Neurosurgery

Acromegaly and Gigantism

What do I have?

Acromegaly is a condition in which a benign tumor in the pituitary gland produces too much growth hormone in an adult who no longer needs growth hormone to grow. Gigantism is a similar condition, but is specific to children who are still able to grow abnormally in response to the abnormally high amount of growth hormone produced by the pituitary tumor. When these tumors occur in adults, they usually appear in middle age, and can result in a number of dangerous changes that can be easy to miss because these changes occur slowly.

What does it do?

The tumor produces abnormally high amounts of growth hormone (GH) that circulate in the blood, leading to slow but dramatic changes such as growth of the bones of the skull (causing changes in facial features and wide gaps between the teeth), growth of the hands and feet, growth of the tongue and throat tissue (causing sleep apnea), changes in the joints and ligaments throughout the body (often leading to joint pain and swelling, and in some cases carpal tunnel syndrome). These are the changes that patients can notice, and some of these changes, like sleep apnea, can be life threatening. Unfortunately, the most dangerous changes are not evident to patients, and these include changes in the heart that can lead to heart disease or sudden death, and changes in the colon that can lead to colon polyps which may degenerate into colon cancer. If left untreated, acromegaly can result in serious illness and death, but it is important to emphasize that almost all pituitary adenomas are benign tumors and not cancer.

In addition to the effects of having too much growth hormone, these tumors can eventually grow large enough to compress the normal pituitary gland, leading to loss of normal hormones that are important for normal life and reproduction. If the tumor gets very large, it can cause compression of other nerves or arteries that are located near the pituitary gland, especially the optic nerves which connect the eyes to the brain; this can cause slow but progressive loss of vision.

How long have I had it?

Often the changes associated with these tumors are subtle, and they are typically slow growing, so it is likely that the tumor has been there for years before it was discovered.

Can it be cured?

The majority of these tumors can be cured or controlled through a combination of surgery, radiation therapy, and medical treatment. The first line of treatment is surgery, unless the patient has other medical conditions that make surgery unsafe. If surgery is not possible, or if the whole tumor cannot be removed, then radiation therapy (often radiosurgery) is an option as well. Medical treatment also exists, and can be very effective in controlling or eliminating the risks associated with these tumors, but it requires close endocrinologic monitoring and adjustment.

What caused it?

For the majority of pituitary tumors, there is no known cause. These tumors appear to develop in a surprising number of patients without any clear reason. Recent work has suggested that there may be certain changes in the DNA of these tumors that lead to their formation, but their true cause remains elusive and is an active area of research.

In rare cases, these tumors may be part of a syndrome that can be inherited in families. For that reason, if anyone else in your family has ever had kidney stones, or has ever had a pituitary tumor, or has had any tumor of other hormone-producing organs (like the thyroid gland, the parathyroid gland, the adrenal glands, the pancreas, or the reproductive organs), let us know so that we can determine if your family is potentially a carrier for this inheritance.


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