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This information is produced and provided by the National Cancer Institute (NCI). The information in this topic may have changed since it was written. For the most current information, contact the National Cancer Institute via the Internet web site at http://cancer.gov or call 1-800-4-CANCER.
Childhood craniopharyngiomas are benign brain tumors found near the pituitary gland.
Childhood craniopharyngiomas are rare tumors 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).
Anatomy of the inside of the brain, showing the pineal and pituitary glands, optic nerve, ventricles (with cerebrospinal fluid shown in blue), and other parts of the brain.
Craniopharyngiomas are usually part solid mass and part fluid -filled cyst. 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 or other areas, including the pituitary gland, the optic chiasm, optic nerves, and fluid-filled spaces in the brain. Craniopharyngiomas may affect many functions of the brain. They may affect hormone making, growth, and vision. Benign brain tumors need treatment.
This summary is about the treatment of primary brain tumors (tumors that begin in the brain). Treatment for metastatic brain tumors, which are tumors formed by cancer cells that begin in other parts of the body and spread to the brain, is not covered in this summary. See the PDQ treatment summary on Childhood Brain and Spinal Cord Tumors Treatment Overview for information about the different types of childhood brain and spinal cord tumors.
Brain tumors can occur in both children and adults; however, treatment for children may be different than treatment for adults. (See the PDQ summary on Adult Central Nervous System Tumors Treatment for more information.)
There are no known risk factors for childhood craniopharyngioma.
Craniopharyngiomas are rare in children younger than 2 years of age and are most often diagnosed in children aged 5 to 14 years. It is not known what causes these tumors.
Signs of childhood craniopharyngioma include vision changes and slow growth.
These and other signs and symptoms may be caused by craniopharyngiomas or by other conditions. Check with your child's doctor if your child has any of the following:
Tests that examine the brain, vision, and hormone levels are used to detect (find) childhood craniopharyngiomas.
The following tests and procedures may be used:
Childhood craniopharyngiomas are diagnosed and may be removed in the same surgery.
Doctors may think a mass is a craniopharyngioma based on where it is in the brain and how it looks on a CT scan or MRI. In order to be sure, a sample of tissue is needed.
One of the following types of biopsy procedures may be used to take the sample of tissue:
A pathologist views the tissue under a microscope to look for tumor cells. If tumor cells are found, as much tumor as safely possible may be removed during the same surgery.
The following laboratory test may be done on the sample of tissue that is removed:
Certain factors affect prognosis (chance of recovery) and treatment options.
The prognosis (chance of recovery) and treatment options depend on the following:
The process used to find out if cancer has spread within the brain or to other parts of the body is called staging. There is no standard system for staging childhood craniopharyngioma. Craniopharyngioma is described as newly diagnosed disease or recurrent disease.
The results of the tests and procedures done to diagnose craniopharyngioma are used to help make decisions about treatment.
Recurrent craniopharyngioma is a tumor that has recurred (come back) after it has been treated. The tumor may come back in the same area of the brain where it was first found.
There are different types of treatment for children with craniopharyngioma.
Different types of treatments are available for children with craniopharyngioma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with tumors. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.
Because tumors in children are rare, taking part in a clinical trial should be considered. Clinical trials are taking place in many parts of the country. Information about ongoing clinical trials is available from the NCI website. Choosing the most appropriate treatment is a decision that ideally involves the patient, family, and health care team.
Children with craniopharyngioma should have their treatment planned by a team of health care providers who are experts in treating brain tumors in children.
Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with tumors. The pediatric oncologist works with other pediatric healthcare providers who are experts in treating children with brain tumors and who specialize in certain areas of medicine. These may include the following specialists:
Childhood brain tumors may cause signs or symptoms that begin before the cancer is diagnosed and continue for months or years.
Signs or symptoms caused by the tumor may begin before diagnosis and continue for months or years. It is important to talk with your child's doctors about signs or symptoms caused by the tumor that may continue after treatment.
Treatment for childhood craniopharyngioma may cause side effects.
For information about side effects that begin during treatment for cancer, see our Side Effects page.
