Childhood craniopharyngioma is a rare tumor usually found near the pituitary gland (a pea-sized organ at the bottom of the brain that controls other glands) and the hypothalamus (a small cone-shaped organ connected to the pituitary gland by nerves). Craniopharyngiomas can occur at any age but are most often diagnosed in children aged 5 to 14 years and older adults. They are rare in children younger than 2.
EnlargeCraniopharyngiomas are rare brain tumors that usually form near the pituitary gland and the hypothalamus. They are benign (not cancer) and do not spread to other parts of the brain or to other parts of the body. However, they may grow and press on nearby parts of the brain, including the pituitary gland, optic chiasm, and optic nerve. Craniopharyngiomas usually occur in children and young adults.
Craniopharyngiomas are usually part solid mass and part fluid-filled cyst. They are not cancer and do not spread to other parts of the brain or other parts of the body. However, they can grow and press on nearby parts of the brain, such as the pituitary gland. Or they may press on other areas, such as:
Craniopharyngiomas may affect many brain functions, including hormone production, growth, and vision. Treatments help stop the tumor from pushing on other areas of the brain.
Causes and risk factors for childhood craniopharyngioma
Craniopharyngioma is caused by certain changes to the way the brain cells function, especially how they grow and divide into new cells. Often, the exact cause of these changes is unknown.
There are no known risk factors for childhood craniopharyngioma.
Symptoms of childhood craniopharyngioma
The symptoms of childhood craniopharyngioma depend on where the tumor grows in the brain. It's important to check with your child's doctor if your child has:
headaches, including morning headache or headache that goes away after vomiting
These symptoms may be caused by problems other than craniopharyngioma. The only way to know is for your child to see a doctor.
Some symptoms caused by the tumor may continue for months or years after treatment. It is important to talk with your child's doctors about problems that may continue after treatment.
Tests to diagnose childhood craniopharyngioma
If your child has symptoms that suggest a craniopharyngioma, the doctor will need to find out if they are due to a tumor or another problem. The doctor will ask when the symptoms started and how often your child has been having them. They will also ask about your child's personal and family medical history and do a physical exam, including a neurologic exam. Depending on these results, they may recommend other tests. If your child is diagnosed with craniopharyngioma, the results of these tests will help you and your child's doctor plan treatment.
The tests to diagnose craniopharyngioma may include:
Visual field exam
A visual field exam checks a person’s field of vision (the total area in which objects can be seen). This test measures both central vision (how much a person can see when looking straight ahead) and peripheral vision (how much a person can see in all other directions while staring straight ahead). Any loss of vision may be a sign of a tumor that has damaged or pressed on the parts of the brain that affect eyesight.
CT scan (CAT scan)
CT scan uses a computer linked to an x-ray machine to make a series of detailed pictures of areas inside the body. The pictures are taken from different angles and are used to create 3-D views of tissues and organs. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. Learn more about Computed Tomography (CT) Scans and Cancer.
Magnetic resonance imaging (MRI) with gadolinium
MRI uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body, such as the brain and the spine. A substance called gadolinium is injected into a vein. The gadolinium collects around the tumor cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI).
Blood hormone studies
Blood hormone studies use a blood sample to measure the amounts of certain hormones released into the blood by organs and tissues in the body. If the amount of a hormone is higher or lower than normal, it can be a sign of disease in the organ or tissue that makes it. For craniopharyngioma, the blood may be checked for unusual levels of thyroid-stimulating hormone (TSH) or adrenocorticotropic hormone (ACTH). These hormones are made by the pituitary gland.
Biopsy
If the CT scan or MRI show there may be a brain tumor, your child will have a biopsy to remove a sample of the tumor.
Types of biopsy that may be used to take the sample of tissue include:
Open biopsy: A surgeon inserts a hollow needle through a hole in the skull into the brain.
Computer-guided needle biopsy: A surgeon inserts a hollow needle guided by a computer through a small hole in the skull into the brain.
Transsphenoidal biopsy: The surgeon inserts instruments through the nose and sphenoid bone (a butterfly-shaped bone at the base of the skull) and into the brain.
A pathologist views the tissue under a microscope to look for tumor cells. If they find tumor cells, the surgeon will remove as much tumor as safely possible during the same surgery.
Immunohistochemistry
Immunohistochemistry uses antibodies to check for certain antigens (markers) in a sample of a patient’s cells or tissue. The antibodies are usually linked to an enzyme or a fluorescent dye. After the antibodies bind to a specific antigen in the tissue sample, the enzyme or dye is activated, and the antigen can then be seen under a microscope. This type of test is used to help diagnose cancer and to help tell one type of cancer from another type of cancer.
