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. Tumors may be benign (noncancerous) or malignant (cancerous).
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:
- The cerebrum is the largest part of the brain. It is at the top of the head. The cerebrum controls thinking, learning, problem solving, emotions, speech, reading, writing, and voluntary movement.
- The cerebellum, which is
in the lower back of the brain (near the middle of the back of the head), controls movement, balance, and
posture.
- The brain stem connects the brain to the spinal cord. It is
in the lowest part of the brain (just above the back of the neck). The
brain stem controls breathing, heart rate, and the nerves and muscles used in seeing, hearing, walking, talking, and eating.
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 signal from the brain to cause muscles to move or from the skin to the brain about the sense of 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 treatment summary on Adult Brain Tumors for more information.)
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, which are
tumors formed by cancer cells that
begin in other parts of the body and spread to the brain or spinal cord, is not covered in
this summary.
There are different types of childhood brain and spinal cord tumors.
Childhood brain and spinal cord tumors are named based on the type of cell they formed in and where the tumor first formed in the CNS.
Astrocytomas
Childhood astrocytomas are tumors that form in cells called astrocytes. They can be low-grade or high-grade tumors. The grade of the tumor describes how abnormal the cancer cells look under a microscope and how quickly the tumor is likely to grow and spread. High-grade astrocytomas are fast-growing, malignant tumors. Low-grade astrocytomas are slow-growing tumors that are less likely to be malignant.
Atypical Teratoid/Rhabdoid Tumor
Childhood atypical teratoid/rhabdoid tumors are fast-growing tumors that often form in the cerebellum. They may also form in other parts of the brain and in the spinal cord. (See the PDQ summary on Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor Treatment for more information.)
Brain Stem Glioma
Childhood brain stem gliomas form in the brain stem (the part of the brain connected to the spinal cord). (See the PDQ summary on Childhood Brain Stem Glioma Treatment for more information.)
Central Nervous System Embryonal Tumor
Childhood CNS embryonal tumors form in brain and spinal cord cells when the fetus is beginning to develop. They include the following types of tumors:
- CNS atypical teratoid/rhabdoid tumors (See the PDQ summary on Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor Treatment for more information.)
- Ependymoblastoma
- Medulloblastoma
- Medulloepithelioma
- Pinealparenchymal tumors
- Pineoblastoma
- Supratentorialprimitive neuroectodermal tumors (SPNET)
(See the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.)
Central Nervous System Germ Cell Tumor
Childhood CNS germ cell tumors form in germ cells, which are cells that develop into sperm or ova (eggs). There are different types of childhood germ cell tumors. These include germinomas, embryonal yolk sac carcinomas, choriocarcinomas, and teratomas. A mixed germ cell tumor has two types of germ cell tumors in it. Germ cell tumors can be either benign or malignant.
Germ cell brain tumors usually form in the center of the brain, near the pineal gland. The pineal gland is a tiny organ in the brain that makes melatonin, which is a substance that helps control the sleeping and waking cycle. Germ cell tumors can spread to other parts of the brain and spinal cord.
Craniopharyngioma
Childhood craniopharyngiomas are tumors that usually form just above the pituitary gland. The pituitary gland is found in the center of the brain behind the back of the nose. It is about the size of a pea and controls many important body functions including growth. Craniopharyngiomas rarely spread, but may affect important areas of the brain, such as the pituitary gland. (See the PDQ summary on Childhood Craniopharyngioma Treatment for more information.)
Ependymoma
Childhood ependymomas are slow-growing tumors formed in cells that line the fluid-filled spaces in the brain and spinal cord. (See the PDQ summary on Childhood Ependymoma Treatment for more information.)
Medulloblastoma
Childhood medulloblastomas form in the cerebellum. (See the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.)
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 brain.
Supratentorial Primitive Neuroectodermal Tumor
Childhood supratentorial primitive neuroectodermal tumors (SPNET) form in immature cells in the cerebrum. (See the PDQ treatment summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.)
The cause of most childhood brain and spinal cord tumors is unknown.
The symptoms of childhood brain and spinal cord tumors are not the same in every child.
Headaches and other symptoms may be caused by childhood brain and spinal cord tumors. Other conditions may cause the same symptoms. A doctor should be consulted if any of the following problems occur:
Brain Tumors
- Morning headache or headache that goes away after vomiting.
- Frequent nausea and vomiting.
- Vision, hearing, and speech problems.
- Loss of balance and trouble walking.
- Unusual sleepiness or change in activity level.
- Unusual changes in personality or behavior.
- Seizures.
- Increase in the head size (in infants).
Spinal Cord Tumors
- Back pain or pain that spreads from the back towards the arms or legs.
