“The good physician treats the disease; the great physician treats the patient who has the disease.”  –William Osler

Acromegaly is a rare disease with a wide range of symptoms, and many patients wait years before they are diagnosed and treatment is initiated. During this interval, serious complications can develop. Early diagnosis and treatment can lessen morbidity and mortality and can significantly improve patients’ quality of life.

Treatment of acromegaly may require the coordinated efforts of a team of specialists, including primary care physicians, endocrinologists, and neurosurgeons. Due to the variable nature of the disease, treatment must be individualized for each patient, based on that person’s symptoms. In general, the aims of treatment are to:

• reduce growth hormone (GH) production to normal levels
• treat hormone deficiencies
• relieve pressure the tumour responsible for the disease may be putting on surrounding tissues
• reduce mortality risk
• improve symptoms

Most patients will require surgery to remove or debulk a pituitary tumour. Some will require medication or radiotherapy either in place of surgery or in combination with it.

Surgery

Because surgery can result in rapid reduction in GH levels which, in turn, can alleviate symptoms, the primary form of treatment for the majority of acromegaly patients is transsphenoidal adenomectomy performed by a neurosurgeon who has experience with the procedure. However, success is closely correlated with the size and degree of the invasiveness of the tumour, along with the expertise of the surgeon.

When performed by experienced pituitary surgeons, microscopic or endoscopic transsphenoidal microsurgery results in endocrine remission of 90 percent for microadenomas and between 50 percent and 60 percent for macroadenomas. However, surgery may not be successful in resolving all symptoms (for example, headaches may persist).

Despite its rate of success, like any other surgery, it is not without risks. Complications from surgery may include bleeding, spinal fluid leak, meningitis, sodium and water imbalance, and hypopituitarism. Significant complications occur in about  5 percent of individuals.

In some patients, surgery improves hormone levels, but does not return them to normal, necessitating further treatment. Sometimes removal of the entire tumour may not be possible, resulting in GH levels that remain too high. In this situation, debulking of the tumour may be helpful.

Medical Therapy

Patients who are not candidates for surgery and those who need further treatment after surgery may respond to medical therapy. Using somatostatin analogs and GH-receptor antagonists is considered as principal therapy in these situations. Somatostatin receptor ligands, such as octreotide and lanreotide, have been used effectively for two decades. In selected patients, dopamine receptor agonists are another option.

Each of these medications has its own set of advantages and disadvantages, and specific risks and benefits must be carefully matched to individual patients.

Sometimes several different types of treatment are needed to achieve the desired results. In patients who fail to achieve biological control of their acromegaly after surgery and/or medical therapy, radiotherapy is considered to be the best curative method.

Radiotherapy

Radiotherapy is generally reserved for patients with tumours that have recurred following surgical removal and for patients with tumours that have persisted after surgery and have not responded to medical therapy.

There are two radiotherapy options:

• conventional external beam radiotherapy
• stereotactic radiotherapy (SRT) with the use of a gamma knife, which delivers a single radiation fraction to a small tumour target

Drawbacks of SRT are that it may not be available in all treatment locations and that it is not appropriate when there is a significant residual tumour burden or when the tumour is too close to the optic chiasm.

One advantage of radiotherapy is that it may lead to biochemical control, which can limit the need for lifelong medical therapy. However, it can take years for the full therapeutic effect to be achieved, and some patients may experience only a limited response. Therefore, medical therapy must be continued while awaiting the response from the radiotherapy. Periodic withdrawal of medical therapy for one to three months (depending on the specific drug treatment) for the assessment of GH and IGF-1 levels may be advisable.

In almost all cases of acromegaly, long-term treatment and monitoring are essential.

The Bottom Line

Diagnosis and treatment of acromegaly requires a multidisciplinary team for optimal disease management and improvement in patients’ quality of life. In addition, physicians should encourage better communication and collaboration between patients and their healthcare team.

Progress in both earlier diagnosis and in disease management have resulted in adequate hormonal control being achieved in the majority of cases, bringing the life expectancy of patients with acromegaly to a level comparable to that of the general population.

You can make a significant impact on your patients’ quality of life with the early diagnosis and prompt initiation of the right treatment. Learn more about acromegaly by taking a free course available at MDBriefCase:

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