Craniopharyngioma

Definition

A craniopharyngioma is a benign tumor that develops near the pituitary gland (a small endocrine gland at the base of the brain).

Alternative Names

Causes, incidence, and risk factors

This tumor most commonly affects children 5 - 10 years of age. Adults can sometimes be affected. Boys and girls are equally likely to develop this condition.

Symptoms

Craniopharyngioma causes symptoms in the following ways:

• Increasing the pressure on the brain (intracranial pressure)
• Disrupting the function of the pituitary gland
• Damaging the optic nerve

Increased pressure on the brain causes headache, nausea, vomiting (especially in the morning), and difficulty with balance.

Damage to the pituitary gland causes hormone imbalances that can lead to excessive thirst, excessive urination, and stunted growth.

When the optic nerve is damaged by the tumor, vision problems develop. These defects are often permanent, and may get worse after surgery to remove the tumor.

Behaviorial and learning problems may be present.

Most patients have at least some vision problems and evidence of decreased hormone production at the time of diagnosis.

Signs and tests

• Endocrine hormone evaluations to look for any imbalances
• CT scan or MRI scan of the brain
• Neurological examination

Treatment

Traditionally, surgery has been the main treatment for craniopharyngioma. However, radiation treatment instead of surgery or along with a smaller surgery may be the best choice for some patients.

In tumors that cannot be removed completely with surgery alone, radiation therapy is usually necessary. If the tumor has a classic appearance on CT scan, a biopsy may not be necessary if treatment with radiation alone is planned.

Stereotactic radiosurgery is performed at some medical centers.

This tumor is best treated at a center with experience in treating patients with craniopharyngiomas.

Support Groups

Expectations (prognosis)

In general, the prognosis for patients with craniopharyngioma is good, with an 80-90% chance of permanent cure if the tumor can be completely removed with surgery or treated with high doses of radiation. Most recurrences occur within the first 2 years after surgery.

However, the prognosis for an individual patient depends on several factors, including the ability of the tumor to be completely removed, and the neurological deficits and hormonal imbalances caused by the tumor and the treatment.

Most of the problems with hormones and vision do not improve with treatment, and sometimes the treatment may even make them worse.

Complications

A significant percentage of patients have long-term hormonal, visual, and neurological problems following the treatment of craniopharyngioma.

In patients where the tumor is not completely removed, the condition may recur.

Calling your health care provider

Call your health care provider if there are any signs of increased intracranial pressure (headache, nausea, vomiting, imbalance), any visual changes, increased thirst and urination, or poor growth in a child.

Prevention

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References

Maity A, Pruitt AA, Judy KD, Phillips PC, Lustig R. Cancer of the central nervous system. In: Abeloff MD, Armitage JO, Niederhuber JE, Kastan MB, McKenna WG, eds. Abeloff’s Clinical Oncology. 4th ed. Philadelphia, Pa: Elsevier Churchill Livingstone; 2008:chap 70.