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Childhood Brain and Spinal Cord Tumors Treatment Overview (PDQ)


General Information About Childhood Brain and Spinal Cord Tumors
Staging Childhood Brain and Spinal Cord Tumors
Treatment Option Overview
Treatment of Newly Diagnosed and Recurrent Childhood Brain Tumors
Treatment of Newly Diagnosed and Recurrent Childhood Spinal Cord Tumors
To Learn More About Childhood Brain and Spinal Cord Tumors
About This PDQ Summary

General Information About Childhood Brain and Spinal Cord Tumors

A childhood brain or spinal cord tumor is a disease in which abnormal cells form in the tissues of the brain or spinal cord.

There are many types of childhood brain and spinal cord tumors. The tumors are formed by the abnormal growth of cells and may begin in different areas of the brain or spinal cord.

The tumors may be benign (not cancer) or malignant (cancer). Benign brain tumors grow and press on nearby areas of the brain. They rarely spread into other tissues. Malignant brain tumors are likely to grow quickly and spread into other brain tissue. When a tumor grows into or presses on an area of the brain, it may stop that part of the brain from working the way it should. Both benign and malignant brain tumors can cause signs or symptoms and need treatment.

Together, the brain and spinal cord make up the central nervous system (CNS).

The brain controls many important body functions.

The brain has three major parts:

Anatomy of the brain; the right panel shows the supratentorial area (the upper part of the brain) and the posterior fossa/infratentorial area (the lower back part of the brain). The supratentorial area contains the cerebrum, lateral ventricle, third ventricle, choroid plexus, hypothalamus, pineal gland, pituitary gland, and optic nerve. The posterior fossa/infratentorial area contains the cerebellum, tectum, fourth ventricle, and   brain stem (pons and medulla). The tentorium and spinal cord are also shown. The left panel shows the cerebrum, ventricles (fluid-filled spaces), meninges, skull, cerebellum, brain stem (pons and medulla) and spinal cord.Anatomy of the brain. The supratentorial area (the upper part of the brain) contains the cerebrum, lateral ventricle and third ventricle (with cerebrospinal fluid shown in blue), choroid plexus, hypothalamus, pineal gland, pituitary gland, and optic nerve. The posterior fossa/infratentorial area (the lower back part of the brain) contains the cerebellum, tectum, fourth ventricle, and brain stem (pons and medulla). The tentorium separates the supratentorium from the infratentorium (right panel). The skull and meninges protect the brain and spinal cord (left panel).

The spinal cord connects the brain with nerves in most parts of the body.

The spinal cord is a column of nerve tissue that runs from the brain stem down the center of the back. It is covered by three thin layers of tissue called membranes. These membranes are surrounded by the vertebrae (back bones). Spinal cord nerves carry messages between the brain and the rest of the body, such as a message from the brain to cause muscles to move or a message from the skin to the brain to feel touch.

Brain and spinal cord tumors are a common type of childhood cancer.

Although cancer is rare in children, brain and spinal cord tumors are the third most common type of childhood cancer, after leukemia and lymphoma. Brain tumors can occur in both children and adults. Treatment for children is usually different than treatment for adults. (See the PDQ summary on Adult Central Nervous System Tumors Treatment for more information about the treatment of adults.)

This summary describes the treatment of primary brain and spinal cord tumors (tumors that begin in the brain and spinal cord). Treatment of metastatic brain and spinal cord tumors is not covered in this summary. Metastatic tumors are formed by cancer cells that begin in other parts of the body and spread to the brain or spinal cord.

The cause of most childhood brain and spinal cord tumors is unknown.

The signs and symptoms of childhood brain and spinal cord tumors are not the same in every child.

Signs and symptoms depend on the following:

Signs and symptoms may be caused by childhood brain and spinal cord tumors or by other conditions, including cancer that has spread to the brain. Check with your child's doctor if your child has any of the following:

In addition to these signs and symptoms of brain and spinal cord tumors, some children are unable to reach certain growth and development milestones such as sitting up, walking, and talking in sentences.

Tests that examine the brain and spinal cord are used to detect (find) childhood brain and spinal cord tumors.

The following tests and procedures may be used:

Most childhood brain tumors are diagnosed and removed in surgery.

