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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Craniopharyngioma


Other Names for this Disease
  • Adamantinoma
  • Adamantinomatous tumor
  • Craniopharyngeal duct tumor
  • Dysodontogenic epithelial tumor
  • Rathke's pouch tumor
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Your Question

I underwent surgery for a craniopharyngioma last year. Although my cognitive symptoms are improved, I continue to suffer from hypersomnolence. Can you provide me with information?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is a craniopharyngioma?

A craniopharyngioma is a slow-growing benign tumor that develops near the pituitary gland (a small endocrine gland at the base of the brain) and the hypothalamus (a small cone-shaped organ connected to the pituitary gland by nerves).[1][2][3] This tumor most commonly affects children between 5 and 10 years of age; however, adults can sometimes be affected.[1] Craniopharyngiomas are thought to arise from remnants of the craniopharyngeal duct and/or Rathke cleft or from metaplasia (abnormal transformation of cells) of squamous epithelial cell remnants of the stomadeum.[orphanet] Craniopharyngioma is treated with surgery alone or by surgery followed by radiation.[4]

Last updated: 4/3/2009

What symptoms may be associated with craniopharyngioma?

Craniopharyngioma causes symptoms in three different ways:[1]
  • 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.[1]

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.[1]

Most patients have at least some visual defects and evidence of decreased hormone production at the time of diagnosis.[1]

Last updated: 4/3/2009

What causes craniopharyngioma?

Craniopharyngiomas are thought to arise from epithelial remnants of the craniopharyngeal duct or Rathke's pouch (adamantinomatous type tumours) or from metaplasia of squamous epithelial cell rests that are remnants of the part of the stomadeum that contributed to the buccal mucosa (squamous papillary type tumours).[5]
Last updated: 4/3/2009

How might craniopharyngiomas be treated?

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.[1]
Last updated: 4/3/2009

What is the prognosis for individuals with craniopharyngioma?

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.[1]
Last updated: 4/3/2009

Is there an association between craniopharyngioma and cataplexy and/or narcolepsy?

Several journal articles describe an association between craniopharyngioma and cataplexy (sudden loss of muscle tone) and/or narcolepsy. Most of these attribute the hypersomnolence to damage in the hypothalamus.[6][7][8]

To read more about the association between craniopharyngioma and cataplexy and/or narcolepsy, visit the following link to articles from PubMed, a searchable database of medical literature.
Last updated: 4/3/2009

References