Craniopharyngioma

Date Updated: 07/31/2021

Overview

Craniopharyngioma is a rare type of noncancerous (benign) brain tumor.

Craniopharyngioma begins near the brain's pituitary gland, which secretes hormones that control many body functions. As a craniopharyngioma slowly grows, it can affect the function of the pituitary gland and other nearby structures in the brain.

Craniopharyngioma can occur at any age, but it occurs most often in children and older adults. Symptoms include gradual changes in vision, fatigue, excessive urination and headaches. Children with craniopharyngioma may grow slowly and may be smaller than expected.

Diagnosis

Tests and procedures used to diagnose craniopharyngioma include:

Physical exam. Diagnosing a craniopharyngioma usually starts with a medical history review and a neurological exam by your doctor. During this procedure, your vision, hearing, balance, coordination, reflexes, and growth and development are tested.
Blood tests. Blood tests may reveal changes in hormone levels that indicate a tumor is affecting your pituitary gland.
Imaging tests. Tests to create images of your brain may include X-rays, magnetic resonance imaging (MRI) and computerized tomography (CT).

Treatment

Craniopharyngioma treatment options include:

Surgery. Surgery to remove all or most of the tumor is most often recommended for people with craniopharyngioma. What type of operation is performed depends on the location and size of your tumor.

Open craniopharyngioma surgery (craniotomy) involves opening the skull to gain access to the tumor. During minimally invasive craniopharyngioma surgery (transsphenoidal procedure), special surgical tools are inserted through your nose. The tools pass through a natural corridor to the tumor, without affecting the brain.

When possible, surgeons remove the entire tumor. But because there are often many delicate and important structures nearby, doctors sometimes don't remove the entire tumor in order to ensure a good quality of life after the operation. In those situations, other treatments may be used after surgery.
Radiation therapy. External beam radiation therapy may be used after surgery to treat craniopharyngioma. This treatment uses powerful energy beams, such as X-rays and protons, to kill tumor cells. During external beam radiation therapy, you lie on a table while a machine precisely aims the energy to the tumor cells.

Specialized external beam radiation technology, such as proton beam therapy and intensity-modulated radiation therapy (IMRT), allows doctors to carefully shape and aim the radiation beam so that it delivers treatment to the tumor cells and spares nearby healthy tissue.

A type of radiation therapy called stereotactic radiosurgery may be recommended in uncommon situations when the tumor doesn't touch the bundle of nerve fibers that transmits visual information from your eye to your brain (optic nerve). Technically a type of radiation and not an operation, stereotactic radiosurgery focuses multiple beams of radiation on precise points to kill the tumor cells.

Another type of radiation therapy called brachytherapy involves placement of radioactive material directly into the tumor where it can radiate the tumor from the inside.
Chemotherapy. Chemotherapy is a drug treatment that uses chemicals to kill tumor cells. Chemotherapy can be injected directly into the tumor so that the treatment reaches the target cells and isn't likely to damage nearby healthy tissue.
Treatment for papillary craniopharyngioma. An uncommon type of craniopharyngioma called papillary craniopharyngioma may respond to targeted therapy. Targeted therapy is a drug treatment that focuses on specific abnormalities within the tumor cells that allows them to survive.

Nearly all papillary craniopharyngioma cells contain a mutation in a gene called the BRAF gene. Targeted therapy aimed at this mutation may be a treatment option. Specialized laboratory testing can reveal whether your craniopharyngioma contains papillary cells and whether those cells have the BRAF gene mutation.
Clinical trials. Clinical trials are studies of new treatments or new ways of using existing treatments. A clinical trial gives you the chance to try the latest treatments, but the side effects may not be known. Ask your doctor whether you're eligible to participate in clinical trials.