- A change in bowel habits or trouble urinating.
- Weakness in the legs.
- Trouble walking.
In addition to these 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:
- Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
- Neurological exam: A series of questions and tests to check the brain, spinal cord, and nerve function. The exam checks a person’s mental status, coordination, and ability to walk normally, and how well the muscles, senses, and reflexes work. This may also be called a neuro exam or a neurologic exam.
- Serum tumor marker test: A procedure in which a sample of blood is examined to measure the amounts of certain substances released into the blood by organs, tissues, or tumor cells in the body. Certain substances are linked to specific types of cancer when found in increased levels in the blood. These are called tumor markers.
- MRI (magnetic resonance imaging) with gadolinium: A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of the brain and spinal cord. A substance called gadolinium is injected into a vein. The gadolinium collects around the cancer cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI).
- CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. 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.
- Angiogram: A procedure to look at blood vessels and the flow of blood in the brain. A contrast dye is injected into the blood vessel. As the contrast dye moves through the blood vessel, x-rays are taken to see if there are any blockages.
- PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactiveglucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do.
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.
The following tests may be done on the sample of tissue that is removed:
- Immunohistochemistry study: A laboratory test in which a substance such as an antibody, dye, or radioisotope is added to a sample of cancer tissue to test for certain antigens. This type of study is used to tell the difference between different types of cancer.
- Light and electron microscopy: A laboratory test in which cells in a sample of tissue are viewed under regular and high-powered microscopes to look for certain changes in the cells.
- Cytogenetic analysis: A laboratory test in which cells in a sample of tissue are viewed under a microscope to look for certain changes in the chromosomes.
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:
- Whether there are any cancer cells left after surgery.
- The type of tumor.
- The location of the tumor.
- The child's age.
- Whether the tumor has just been diagnosed or has recurred (come back).
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Stages of 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 type of tumor and where the tumor formed in the brain.
- Whether the tumor is newly diagnosed or recurrent. A newly diagnosed brain or spinal cord tumor is one that has never been treated. 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, or spinal cord. Sometimes they come back in another part of the body. The tumor may come back many years after first being treated. Tests and procedures, including biopsy, that were done to diagnose and stage the tumor may be done to find out if the tumor has recurred.
- The grade of the 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. It is important to know the grade of the tumor and if there were any cancer cells remaining after surgery in order to plan treatment. The grade of the tumor is not used to plan treatment for all types of brain and spinal cord tumors.
- The tumor risk group. Risk groups are either average risk and poor risk or low, intermediate, and high risk. The risk groups are based on the amount of tumor remaining after surgery, the spread of cancer cells within the brain and spinal cord or to other parts of the body, where the tumor has formed, and the age of the child. The risk group is not used to plan treatment for all types of brain and spinal cord tumors.
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:
- Lumbar puncture: A procedure used to collect cerebrospinal fluid from the spinal column. This is done by placing a needle into the spinal column. Lumbar puncture is usually not used to stage childhood spinal cord tumors. This procedure is also called an LP or spinal tap.
 |
| Lumbar puncture. A patient lies in a curled position on a table. After a small area on the lower back is numbed, a spinal needle (a long, thin needle) is inserted into the lower part of the spinal column to remove cerebrospinal fluid (CSF, shown in blue). The fluid may be sent to a laboratory for testing. |
- Bone scan: A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones and is detected by a scanner.
- Chest x-ray: An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
- Bone marrow aspiration and biopsy: The removal of bone marrow, blood, and a small piece of bone by inserting a hollow needle into the hipbone or breastbone. A pathologist views the bone marrow, blood, and bone under a microscope to look for signs of cancer.
 |
| Bone marrow aspiration and biopsy. After a small area of skin is numbed, a Jamshidi needle (a long, hollow needle) is inserted into the patient’s hip bone. Samples of blood, bone, and bone marrow are removed for examination under a microscope. |
There are three ways that cancer spreads in the body.
The three ways that cancer spreads in the body are:
- Through tissue. Cancer invades the surrounding normal tissue.
- Through the lymph system. Cancer invades the lymph system and travels through the lymph vessels to other places in the body.
- Through the blood. Cancer invades the veins and capillaries and travels through the blood to other places in the body.
When cancer cells break away from the primary (original) tumor and travel through the lymph or blood to other places in the body, another (secondary) tumor may form. This process is called metastasis. The secondary (metastatic) tumor is the same type of cancer as the primary tumor. For example, if breast cancer spreads to the bones, the cancer cells in the bones are actually breast cancer cells. The disease is metastatic breast cancer, not bone cancer.
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Recurrent Childhood Brain and Spinal Cord Tumors
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 may 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 confirm the tumor has recurred.