If doctors think there might be a brain tumor, a biopsy may be done to remove a sample of tissue. For tumors in the brain, the biopsy is done by removing part of the skull and using a needle to remove a sample of tissue. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are found, the doctor may remove as much tumor as safely possible during the same surgery. The pathologist checks the cancer cells to find out the type and grade of brain tumor. The grade of the tumor is based on how abnormal the cancer cells look under a microscope and how quickly the tumor is likely to grow and spread.

Drawing of a craniotomy showing a section of the scalp that has been pulled back to remove a piece of the skull; the dura covering the brain has been opened to expose the brain. The layer of muscle under the scalp is also shown.Craniotomy: An opening is made in the skull and a piece of the skull is removed to show part of the brain.

The following test may be done on the sample of tissue that is removed:

Some childhood brain and spinal cord tumors are diagnosed by imaging tests.

Sometimes a biopsy or surgery cannot be done safely because of where the tumor formed in the brain or spinal cord. These tumors are diagnosed based on the results of imaging tests and other procedures.

Certain factors affect prognosis (chance of recovery).

The prognosis (chance of recovery) depends on the following:

Staging Childhood Brain and Spinal Cord Tumors

In childhood brain and spinal cord tumors, treatment options are based on several factors.

Staging is the process used to find how much cancer there is and if cancer has spread within the brain, spinal cord, or to other parts of the body. It is important to know the stage in order to plan cancer treatment.

In childhood brain and spinal cord tumors, there is no standard staging system. Instead, the plan for cancer treatment depends on several factors:

The information from tests and procedures done to detect (find) childhood brain and spinal cord tumors is used to determine the tumor risk group.

After the tumor is removed in surgery, some of the tests used to detect childhood brain and spinal cord tumors are repeated to help determine the tumor risk group (see the General Information section). This is to find out how much tumor remains after surgery.

Other tests and procedures may be done to find out if cancer has spread:

Childhood brain and spinal cord tumors may recur (come back) after treatment.

A recurrent childhood brain or spinal cord tumor is one that has recurred (come back) after it has been treated. Childhood brain and spinal cord tumors may come back in the same place or in another part of the brain. Sometimes they come back in another part of the body. The tumor may come back many years after first being treated. Diagnostic and staging tests and procedures, including biopsy, may be done to make sure that the tumor has recurred.

Treatment Option Overview

There are different types of treatment for children with brain and spinal cord tumors.

Different types of treatment are available for children with brain and spinal cord tumors. 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 cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.

Because cancer in children is rare, taking part in a clinical trial should be considered. Clinical trials are taking place in many parts of the country. Some clinical trials are open only to patients who have not started treatment.

Children with brain or spinal cord tumors should have their treatment planned by a team of health care providers who are experts in treating childhood brain and spinal cord tumors.

Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. 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. These may include the following specialists:

Childhood brain and spinal cord tumors may cause signs or symptoms that begin before the cancer is diagnosed and continue for months or years.

Childhood brain and spinal cord tumors may cause signs or symptoms that continue for months or years. Signs or symptoms caused by the tumor may begin before diagnosis. Signs or symptoms caused by treatment may begin during or right after treatment.

Some cancer treatments cause side effects months or years after treatment has ended.

These are called late effects. Late effects of cancer treatment may include the following:

Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information).

Three types of standard treatment are used:

Surgery

Surgery may be used to diagnose and treat childhood brain and spinal cord tumors. See the General Information section of this summary.

Radiation therapy

Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer 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 cancer being treated. External radiation therapy is used to treat childhood brain and spinal cord tumors.

Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer 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 cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly in the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated.

Anticancer drugs given by mouth or vein to treat brain and spinal cord tumors cannot cross the blood-brain barrier and enter the fluid that surrounds the brain and spinal cord. Instead, an anticancer drug is injected into the fluid-filled space to kill cancer cells there. This is called intrathecal chemotherapy.

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.

High-dose chemotherapy with stem cell transplant

High-dose chemotherapy with stem cell transplant is a way of giving high doses of chemotherapy and replacing blood-forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body’s blood cells.

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 cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.

Many of today's standard treatments for cancer 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 cancer 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.

Patients can enter clinical trials before, during, or after starting their cancer treatment.

Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.

Clinical trials are taking place in many parts of the country. Information about clinical trials is available from the NCI website.

Follow-up tests may be needed.

Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer 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 or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.

Treatment of Newly Diagnosed and Recurrent Childhood Brain Tumors

The brain is made of different kinds of cells. Childhood brain tumors are grouped and treated based on the type of cell the cancer formed in and where the tumor began growing in the CNS. Some types of tumors are divided into subtypes based on how the tumor looks under a microscope and whether it has certain gene changes. See Table 1 for a list of tumor types and staging and treatment information for newly diagnosed and recurrent childhood brain tumors.

