A craniopharyngioma (kray-nee-oh-fair-in-jee-OH-muh) is a rare type of noncancerous (benign) brain tumor that usually forms near the base of the pituitary gland.
When a human embryo is developing in the womb, there is a small depression called Rathke's pouch in the roof of the mouth. As the fetus develops, Rathke's pouch becomes one part of the pituitary gland, a small organ located near the bottom of the brain.
In rare cases, remnants of Rathke's pouch develop into benign tumors. The tumors, called craniopharyngiomas, don't cause cancer but can cause problems in the brain and pituitary gland.
Craniopharyngiomas can grow and press on the brain, pituitary gland, optic nerve (the nerve the connects the eye to the brain), or fluid-filled spaces within the brain. This can interfere with the production of hormones, affect the normal working of the brain, and cause headaches, slow growth, delayed puberty, and vision problems. Craniopharyngiomas are most common in children ages 5-14, but they can affect people of any age.
Surgery to totally remove a craniopharyngioma can sometimes damage surrounding parts of the brain and cause a permanent shortage of some hormones. Therefore, for most patients, a neurosurgeon first removes part of the craniopharyngioma, and then uses radiation therapy for any remaining tumor. This approach usually results in effective treatment of the tumor and better quality of life for the patient.
The overall survival rate for people with craniopharyngiomas is very high with treatment. If the tumor can be removed or treated with high doses of radiation, the chance of a permanent cure is about 80%-90%.
All A to Z dictionary entries are regularly reviewed by KidsHealth medical experts.