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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 pediatriconcologist, 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:
- Neurosurgeon.
- Neurologist.
- Neuro-oncologist.
- Neuropathologist.
- Neuroradiologist.
- Radiation
oncologist.
- Endocrinologist.
- Psychologist.
- Ophthalmologist.
- Rehabilitation specialist.
- Social worker.
- Nursespecialist.
Childhood brain and spinal cord tumors may cause symptoms that begin before diagnosis and continue for months or years.
Childhood brain and spinal cord tumors may cause symptoms that continue for months or years. Symptoms caused by the tumor may begin before diagnosis. 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:
- Physical problems.
- Changes in mood, feelings, thinking, learning, or memory.
- Second cancers (new types of cancer).
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 as discussed in 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. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer.
Radiation therapy to the brain can affect growth and development in young children. For this reason, clinical trials are studying ways of using chemotherapy to
delay, reduce, or end the need for radiation therapy. Also, ways of giving radiation therapy that lessen damage to healthy brain tissue are being used. Stereotactic radiosurgery is a type of radiation therapy that uses a rigid head frame attached to the skull to aim high-dose radiation beams directly at the tumors, which causes less damage to nearby healthy tissue. It is also called stereotaxic radiosurgery and radiation surgery. This procedure does not involve surgery.
The way the radiation therapy is given depends on the type and stage of the cancer being treated.
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 spinal column, 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 Web site.
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. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's clinical trials database.
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. This is sometimes called re-staging.
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 or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.
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Treatment Options by Type of Childhood Brain and Spinal Cord Tumor
A link to a list of current clinical trials is included for each treatment section. For some types or stages of cancer, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you.
Childhood Astrocytoma
Childhood astrocytomas include both low-grade and high-grade astrocytomas. See the PDQ summary on Childhood Astrocytomas Treatment for more information.
Childhood Atypical Teratoid/Rhabdoid Tumor
See the PDQ summary on Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor Treatment for information.
Childhood Brain Stem Glioma
See the PDQ summary on Childhood Brain Stem Glioma Treatment
for information.
Childhood Central Nervous System Embryonal Tumors
Childhood central nervous system (CNS) embryonal tumors include CNS atypical teratoid/rhabdoid tumors (see the PDQ summary on Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor Treatment for more information), ependymoblastoma, medulloblastoma, medulloepithelioma, pinealparenchymaltumors, pineoblastoma, and supratentorialprimitive neuroectodermal tumors.(See the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.)
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with childhood embryonal tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.
Childhood Central Nervous System Germ Cell Tumors
Treatment of childhood CNSgerm cell tumors may
include:
- Radiation therapy, usually to the whole brain and spine.
- Chemotherapy.
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with childhood central nervous system germ cell tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.
Childhood Craniopharyngioma
(See the PDQ summary on Childhood Craniopharyngioma Treatment for information.)
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with childhood craniopharyngioma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.
Childhood Ependymoma
See the PDQ summary on Childhood Ependymoma Treatment for information.
Childhood Medulloblastoma
See the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment
for information.
Childhood Spinal Cord Tumors
Treatment of childhood spinal cordtumors may include the
following:
- Surgery with or without radiation therapy.
- Chemotherapy. (In very young children, chemotherapy is used only for low-grade tumors.)
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with childhood spinal cord neoplasm. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.
Childhood Supratentorial Primitive Neuroectodermal Tumors and Pineoblastoma
See the PDQ treatment summary on Childhood Central Nervous System Embryonal Tumors Treatment for information.
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To Learn More About Childhood Brain and Spinal Cord Tumors
For more information from the National Cancer Institute about childhood brain and spinal cord tumors, see the following:
For more childhood cancer information and other general cancer resources from the National Cancer Institute, see the following:
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This information is provided by the National Cancer Institute.
This information was last updated on October 15, 2009.
Purpose of This PDQ Summary
This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of select childhood brain and spinal cord tumors. It also provides links to the other PDQ childhood brain and spinal cord tumor summaries. This summary is reviewed regularly and updated as necessary by the PDQ Pediatric Treatment Editorial Board.
Information about the following is included in this summary:
- Classification of childhood brain and spinal cord tumors.
- General approach to care for childhood brain tumor patients.
- Stage information about select childhood brain and spinal cord tumors, and links to the other PDQ childhood brain and spinal cord tumor summaries.
- Treatment options about select childhood brain and spinal cord tumors, and links to the other PDQ childhood brain and spinal cord tumor summaries.
This summary is intended as a resource to inform and assist clinicians and other health professionals who care for pediatric cancer patients. It does not provide formal guidelines or recommendations for making health care decisions.