Table 1. Newly Diagnosed or Recurrent Tumor Type and Its Related PDQ Treatment Summary

Tumor Type Tumor Subtype Related PDQ Treatment Summary
Astrocytomas and Other Tumors of Glial Origin
Low-Grade AstrocytomasAngiocentric gliomaSee Childhood Astrocytomas Treatment for information on low-grade astrocytomas.
Choroid glioma of the third ventricle
Diffuse astrocytoma, IDH-mutant, IDH-wild type, or NOS
Pilocytic astrocytoma
Pleomorphic xanthoastrocytoma
Subependymal giant cell astrocytoma
High-Grade AstrocytomasAnaplastic astrocytoma, IDH-mutant or IDH-wild typeSee Childhood Astrocytomas Treatment for information on high-grade astrocytomas.
Anaplastic pleomorphic xanthoastrocytoma
Diffuse midline glioma, H3 K27M-mutant
Glioblastoma, IDH-mutant
Glioblastoma, IDH-wildtype
Other Astrocytomas or GliomasAstroblastomaSee Childhood Astrocytomas Treatment for information on other astrocytomas or gliomas.
Pilomyxoid astrocytoma
Brain Stem Glioma
Diffuse intrinsic pontine gliomas, H3 K27M-mutant See Childhood Brain Stem Glioma Treatment.
Focal or low-grade brain stem gliomas
Central Nervous System Embryonal Tumors
Medulloblastoma See Childhood Central Nervous System Embryonal Tumors Treatment for information on medulloblastoma and nonmedulloblastomas.
NonmedulloblastomasCentral nervous system ganglioneuroblastoma
Central nervous system neuroblastoma
Embryonal tumor with multilayered rosettes (ETMR), C19MC-altered or NOS
Medulloepithelioma
Central Nervous System Atypical Teratoid/Rhabdoid TumorSee Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor Treatment.
Tumors of the Pineal RegionPineoblastomaSee Childhood Central Nervous System Embryonal Tumors Treatment.
Central Nervous System Germ Cell Tumors
GerminomasSee Childhood Central Nervous System Germ Cell Tumors Treatment.
TeratomasImmature teratomas
Mature teratomas
Teratomas with malignant transformation
Non-Germinomatous Germ Cell TumorsChoriocarcinoma
Embryonal carcinoma
Mixed germ cell tumors
Yolk sac tumor
CraniopharyngiomaSee Childhood Craniopharyngioma Treatment.
Ependymoma
Subependymoma (WHO grade I)See Childhood Ependymoma Treatment.
Myxopapillary ependymoma (WHO grade I)
Ependymoma (WHO grade II)
RELA fusion-positive ependymoma (WHO grade II or grade III)
Anaplastic ependymoma (WHO grade III)
Tumors of the Choroid Plexus
NOS = not otherwise specified; WHO = World Health Organization.

Treatment of Newly Diagnosed and Recurrent Childhood Spinal Cord Tumors

Tumors of many different cell types may form in the spinal cord. Low-grade spinal cord tumors usually do not spread. High-grade spinal cord tumors may spread to other places in the spinal cord or to the brain. See the following PDQ summaries for more information on staging and treatment of newly diagnosed and recurrent childhood spinal cord tumors:

To Learn More About Childhood Brain and Spinal Cord Tumors

For more information about childhood brain and spinal cord tumors, see the following:

For more childhood cancer information and other general cancer resources, see the following:

About This PDQ Summary

About PDQ

Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish.

PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH.

Purpose of This Summary

This PDQ cancer information summary has current information about the treatment of childhood brain and spinal cord tumors. 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.

Reviewers and Updates

Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Date Last Modified") is the date of the most recent change.

The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.

Clinical Trial Information

A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.

Clinical trials are listed in PDQ and can be found online at NCI's website. Many cancer doctors who take part in clinical trials are also listed in PDQ. For more information, call the Cancer Information Service 1-800-4-CANCER (1-800-422-6237).

Permission to Use This Summary

PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”

The best way to cite this PDQ summary is:

PDQ Pediatric Treatment Editorial Board. PDQ Childhood Brain and Spinal Cord Tumors Treatment Overview. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/brain/patient/child-brain-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389351]

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Physicians version: CDR0000062680
Date last modified: 2017-08-18