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Craniopharyngioma

 

Definition

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

Causes

This tumor most commonly affects children between 5 and 10 years of age; however, adults can sometimes be affected. Boys and girls are equally likely to develop this condition.

Symptoms

Craniopharyngioma causes symptoms in three different ways:

  • by increasing the pressure on the brain (intracranial pressure)
  • by disrupting the function of the pituitary gland
  • by 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 and urination (diabetes insipidus) and stunted growth. When the optic nerve is damaged by the tumor, vision problems develop. These defects are often permanent, and may be worse after surgery to remove the tumor.

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

Signs and tests
  • CT scan and/or MRI scan of the brain
  • A thorough neurological examination
  • Endocrine hormone evaluations to look for any imbalances
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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. 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.

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.

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.

Treatments

Traditionally, surgery has been the main treatment for craniopharyngioma. However, radiation treatment instead of 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, then even a biopsy may not be necessary, if treatment with radiation alone is planned.

This tumor is best treated at a center with experience managing craniopharyngiomas.

Prevention


Review Date: 4/23/2004
Reviewed By: Samuel Blackman, M.D., Department of Pediatrics , Cincinnati Children's Hospital Medical Center, Cincinnati, OH. Review provided by VeriMed Healthcare Network.
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