In this summary, treatments are described as “standard” or “conventional” and “under clinical evaluation.” These designations should not be used as a basis for reimbursement determinations.
This summary is also available in a patient version, which is written in less technical language, and in Spanish.
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General Information
Note: Separate PDQ summaries on Childhood Astrocytomas Treatment, Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor Treatment, Childhood Brain Stem Glioma Treatment, Childhood Central Nervous System Embryonal Tumors Treatment, Childhood Ependymoma Treatment, and Childhood Craniopharyngioma Treatment are also available.
The National Cancer Institute (NCI) provides the PDQ pediatric cancer information treatment summaries as a public service to increase the availability of evidence-based cancer information to health professionals, patients, and the public.
Information about ongoing clinical trials is available from the NCI Web site.
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Classification of Brain Tumors
Primary brain tumors are a diverse group of diseases that together constitute
the most common solid tumor in childhood. Between 2,500 and 3,500 children are diagnosed in the United States each year. Immunohistochemical analysis,
cytogenetic and molecular genetic findings, and measures of mitotic activity
are increasingly used in tumor diagnosis and classification, and will likely alter classification and nomenclature in the future.
The classification of childhood brain tumors is based on histology and location.[1] Tumors are classically categorized as infratentorial, supratentorial, sellar, or suprasellar.
Common
infratentorial (posterior fossa) tumors include:
- Cerebellar astrocytomas (usually pilocytic but also fibrillary and, less frequently,
high-grade).
- Medulloblastomas (primitive neuroectodermal tumors [PNETs]).
- Ependymomas (cellular, papillary, clear cell, tanycytic, or anaplastic).
- Brain stem gliomas are typically diffuse intrinsic pontine gliomas or diffuse intrinsic high-grade tumors that are diagnosed neuroradiographically without biopsy. Focal, tectal, and exophytic cervicomedullary tumors are generally low-grade tumors.
- Atypical teratoid/rhabdoid tumors.
Supratentorial tumors include:
- Low-grade cerebral hemispheric astrocytomas (grade 1 [pilocytic] or grade 2).
- High-grade or malignant astrocytomas (anaplastic astrocytomas, glioblastomas
multiforme [grade 3 or grade 4]).
- Mixed gliomas (low-grade or high-grade).
- Oligodendrogliomas (low-grade or high-grade).
- PNETs (including cerebral neuroblastomas, pineoblastomas, and ependymoblastomas).
- Atypical teratoid/rhabdoid tumors.
- Ependymomas (cellular or anaplastic).
- Meningiomas.
- Choroid plexus tumors (papillomas and carcinomas).
- Pineal parenchymal tumors (pineocytomas, mixed pineal
parenchymal tumors, and pineal parenchymal tumors of intermediate differentiation).
- Neuronal and mixed neuronal glial tumors (gangliogliomas, desmoplastic
infantile gangliogliomas, and dysembryoplastic neuroepithelial tumors).
- Metastasis (rare) from extraneural malignancies.
Sellar or suprasellar tumors include:
- Craniopharyngiomas.
- Diencephalic astrocytomas (central tumors involving the chiasm, hypothalamus, and/or thalamus) that are generally low-grade
(including astrocytomas, grade 1 [pilocytic] or grade 2).
- Germ cell tumors (germinomas or nongerminomatous).
References:
Kleihues P, Cavenee WK, eds.: Pathology and Genetics of Tumours of the Nervous System. Lyon, France: International Agency for Research on Cancer, 2000.
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Classification of Spinal Cord Tumors
Primary central nervous system spinal cord tumors comprise approximately
1% to 2% of all childhood nervous system tumors.[1][2][3] As is the case for
primary brain tumors, such lesions are histologically heterogeneous.
Approximately 70% of all intramedullary spinal cord tumors will be low-grade
astrocytomas and/or gangliogliomas. Other tumor types that occur include
ependymomas (refer to the PDQ summary on Childhood Ependymoma Treatment for more information), higher-grade glial tumors, and (rarely) primitive neuroectodermal
tumors (refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information). Myxopapillary ependymomas have a tendency to develop in the conus
and cauda equina regions. Symptoms and signs of spinal cord tumors are highly
dependent on the location of the tumor and its extent; some low-grade spinal
cord tumors are associated with large cysts that extend rostrally and caudally. At times it is impossible to
distinguish a tumor that arises in the medulla from a tumor that arises in
the upper cervical cord.
The classification of spinal cord tumors is based on histopathologic
characteristics of the tumor and does not differ from that of primary brain
tumors.[1][2][3]
References:
Constantini S, Miller DC, Allen JC, et al.: Radical excision of intramedullary spinal cord tumors: surgical morbidity and long-term follow-up evaluation in 164 children and young adults. J Neurosurg 93 (2 Suppl): 183-93, 2000.