Getting a second opinion
You may want to get a second opinion to confirm your child's diagnosis and treatment plan. If you seek a second opinion, you will need to get medical test results and reports from the first doctor to share with the second doctor. The second doctor will review the pathology report, slides, and scans. This doctor may agree with the first doctor, suggest changes to the treatment plan, or provide more information about your child's tumor.
To learn more about choosing a doctor and getting a second opinion, visit Finding Cancer Care. You can contact NCI's Cancer Information Service via chat, email, or phone (both in English and Spanish) for help finding a doctor or hospital that can provide a second opinion. For questions you might want to ask at your child's appointments, visit Questions to Ask Your Doctor about Cancer.
Types of treatment for childhood craniopharyngioma
Who treats children with craniopharyngioma?
A pediatric oncologist, a doctor who specializes in treating children with cancer, oversees treatment for childhood craniopharyngioma. The pediatric oncologist works with other health care providers who are experts in treating children with brain tumors and who specialize in certain areas of medicine. Other specialists may include:
There are different types of treatment for children and adolescents with craniopharyngioma. Although craniopharyngioma is not cancer, treatment is often similar to cancer treatment and may include surgery, radiation therapy, and other approaches. You and your child's care team will work together to decide treatment. Many factors will be considered, such as your child's age and overall health, where the tumor is located and whether it has spread into nearby tissue, and the possible side effects and late effects of treatment.
Your child's treatment plan will include information about the tumor, the goals of treatment, treatment options, and the possible side effects. It will be helpful to talk with your child's care team before treatment begins about what to expect. For help every step of the way, visit our booklet, Children with Cancer: A Guide for Parents.
Types of treatment your child might have include:
Surgery
The type of surgery your child will have depends on the size of the tumor, where it is in the brain, and whether it has grown into nearby tissue in a finger-like way. It also depends on expected late effects that may occur after surgery.
The types of surgery that may be used to remove the tumor that can be seen with the eye include:
Transsphenoidal surgery is a type of surgery in which a surgeon inserts instruments into the brain by going through a cut made under the upper lip or at the bottom of the nose between the nostrils. Then they go through the sphenoid bone (a butterfly-shaped bone at the base of the skull) to reach the tumor near the pituitary gland and hypothalamus.EnlargeTranssphenoidal surgery. An endoscope and a curette are inserted through the nose and sphenoid sinus to remove the tumor.
A craniotomy is surgery to remove the tumor through an opening made in the skull.EnlargeCraniotomy. An opening is made in the skull and a piece of the skull is removed to show part of the brain.
To help make a diagnosis, sometimes the surgeon will remove only part of the tumor. If a tumor is near the pituitary gland or hypothalamus, it will not be removed. Leaving the tumor helps reduce serious side effects from the surgery.
Sometimes, the surgeon will remove all of the tumor that they can see and no further treatment is needed. At other times, they may not be able to remove the tumor because it is growing into or pressing on nearby organs.
Surgery for cysts
If your child's tumor is mostly a fluid-filled cyst, they may have surgery to drain it. Draining it lowers the pressure in the brain and relieves symptoms.
A surgery called a partial resection can be used to remove fluid from cystic craniopharyngiomas. Or a thin tube called a catheter can be inserted into the cyst, and a small container placed under the skin. The fluid drains into the container and is later removed.
Sometimes, after the cyst is drained, a drug is put through the catheter into the cyst. This causes the inside wall of the cyst to scar and stops the cyst from making fluid. Or it can slow down how long it takes for the fluid to build up again. Surgery to remove the tumor or radiation therapy may be done after the cyst is drained.
Radiation therapy
Radiation therapy uses high-energy x-rays or other types of radiation to kill tumor cells or keep them from growing. It is often given after surgery to kill any tumor that is left in the brain.
Both external radiation therapy and internal radiation therapy (also called brachytherapy) are used to treat craniopharyngiomas.
External radiation therapy uses a machine outside the body to send radiation toward the area of the body with the tumor.
Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the tumor.
Radiation therapy to the brain can affect growth and development in young children, so ways of giving radiation therapy that have fewer side effects are often used. These include:
Stereotactic radiosurgery may be used for very small craniopharyngiomas at the base of the brain. For this treatment, a rigid head frame is attached to the skull to keep the head still during the treatment. Then, a machine aims a single large dose of radiation directly at the tumor. This procedure is a type of radiation therapy and does not involve surgery. It is also called stereotaxic radiosurgery, radiosurgery, and radiation surgery.
