monitor your recovery progress and address any questions or concerns.

In cases where the tumor was unable to be fully excised, if it reoccurs, or if surgery is considered too hazardous, healthcare providers may advise medications as a potential treatment option.

There are three categories of medications currently used for the treatment of acromegaly: somatostatin analogs, dopamine agonists, and growth hormone antagonists.

Somatostatin analogs (Octreotide, Lanreotide): These medications operate by emulating the actions of somatostatin, a natural hormone that inhibits the release of growth hormone and other hormones from the pituitary gland. Octreotide and lanreotide are typically given every four weeks via injection beneath the skin or into the muscle, most commonly in the thigh or outer aspect of the buttocks. These drugs effectively reduce blood growth hormone levels, leading to symptom improvement, and can shrink pituitary tumors in some cases.

Though generally well-tolerated, they may cause side effects such as abdominal discomfort, nausea, diarrhea, and injection site reactions. Prolonged use may also be associated with gallstones or impaired glucose tolerance in some individuals.

Dopamine agonists (Cabergoline, Bromocriptine): Dopamine agonists suppress growth hormone production, though their precise mechanism of action remains unclear. Dopamine agonists may be used alone or with other medications like somatostatin analogs.

These medications, taken orally, can be a valuable alternative for patients intolerant to somatostatin analogs or when these analogs prove ineffective. Common side effects include nausea, vomiting, headaches, dizziness, fatigue, mood fluctuations, and sleep disturbances.

Growth Hormone Antagonists (Pegvisomant): These medications bind to growth hormone receptors, and help to reduce the production of insulin-like growth factor 1 (IGF-1), responsible for many acromegaly symptoms.

Pegvisomant is typically administered via injection and has proven effective in alleviating symptoms, such as enlarged hands, feet, and facial features. Pegvisomant can even improve glucose levels in patients with acromegaly-related diabetes, with nearly 90% of patients showing normalization of their IGF-1 levels in recent studies.

While generally well-tolerated, pegvisomant may induce side effects like headaches, fatigue, and elevated liver enzyme levels. The cost of the medication and the necessity for regular monitoring of IGF-1 levels to ensure appropriate dosing are important considerations.

Radiation therapy can be administered in two ways: through multiple shorter sessions with smaller doses, known as fractionated radiotherapy, or in a single extended session with a higher dose, referred to as stereotactic radiosurgery.

The term "stereotactic" denotes image-guided techniques that ensure precise targeting of radiation beams to a specific body location. While not a surgical procedure, the term "radiosurgery" underscores its remarkable precision.

Stereotactic radiosurgery, including systems like the Gamma Knife and linear accelerator-based methods, can be employed to precisely deliver radiation to the pituitary adenoma responsible for excessive growth hormone production in acromegaly. This radiation helps reduce the size of the tumor and curtail the overproduction of growth hormone, thereby alleviating acromegaly symptoms.

Typically used in combination therapy and after other treatment approaches, the effectiveness of radiosurgery as a standalone therapy lacks definitive evidence.

Nevertheless, studies have noted a tumor size reduction of 50% or more with its use, along with a significant decrease in excessive hormone secretion in up to 85% of cases treated with radiation therapy.

The rate of progression in acromegaly can significantly vary from person to person. Typically, acromegaly unfolds slowly, with symptoms often developing over several years or even decades.

This gradual onset of acromegaly can pose a diagnostic challenge, as it may take approximately 5 to 10 years from the emergence of symptoms for an accurate diagnosis to be made.

The prognosis for individuals with acromegaly is intricately linked to the effectiveness of treatment. In the absence of proper intervention, acromegaly can give rise to substantial health complications and potentially lead to premature mortality.

Conversely, successful treatment frequently leads to an improvement in symptoms or their complete resolution. Through appropriate management, life expectancy can be restored to normal levels, allowing individuals to enjoy healthier and longer lives.

Observation therapy, also known as, "watchful waiting," may be considered for certain cases of acromegaly where the tumor is very small and not causing significant symptoms or hormonal imbalances. In these instances, regular monitoring through medical assessments, including imaging studies and hormone level tests, is conducted to track the tumor's growth and hormone production.

The goal is to determine if the condition remains stable or progresses, at which point more active treatment approaches like surgery, radiation therapy, or medication may be initiated. Observation therapy is generally reserved for carefully selected individuals who meet specific criteria, as it aims to strike a balance between minimizing intervention and ensuring timely treatment when necessary: mild symptoms, elderly patients, small tumors, or patient preference.

• Transsphenoidal surgery serves as a common treatment approach for acromegaly.
• Following the surgery, patients receive careful monitoring in the recovery room, with close attention to vital signs.
• Stereotactic radiosurgery offers a precise means of delivering targeted radiation to the pituitary tumor responsible for excessive growth hormone production.
• Medications employed in acromegaly treatment function by either reducing the production of growth hormone or inhibiting its effects.
• Depending on the individual circumstances, a combination of surgery, radiation therapy, and medications may be used.
• In cases where acromegaly symptoms are mild and minimally impacting the patient's quality of life, observation may be considered as a potential approach.

• Pituitary Network Association: Acromegaly
• The Pituitary Foundation [UK]: Acromegaly
• National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK): Acromegaly
• National Health Service (NHS) [UK]: Acromegaly