Bouffet E, Pierre-Kahn A, Marchal JC, et al.: Prognostic factors in pediatric spinal cord astrocytoma. Cancer 83 (11): 2391-9, 1998.
Hardison HH, Packer RJ, Rorke LB, et al.: Outcome of children with primary intramedullary spinal cord tumors. Childs Nerv Syst 3 (2): 89-92, 1987.
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General Approach to Care for Children with Brain and Spinal Cord Tumors
Important concepts that should be understood by those treating and caring for a
child who has a brain or spinal cord tumor include the following:
- The cause of most childhood brain tumors remains unknown.[1][2][3]
- Selection of an appropriate therapy can only occur if the correct
diagnosis is made and the stage of the disease is accurately determined.
- Children with primary brain or spinal cord tumors represent a major therapy challenge that,
for optimal results, requires the coordinated efforts of pediatric specialists
in fields such as neurosurgery, neuropathology,
radiation oncology, pediatric oncology, neuro-oncology, neurology, rehabilitation, neuroradiology, endocrinology, and
psychology, who have special expertise in the care of patients with these
diseases.[4][5][6] For example, radiation therapy of pediatric brain tumors is very technically demanding and should be carried out in centers that have experience in this area.
- For most childhood brain and spinal cord tumors, the optimal treatment regimen
has not been determined. Children who have brain and spinal cord tumors should be
considered for enrollment in a clinical trial when an appropriate study
is available. Such clinical trials are being carried out by institutions
and cooperative groups.
Many of the improvements in survival in childhood cancer have been made as a result of clinical trials that have attempted to improve on the best accepted therapy available. Information about ongoing clinical trials is available from the NCI
Web site.
- While more than 50% of children diagnosed with brain tumors will survive more than 5
years from diagnosis, survival rates are wide-ranging depending on tumor type and stage. Long-term sequelae related to the initial presence of the tumor and subsequent treatment are common.[7][8][9] For more information about possible long-term or late effects, refer to the PDQ summary on Late Effects of Treatment for Childhood Cancer.
- Guidelines for pediatric cancer centers and their role in the treatment
of pediatric patients with cancer have been outlined by the American
Academy of Pediatrics.[10]
References:
Kuijten RR, Bunin GR: Risk factors for childhood brain tumors. Cancer Epidemiol Biomarkers Prev 2 (3): 277-88, 1993 May-Jun.
Kuijten RR, Strom SS, Rorke LB, et al.: Family history of cancer and seizures in young children with brain tumors: a report from the Childrens Cancer Group (United States and Canada). Cancer Causes Control 4 (5): 455-64, 1993.
Fisher JL, Schwartzbaum JA, Wrensch M, et al.: Epidemiology of brain tumors. Neurol Clin 25 (4): 867-90, vii, 2007.
Strother DR, Poplack IF, Fisher PG, et al.: Tumors of the central nervous system. In: Pizzo PA, Poplack DG, eds.: Principles and Practice of Pediatric Oncology. 4th ed. Philadelphia, Pa: Lippincott, Williams and Wilkins, 2002, pp 751-824.
Pollack IF: Brain tumors in children. N Engl J Med 331 (22): 1500-7, 1994.
Cohen ME, Duffner PK, eds.: Brain Tumors in Children: Principles of Diagnosis and Treatment. 2nd ed. New York: Raven Press, 1994.
Ris MD, Packer R, Goldwein J, et al.: Intellectual outcome after reduced-dose radiation therapy plus adjuvant chemotherapy for medulloblastoma: a Children's Cancer Group study. J Clin Oncol 19 (15): 3470-6, 2001.
Johnson DL, McCabe MA, Nicholson HS, et al.: Quality of long-term survival in young children with medulloblastoma. J Neurosurg 80 (6): 1004-10, 1994.
Packer RJ, Sutton LN, Goldwein JW, et al.: Improved survival with the use of adjuvant chemotherapy in the treatment of medulloblastoma. J Neurosurg 74 (3): 433-40, 1991.
Guidelines for the pediatric cancer center and role of such centers in diagnosis and treatment. American Academy of Pediatrics Section Statement Section on Hematology/Oncology. Pediatrics 99 (1): 139-41, 1997.
Top
Stage Information
Childhood Astrocytoma
Childhood astrocytomas are classified as low-grade or high-grade.
Childhood Low-Grade Astrocytomas:
- Pilocytic astrocytoma.
- Diffuse fibrillary astrocytoma.
Childhood High-Grade Astrocytomas:
- Anaplastic astrocytoma.
- Glioblastoma multiforme.
Refer to the PDQ summary on Childhood Astrocytomas Treatment for more information.