Side effects from tumor treatment that begin after treatment and continue for months or years are called late effects. Late effects of tumor treatment may include the following:
The following serious physical problems may occur if the pituitary gland, hypothalamus, optic nerves, or carotid artery are affected during surgery or radiation therapy:
Some late effects may be treated or controlled. Life-long hormone replacement therapy with several medicines may be needed. It is important to talk with your child's doctors about the effects tumor treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information).
Five types of treatment are used:
The way the surgery is done depends on the size of the tumor and where it is in the brain. It also depends on whether the tumor has grown into nearby tissue in a finger-like way and expected late effects after surgery.
The types of surgery that may be used to remove all of the tumor that can be seen with the eye include the following:
Sometimes all of the tumor that can be seen is removed in surgery and no further treatment is needed. At other times, it is hard to remove the tumor because it is growing into or pressing on nearby organs. If there is tumor remaining after the surgery, radiation therapy is usually given to kill any tumor cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy.
Surgery and radiation therapy
Partial resection is used to treat some craniopharyngiomas. It is used to diagnose the tumor, remove fluid from a cyst, and relieve pressure on the optic nerves. If the tumor is near the pituitary gland or hypothalamus, it is not removed. This reduces the number of serious side effects after surgery. Partial resection is followed by radiation therapy.
Radiation therapy is a tumor treatment that uses high-energy x-rays or other types of radiation to kill tumor cells or keep them from growing. There are two types of radiation therapy:
The way the radiation therapy is given depends on the type of tumor, whether the tumor is newly diagnosed or has come back, and where the tumor formed in the brain. External and internal radiation therapy are used to treat childhood craniopharyngioma.
Because radiation therapy to the brain can affect growth and development in young children, ways of giving radiation therapy that have fewer side effects are being used. These include:
Surgery with cyst drainage
Surgery may be done to drain tumors that are mostly fluid-filled cysts. This lowers pressure in the brain and relieves symptoms. A catheter (thin tube) is inserted into the cyst and a small container is 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 increases the amount of the time it takes for the fluid to build up again. Surgery to remove the tumor may be done after the cyst is drained.
Chemotherapy is a treatment that uses anticancer drugs to stop the growth of tumor cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach tumor cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid or an organ, the drugs mainly affect tumor cells in those areas (regional chemotherapy).
Intracavitary chemotherapy is a type of regional chemotherapy that places drugs directly into a cavity, such as a cyst. It is used for craniopharyngioma that has come back after treatment.
Immunotherapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This type of cancer treatment is also called biotherapy or biologic therapy. For craniopharyngioma, the immunotherapy drug (interferon-alpha) is placed in a vein (intravenous) or inside the tumor using a catheter (intracavitary).
In newly diagnosed children, interferon-alpha may be placed directly into the cyst (intracystic) to delay the need for surgery or radiation therapy. In children whose tumor has recurred (come back), intracavitary interferon-alpha is used to treat the cyst part of the tumor.
New types of treatment are being tested in clinical trials.
This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website.
Targeted therapy is a type of treatment that uses drugs or other substances to attack cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do.
Targeted therapy is being studied for the treatment of childhood craniopharyngioma that has recurred.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the medical research process. Clinical trials are done to find out if new treatments are safe and effective or better than the standard treatment.
Many of today's standard treatments are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.
Patients who take part in clinical trials also help improve the way diseases will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
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.
Patients can enter clinical trials before, during, or after starting their treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients who have not improved. There are also clinical trials that test new ways to stop a disease from recurring (coming back) or reduce the side effects of treatment.
Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI's clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.
Follow-up tests may be needed.
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 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.
For information about the treatments listed below, see the Treatment Option Overview section.
Newly Diagnosed Childhood Craniopharyngioma
Treatment of newly diagnosed childhood craniopharyngioma may include the following:
Recurrent Childhood Craniopharyngioma
Craniopharyngioma may recur (come back) no matter how it was treated the first time. Treatment options for recurrent childhood craniopharyngioma depend on the type of treatment that was given when the tumor was first diagnosed and the needs of the child.
Treatment may include the following:
For more information about childhood craniopharyngioma and other childhood brain tumors, see the following:
For more childhood cancer information and other general cancer resources, see the following:
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Purpose of This Summary
This PDQ cancer information summary has current information about the treatment of childhood craniopharyngioma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.
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PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Craniopharyngioma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/brain/patient/child-cranio-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389237]
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Last Revised: 2019-08-20
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