Acromegaly is a serious condition that occurs when the body produces too much of the hormones that control growth. This causes abnormal enlargement of bone, cartilage, muscle, organs, and other tissues. This abnormal growth can cause serious disease and even premature death.

Acromegaly occurs when the body produces too much of any of the hormones that control growth. These include growth hormone (GH), growth hormone-releasing hormone (GHRH), and insulin-like growth factor 1 (IGF-1). Overproduction of these hormones is caused by certain types of tumors. In over 95% of cases, the tumor is an adenoma of the pituitary gland.

Acromegaly is a rare disease. In the United States, about 1 in every 20,000 people has the disease. It can affect people of any age, but is most often diagnosed in adults aged 40-45 years.

Many of the symptoms result from the enlargement of tissues caused by excess growth hormone and IGF-1 in the blood. Arthritis, back pain, and curvature of the spine (kyphosis) - Due to enlargement of bones and cartilage in joints Change in bite or chewing, or spreading out of teeth – Due to enlargement of jaw and other facial bones

Acromegaly can cause enlargement of body organs such as the 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 is not an easy diagnosis, and it is usually missed by primary care doctors. Some of the signs and symptoms of acromegaly are very distinctive. Others are subtle and can be missed or misinterpreted. They are not always recognized as acromegaly because they develop so slowly. Once the diagnosis is suspected, you are usually referred to a specialist in hormonal disorders (endocrinologist). Blood tests are used to help confirm the diagnosis.

Acromegaly can be put into remission. This means that the disease is stopped and many of the signs and symptoms reversed. But, acromegaly can be a lifelong disease. Drug and/or radiation therapy typically goes on for several years. Even after successful therapy, you will need to see your health care provider regularly to have your growth hormone and IGF-1 levels checked.

There is no known way to prevent acromegaly. Early detection is the best hope of preventing severe symptoms and complications.

Acromegaly itself is usually not fatal. The complications of acromegaly, such as heart problems, high blood pressure, and diabetes, can be life threatening. Successful treatment of acromegaly, however, will usually restore normal health.

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 2 ways: by normalizing production of growth hormone and IGF-1 and by reducing the effects of the pituitary tumor on the surrounding tissues. A secondary goal is to avoid damaging normal pituitary tissue. Which treatments are used for a specific individual depend on the cause of the disease.

Surgery alone does bring about remission for some people, but not everyone. “Remission” in this case means to return levels of growth hormone and IGF-1 to normal. Remission is different than cure in that the disease can come back from remission. If it is successful, this operation quickly relieves symptoms caused by the tumor pressing on adjacent tissue. Remission rates are about 80-85% for small adenomas (microadenomas) and 50-65% for large adenomas (macroadenomas).

Growth hormone receptor blockers are the newest category of drugs used for acromegaly. These drugs work by blocking 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. The only drug in this category that has been approved by the US Food and Drug Administration for acromegaly is pegvisomant (Somavert).

Treatments for acromegaly do work in a significant proportion of people. Unfortunately, however, the disease 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, and goiter, and sexual problems, are reversible with treatment.

Somatostatin analogs: octreotide (Sandostatin), octreotide LAR (Sandostatin LAR), lanreotide or lanreotide (Autogel; not currently available in the U.S.).

The goals of treatment in acromegaly include normalization of GH and IGF-1 levels, reduction in tumour size, prevention of tumour recurrence, and alleviation of significant comorbid features, particularly cardiovascular, pulmonary and metastatic derrangements.4,5 There has been considerable debate on the appropriate level of GH to be achieved when treating acromegaly.

Transsphenoidal surgical excision of the pituitary adenoma is usually first-line therapy in acromegaly. The procedure is associated with normalization of GH levels in approximately 90% of patients with well-defined microadenomas.8-11 Surgical remission rates are lower in patients with macroadenomas because of the large, fibrous, and frequently invasive nature of these tumours, which makes complete resection unlikely.

Sandostatin® is typically used as second-line treatment in patients whose GH levels remain elevated after surgery, or in those patients for whom surgical resection is not an option. 5 Based on the results of independent investigations, it has been proposed that it should also be considered as first-line treatment in patients with acromegaly caused by a macroadenoma, in whom the chances of surgical cure are reduced.

Occasionally, but not usually. Most patients have a macroadenoma (tumor greater than 1 cm) at the time of diagnosis. In this situation, surgery to remove as much of the tumor as possible is usually the first treatment. This is particularly important if the tumor is close to the eye nerves (optic chiasm). If the patient cannot undergo surgery, or there is no clear benefit of surgery, medical treatment, preferably with Sandostatin, is used.