Craniopharyngioma
A craniopharyngioma is a noncancerous (benign) tumor that develops at the base of the brain near the pituitary gland.
Endocrine glands release hormones (chemical messengers) into the bloodstream to be transported to various organs and tissues throughout the body. For instance, the pancreas secretes insulin, which allows the body to regulate levels of sugar in the blood. The thyroid gets instructions from the pituitary to secrete hormones which determine the rate of metabolism in the body (the more hormone in the bloodstream, the faster the chemical activity; the less hormone, the slower the activity).
Causes
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.
Symptoms
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.
Exams and Tests
Treatment
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.
Outlook (Prognosis)
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.
Possible Complications
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 to Contact a Medical Professional
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
References
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.
Version Info
Last reviewed on: 4/29/2022
Reviewed 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.
