Craniopharyngioma

The exact cause of the tumor is unknown.

This tumor most commonly affects children from 5 to 14 years of age. Adults 50 to 74 years can sometimes be affected. Boys and girls are equally likely to develop this tumor.

A craniopharyngioma causes symptoms by:

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

Increased pressure on the brain can cause:

• Headache
• Nausea
• Vomiting (especially in the morning)
• Change in appetite and weight
• Confusion and drowsiness

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.

Your health care provider will perform a physical exam. Tests will be done to check for a tumor. These may include:

• Blood tests to measure hormone levels
• A CT scanMRI scan of the brain
• An examination of the nervous system
• Testing your vision

The goal of the treatment is to relieve symptoms. This tumor is best treated at a center with experience in treating craniopharyngiomas.

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 a CT scan, a biopsy may not be needed if treatment with radiation alone is planned. In some people, a shunt may be placed to treat the hydrocephalus.

Stereotactic radiosurgery is performed at some medical centers.

Hormonal replacement, short-term or long-term, might be needed.

In general, the outlook is good. There is an 80% to 90% chance of a cure at 10 years 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. Follow-up includes yearly MRIs and hormonal testing.

The 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.

There may be long-term hormone, vision, and nervous system problems after craniopharyngioma is treated. The hormonal complications include diabetes insipidus, which causes excessive thirst and urination. This can be temporary or permanent.

When the tumor is not completely removed, it may return and cause additional symptoms.

Other complications include:

• Hypothalamic obesity and metabolic issues
• Cognitive and behavioral changes
• Seizures
• Vascular complications such as stroke

Contact your provider for the following symptoms:

• Headache, confusion, drowsiness, nausea, vomiting, or balance problems (signs of increased pressure on the brain)
• Failing to keep up in school
• Weight loss or gain
• Increased thirst and urination
• Poor growth in a child
• Vision changes

Adamantinomatous craniopharyngioma; Ordinary craniopharyngioma; Papillary craniopharyngioma; Rathke pouch tumor; Hypophyseal duct tumor; Adamantinoma

Kayadjanian N, Hsu EA. Treatment priorities in craniopharyngioma: Perspectives of survivors and caregivers. Biomedicines. 2026;14(3):664. PMID: 41898310 pubmed.ncbi.nlm.nih.gov/41898310/.

Pitteloud N, Papadakis GE, Jacobs AN. Physiology and disorders of puberty. In: Melmed S, Auchus RJ, Goldfine AB, Rosen CJ, Kopp PA, eds. Williams Textbook of Endocrinology. 15th ed. Philadelphia, PA: Elsevier; 2025:chap 23.

Suh JH, Chao ST, Murphy ES, Kshettry VR, Recinos PF. Pituitary tumors and craniopharyngiomas. In: Foote RL, Michalski JM, Viswanathan AN, Tepper JE, eds. Gunderson & Tepper's Clinical Radiation Oncology. 6th ed. Philadelphia, PA: Elsevier; 2026:chap 36.

Zaky W. Central nervous system tumors in childhood. In: Kliegman RM, St. Geme JW, Blum NJ, et al, eds. Nelson Textbook of Pediatrics. 22nd ed. Philadelphia, PA: Elsevier; 2025:chap 546.

Updated by: Luc Jasmin, MD, Ph.D., FRCS (C), FACS, Department of Neuroscience, Guam Regional Medical City, Guam; Department of Surgery, Johnson City Medical Center, TN; Department of Maxillofacial Surgery at UCSF, San Francisco, CA. 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.