Neurosurgery

Craniopharyngioma

What do I have?

A craniopharyngioma is not a pituitary tumor per se, but rather a tumor that develops near the pituitary gland and above it, at the base of the brain near the optic nerves that connect the eyes to the brain. Therefore, it can cause a wide variety of hormone and neurologic problems, and treating them requires surgical and medical expertise. In addition, many of these tumors present in children, and the hormonal problems caused by the tumor or by its treatment requires expert management by doctors in many different areas of medicine.

What does it do?

Craniopharyngioma is a benign tumor that will not spread like cancer, but because it has a tendency to grow insidiously and surround very important nerves and blood vessels at the base of the brain, as well as near the pituitary gland and hypothalamus, 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.

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?

Unlike pituitary adenomas, craniopharyngiomas are thought to come from an abnormal remnant or “left-over” of when the pituitary gland and hypothalamus were being created during development before birth. As a consequence, it is likely that the tumor has been present in some form since birth, but for whatever reason it lay “dormant” until it started to grow and cause symptoms.

Can it be cured?

When the tumor is small and has not grown to involve important arteries or nerves near the pituitary gland, it can be removed safely and completely, although the concern always exists that microscopic parts of the tumor may remain. For that reason, even after a complete removal, patients need to have yearly MRI scans to make sure the tumor does not come back. If the tumor is large and deemed unsafe for complete removal, it may still be important to get out some of the tumor to relieve symptoms, and to also make what is left behind small enough to treat with radiation, which is usually very effective at preventing growth of what is left behind.

What caused it?

The cause of these tumors remains unknown, although it is thought to be a abnormality of development in the normal pituitary gland or hypothalamus. Because this is an abnormality in development, it is unlikely that anything after birth can be done to prevent their formation, although what causes them to grow and cause symptoms is an active area of research. As far as anyone can tell, there is no known genetic cause for these tumors.


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