Childhood Central Nervous System (CNS) Atypical Teratoid/Rhabdoid Tumor
Refer to the PDQ summary on Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor Treatment for more information.
Childhood Brain Stem Glioma
Childhood brain stem gliomas include:
- Diffuse intrinsic pontine gliomas.
- Focal or low-grade brain stem gliomas.
Refer to the PDQ summary on Childhood Brain Stem Glioma Treatment for more information.
Childhood CNS Embryonal Tumors
Childhood CNS embryonal tumors include:
- CNS atypical teratoid/rhabdoid tumors. (Refer to the PDQ summary on Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor Treatment for more information.)
- Ependymoblastomas.
- Medulloblastomas.
- Medulloepitheliomas.
- Pineal parenchymal tumors of intermediate differentiation.
- Pineoblastomas.
- Supratentorial primitive neuroectodermal tumors.
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Childhood CNS Germ Cell Tumors
Childhood CNS germ cell tumors include:
- Germinomas.
- Embryonal yolk sac tumors.
- Choriocarcinomas.
- Immature teratomas.
- Mature teratomas.
- Teratomas with malignant transformation.
- Mixed germ cell tumors.
- Nongerminomatous germ cell tumors.
Germ cell brain tumors usually arise in the pineal or suprasellar regions.
Histologic subtypes include teratomas (both mature and immature), germinomas,
choriocarcinomas, and nongerminomatous germ cell tumors (i.e., embryonal cell
carcinoma, yolk cell or endodermal sinus tumors, and mixed germ cell tumors). These tumors have a
propensity for subarachnoid spread. Every patient with a germinoma or
malignant germ cell tumor should be evaluated with diagnostic imaging of the
spinal cord and whole brain. The best method for evaluating spinal cord
subarachnoid metastasis is magnetic resonance imaging with gadolinium enhancement. Cerebrospinal
fluid CSF) should be examined cytologically and levels of alpha-fetoprotein (AFP)
and human chorionic gonadotropin (HCG) determined. AFP and/or HCG may be
elevated in the serum of such patients. Prognosis is related to histology;
patients with pure germinoma have a more favorable outcome than those with
nongerminomatous germ cell tumors (nongerminomas).[1][2]
Childhood Craniopharyngioma
Refer to the PDQ summary on Childhood Craniopharyngioma Treatment for more information.
Childhood Ependymoma
Refer to the PDQ summary on Childhood Ependymoma Treatment for more information.
Childhood Ependymoblastoma
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Childhood Malignant Glioma
Refer to the PDQ summary on Childhood Astrocytomas Treatment for more information.
Childhood Medulloblastoma
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more
information.
Childhood Medulloepithelioma
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Childhood Pineal Parenchymal Tumors
Childhood pineal parenchymal tumors include:
- Pineoblastomas.
- Pineocytomas.
- Pineal parenchymal tumors of intermediate differentiation.
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Childhood Spinal Cord Tumors
There is no uniformly accepted staging system for childhood primary spinal cord
tumors. These tumors are classified based on their location within the spinal
cord and histology. Low-grade spinal cord tumors rarely disseminate elsewhere
in the nervous system; however, higher grade tumors may disseminate.[3][4]
Despite this, because of the location of the tumor and concerns over causing
further neurologic deterioration by CSF attainment, routine
lumbar spinal punctures are not indicated in the evaluation of a child with a
spinal cord tumor. For high-grade glial spinal cord tumors, and possibly lower
grade tumors and ependymomas, (refer to the PDQ summary on Childhood Ependymoma Treatment for more information) neuroimaging of the entire neuroaxis (brain and
entire spine) is indicated at the time of diagnosis for determination of extent
of disease.
Childhood Supratentorial Primitive Neuroectodermal Tumors
Childhood supratentorial primitive neuroectodermal tumors include:
- Primitive neuroectodermal tumors.
- Cerebral neuroblastomas.
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
References:
Matsutani M, Sano K, Takakura K, et al.: Primary intracranial germ cell tumors: a clinical analysis of 153 histologically verified cases. J Neurosurg 86 (3): 446-55, 1997.
Balmaceda C, Modak S, Finlay J: Central nervous system germ cell tumors. Semin Oncol 25 (2): 243-50, 1998.
Constantini S, Miller DC, Allen JC, et al.: Radical excision of intramedullary spinal cord tumors: surgical morbidity and long-term follow-up evaluation in 164 children and young adults. J Neurosurg 93 (2 Suppl): 183-93, 2000.
Bouffet E, Pierre-Kahn A, Marchal JC, et al.: Prognostic factors in pediatric spinal cord astrocytoma. Cancer 83 (11): 2391-9, 1998.
