Learn About Craniopharyngioma

View Main Condition: Brain Tumor

What is the definition of Craniopharyngioma?

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

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What are the causes of Craniopharyngioma?

The exact cause of the tumor is unknown.

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

What are the symptoms of Craniopharyngioma?

Craniopharyngioma causes symptoms by:

  • Increasing pressure on the brain, usually from hydrocephalus
  • Disrupting hormone production by the pituitary gland
  • Pressure or damage to the optic nerve

Increased pressure on the brain can cause:

  • Headache
  • Nausea
  • Vomiting (especially in the morning)

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

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

Behavioral and learning problems may be present.

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What are the current treatments for Craniopharyngioma?

The goal of the treatment is to relieve symptoms. Usually, 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 people.

In tumors that cannot be removed completely with surgery alone, radiation therapy is used. If the tumor has a classic appearance on CT scan, a biopsy may not be needed 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 craniopharyngiomas.

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What is the outlook (prognosis) for Craniopharyngioma?

In general, the outlook is good. There is an 80% to 90% chance of a cure if the tumor can be completely removed with surgery or treated with high doses of radiation. If the tumor returns, it will most often come back within the first 2 years after surgery.

Outlook depends on several factors, including:

  • Whether the tumor can be completely removed
  • Which nervous system problems and hormonal imbalances the tumor and treatment cause

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

What are the possible complications of Craniopharyngioma?

There may be long-term hormone, vision, and nervous system problems after craniopharyngioma is treated.

When the tumor is not completely removed, the condition may return.

When should I contact a medical professional for Craniopharyngioma?

Call your provider for the following symptoms:

  • Headache, nausea, vomiting, or balance problems (signs of increased pressure on the brain)
  • Increased thirst and urination
  • Poor growth in a child
  • Vision changes
Endocrine glands
What are the latest Craniopharyngioma Clinical Trials?
A Clinical and Genetic Investigation of Pituitary and HYPOTHALAMIC Tumors and Related Disorders

Summary: There is a variety of tumors affecting the pituitary gland in childhood; some of these tumors (eg craniopharyngioma) are included among the most common central nervous system tumors in childhood. The gene(s) involved in the pathogenesis of these tumors are largely not known; their possible association with other developmental defects or inheritance pattern(s) has not been investigated. The present...

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Phase 2 Study of the MEK Inhibitor MEKTOVI® (Binimetinib) for the Treatment of Pediatric Adamantinomatous Craniopharyngioma

Summary: MEKTOVI (binimetinib) is an oral, highly selective reversible inhibitor of mitogen-activated extracellular signal regulated kinase 1 (MEK1) and MEK2. The biological activity of binimetinib that has been evaluated bith in vitro and in vivo in a wide variety of tumor types In this Phase II, the drug will be used to treat pediatric patients diagnosed with recurrent Adamantinomatous Craniopharyngioma ...

What are the Latest Advances for Craniopharyngioma?
Analysis of Prognostic Factors, Extent of Resection, and Long-Term Outcome of Craniopharyngioma in Adults and Children.
Endoscopic transcortical expanded transforaminal transvenous transchoroidal approach to third ventricle lesion resection using an endoport.
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Minimally invasive endoscopic approaches to pediatric skull base pathologies.
Who are the sources who wrote this article ?

Published Date: April 29, 2022
Published By: Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

What are the references for this article ?

Styne DM. Physiology and disorders of puberty. In: Melmed S, Auchus RJ, Goldfine AB, Koenig RJ, Rosen CJ, eds. Williams Textbook of Endocrinology. 14th ed. Philadelphia, PA: Elsevier; 2020:chap 26.

Suh JH, Chao ST, Murphy ES, Recinos PF. Pituitary tumors and craniopharyngiomas. In: Tepper JE, Foote RL, Michalski JM, eds. Gunderson & Tepper's Clinical Radiation Oncology. 5th ed. Philadelphia, PA: Elsevier; 2021:chap 34.

Zaky W, Ater JL, Khatua S. Brain tumors in childhood. In: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Textbook of Pediatrics. 21st ed. Philadelphia, PA: Elsevier; 2020:chap 524.