Intracavitary radiation therapy is a type of internal radiation therapy that may be used in tumors that are part solid mass and part fluid-filled cyst. For this treatment, radioactive material is placed inside the tumor. This type of radiation therapy causes less damage to the nearby hypothalamus and optic nerves.
Intensity-modulated photon therapy is a type of radiation therapy that uses x-rays or gamma rays that come from a special machine called a linear accelerator (linac) to kill tumor cells. A computer is used to target the exact shape and location of the tumor. Then thin beams of photons of different strengths are aimed at the tumor from many angles. This type of 3-dimensional radiation therapy may cause less damage to healthy tissue in the brain and other parts of the body.
Proton-beam radiation therapy is a type of radiation therapy that uses streams of protons (tiny particles with a positive charge) to kill tumor cells. This treatment can reduce the amount of radiation damage to healthy tissue near a tumor.
Chemotherapy (also called chemo) uses drugs to stop the growth of tumor cells. Chemotherapy either kills the tumor cells or stops them from dividing.
Chemotherapy can be placed directly into a cavity, such as a cyst. This way of giving chemotherapy is intracavitary chemotherapy. Bleomycin is a type of chemotherapy that can be placed directly into a cystic craniopharyngioma.
Observation means that your child's condition is closely watched without receiving treatment until symptoms appear or change.
Clinical trials
For some children, joining a clinical trial may be an option. There are different types of clinical trials for childhood cancer. For example, a treatment trial tests new treatments or new ways of using current treatments. Supportive care and palliative care trials look at ways to improve quality of life, especially for those who have side effects from cancer and its treatment.
You can use the clinical trial search to find NCI-supported cancer clinical trials accepting participants. The search allows you to filter trials based on the type of cancer, your child's age, and where the trials are being done. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.
Treatment of newly diagnosed childhood craniopharyngioma
Treatment of newly diagnosed childhood craniopharyngioma may include:
complete removal of the tumor with surgery with or without radiation therapy
partial removal of the tumor with surgery followed by radiation therapy
cyst drainage, followed by observation, radiation therapy, or surgery
brachytherapy or chemotherapy placed directly in the cyst or tumor
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
Treatment of progressive or recurrent childhood craniopharyngioma
Treatment options for progressive or recurrent childhood craniopharyngioma depend on the type of treatment that your child received when the tumor was first diagnosed and your child's needs.
Treatment may include:
surgery
external-beam radiation therapy
brachytherapy or intracavitary chemotherapy
observation
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
Prognostic factors for childhood craniopharyngioma
If your child has been diagnosed with craniopharyngioma, you likely have questions about how serious the tumor is and your child's chances of survival. The likely outcome or course of a disease is called prognosis.
The prognosis depends on:
the size of the tumor
where the tumor is in the brain
whether there are tumor cells left after surgery
your child's age
side effects that may occur months or years after treatment
whether the tumor has just been diagnosed or has recurred (come back)
No two people are alike, and responses to treatment can vary greatly. While the prognosis for childhood craniopharyngioma is generally good, the tumor often comes back after surgery. Your child's treatment team is in the best position to talk with you about your child's prognosis.
Side effects and late effects of treatment
Cancer treatments used for craniopharyngioma can cause side effects. Which side effects your child might have depends on the type of treatment they receive, the dose, and how their body reacts. Talk with your child's treatment team about which side effects to look for and ways to manage them.
Problems from treatment that begin 6 months or later after treatment and continue for months or years are called late effects. Late effects of treatment may include:
changes in mood, feelings, thinking, learning, or memory
second cancers (new types of cancer)
Serious physical problems may occur if the pituitary gland, hypothalamus, optic nerves, or carotid artery are affected during surgery or radiation therapy. These problems include:
Some late effects may be treated or controlled. Your child may need life-long hormone replacement therapy with several medicines. It is important to talk with your child's doctors about the effects treatment can have on your child. Learn more about Late Effects of Treatment for Childhood Cancer.
Follow-up care
Some of the tests that were done to diagnose the disease or decide how to treat it may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.
Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed. These tests are sometimes called follow-up tests or check-ups.
After treatment, follow-up testing with MRI will be done for several years to check if the tumor has come back.
Coping with your child's diagnosis
When your child has a tumor, every member of the family needs support. Taking care of yourself during this difficult time is important. Reach out to your child’s treatment team and to people in your family and community for support. To learn more, visit Support for Families When a Child Has Cancer and the booklet Children with Cancer: A Guide for Parents.
Related resources
For more childhood cancer information and other general cancer resources, visit:
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