Top
Treatment Option Overview
Many of the improvements in survival in childhood cancer have been made as a
result of clinical trials that have attempted to improve on the best
accepted therapy available. Clinical trials in pediatrics are designed to compare new
therapy with therapy that is currently accepted as standard. This comparison
may be done in a randomized study of two treatment arms or by evaluating a
single new treatment and comparing the results with those previously obtained
with existing therapy.
Because of the relative rarity of cancer in children, all patients with brain
and spinal cord tumors should be considered for entry into a clinical trial. To determine and
implement optimum treatment, treatment planning by a multidisciplinary team of
cancer specialists who have experience treating childhood brain tumors is
required. Radiation therapy of pediatric brain tumors is very technically demanding and should be carried out in centers that have experience in this
area to ensure optimal results.
Debilitating effects on growth and neurologic development have frequently been
observed following radiation therapy, especially in younger children.[1][2][3]
Secondary tumors have increasingly been diagnosed in long-term survivors.[4]
For this reason, the role of chemotherapy in allowing a delay or reduction in the
administration of radiation therapy is under study, and preliminary results
suggest that chemotherapy can be used to delay, limit, and sometimes obviate, the need
for radiation therapy in children with benign and malignant lesions.[5][6][7]
Long-term management of these patients is complex and requires a
multidisciplinary approach.
References:
Ris MD, Packer R, Goldwein J, et al.: Intellectual outcome after reduced-dose radiation therapy plus adjuvant chemotherapy for medulloblastoma: a Children's Cancer Group study. J Clin Oncol 19 (15): 3470-6, 2001.
Johnson DL, McCabe MA, Nicholson HS, et al.: Quality of long-term survival in young children with medulloblastoma. J Neurosurg 80 (6): 1004-10, 1994.
Packer RJ, Sutton LN, Goldwein JW, et al.: Improved survival with the use of adjuvant chemotherapy in the treatment of medulloblastoma. J Neurosurg 74 (3): 433-40, 1991.
Jenkin D: Long-term survival of children with brain tumors. Oncology (Huntingt) 10 (5): 715-9; discussion 720, 722, 728, 1996.
Duffner PK, Horowitz ME, Krischer JP, et al.: Postoperative chemotherapy and delayed radiation in children less than three years of age with malignant brain tumors. N Engl J Med 328 (24): 1725-31, 1993.
Packer RJ, Lange B, Ater J, et al.: Carboplatin and vincristine for recurrent and newly diagnosed low-grade gliomas of childhood. J Clin Oncol 11 (5): 850-6, 1993.
Mason WP, Grovas A, Halpern S, et al.: Intensive chemotherapy and bone marrow rescue for young children with newly diagnosed malignant brain tumors. J Clin Oncol 16 (1): 210-21, 1998.
Top
Treatment of Newly Diagnosed Childhood Brain and Spinal Cord Tumors
Childhood Astrocytoma
Childhood astrocytomas are classified as low-grade or high-grade.
Childhood Low-Grade Astrocytomas:
- Pilocytic astrocytoma.
- Diffuse fibrillary astrocytoma.
Childhood High-Grade Astrocytomas:
- Anaplastic astrocytoma.
- Glioblastoma multiforme.
Refer to the PDQ summary on Childhood Astrocytomas Treatment for more information.
Childhood Central Nervous System (CNS) Atypical Teratoid/Rhabdoid Tumor
Refer to the PDQ summary on Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor Treatment for more information.
Childhood Brain Stem Glioma
Childhood brain stem gliomas include:
- Diffuse intrinsic pontine gliomas.
- Focal or low-grade brain stem gliomas.
Refer to the PDQ summary on Childhood Brain Stem Glioma Treatment for more
information
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with
childhood brain stem glioma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Childhood CNS Embryonal Tumors
Childhood CNS embryonal tumors include:
- CNS atypical teratoid/rhabdoid tumors. (Refer to the PDQ summary on Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor Treatment for more information.)
- Ependymoblastomas.
- Medulloblastomas.
- Medulloepitheliomas.
- Pineal parenchymal tumors of intermediate differentiation.
- Pineoblastomas.
- Supratentorial primitive neuroectodermal tumors.
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with
childhood embryonal tumor. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Childhood CNS Germ Cell Tumors
Childhood CNS germ cell tumors include:
- Germinomas.
- Embryonal yolk sac tumors.
- Choriocarcinomas.
- Immature teratomas.
- Mature teratomas.
- Teratomas with malignant transformation.
- Mixed germ cell tumors.
- Nongerminomatous germ cell tumors.
Surgery, other than biopsy to establish the diagnosis, rarely plays a role in the
treatment of CNS germinomas. The role of surgical
resection for nongerminomatous germ cell tumors and teratomas remains to be
defined.[1] For germinomas, irradiation with doses of 45 Gy to 54 Gy to the
tumor and 21 Gy to 36 Gy to the whole brain and spine is usually curative. In
selected cases, germinoma can be effectively treated with ventricular field radiation
therapy and at lower dose levels (30–36 Gy) following response to chemotherapy.[1] Although experience with
pre-irradiation chemotherapy has shown that most of these tumors
respond to cyclophosphamide and platinum-containing drugs, the definitive role
of chemotherapy has yet to be determined.[1] Disseminated germinomas are treated with craniospinal
irradiation,[2][3] alone or in combination with chemotherapy. The usual dose to the
tumor is 45 Gy to 54 Gy with 27 Gy to 36 Gy to the whole brain and spine. Although nongerminomatous germ cell tumors (e.g., embryonal carcinomas, yolk cell tumors, and mixed germ cell tumors) may respond
to chemotherapeutic agents (e.g., cisplatin or carboplatin, etoposide,
cyclophosphamide, and vinblastine) as do such histologies outside of the CNS,
optimal combination of agents, and the timing of chemotherapy in relation to radiation therapy
remains to be determined.[4][5][6]
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with
childhood central nervous system germ cell tumor. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Childhood Craniopharyngioma
Refer to the PDQ summary on Childhood Craniopharyngioma Treatment for more information.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with
childhood craniopharyngioma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Childhood Ependymoma
Refer to the PDQ summary on Childhood Ependymoma Treatment for more
information.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with
newly diagnosed childhood ependymoma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Childhood Ependymoblastoma
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with
childhood ependymoblastoma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Childhood Malignant Glioma
Refer to the PDQ summary on Childhood Astrocytomas Treatment for more information.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with
childhood cerebral astrocytoma/malignant glioma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Childhood Medulloblastoma
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more
information.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with
untreated childhood medulloblastoma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Childhood Medulloepithelioma
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with
childhood medulloepithelioma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Childhood Pineal Parenchymal Tumors
Childhood pineal parenchymal tumors include:
- Pineoblastomas.
- Pineocytomas.
- Pineal parenchymal tumors of intermediate differentiation.
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with
childhood pineal parenchymal tumor. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Childhood Spinal Cord Tumors
The optimal treatment for intrinsic/intramedullary glial spinal cord tumors has
not been determined by prospective randomized trials. Therapeutic options
include surgery alone, surgery plus local radiation therapy, and possibly
adjuvant chemotherapy in selected cases.[7][8] Extensive surgical resections
are technically possible for many patients with intramedullary spinal cord
tumors, but may result in worsening neurologic status in at least 10%
of cases.[7][8] Surgery is usually indicated at least to determine the type of
tumor present; for low-grade glial tumors this may be the only treatment
required.[7] In one recent series of 164 children and young adults with
intramedullary low-grade glial tumors or ganglioglial spinal cord tumors, 70%
were controlled for 5 years after extensive surgical resections.[7] Radiation therapy has been demonstrated to control disease in some patients with
low-grade glial tumors after subtotal resections.[8][9][10] The role of
chemotherapy for spinal cord tumors is poorly characterized, but some very
young children with low-grade glial tumors have been successfully treated with
a carboplatin and vincristine drug regimen.[11] Outcomes for patients with
high-grade glial tumors have been extremely poor; most
develop progressive disease within 3 years of treatment with surgery,
radiation, and/or chemotherapy.[7][9][10]
The optimal treatment for children with spinal ependymomas has not been well characterized (refer to the PDQ summary on Childhood Ependymoma Treatment for more information). As is the case for glial tumors, treatment options
predominantly consist of either surgery alone or surgery followed by local
radiation therapy.[7][8] Management of primitive neuroectodermal tumors of the
spinal cord is also not well delineated, and most patients are treated on
treatment protocols designed for children with high-risk medulloblastoma.
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with
childhood spinal cord neoplasm. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Childhood Supratentorial Primitive Neuroectodermal Tumors
Childhood supratentorial primitive neuroectodermal tumors include:
- Primitive neuroectodermal tumors.
- Cerebral Neuroblastomas.
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with
childhood supratentorial primitive neuroectodermal tumor. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
References:
Matsutani M, Sano K, Takakura K, et al.: Primary intracranial germ cell tumors: a clinical analysis of 153 histologically verified cases. J Neurosurg 86 (3): 446-55, 1997.
Dearnaley DP, A'Hern RP, Whittaker S, et al.: Pineal and CNS germ cell tumors: Royal Marsden Hospital experience 1962-1987. Int J Radiat Oncol Biol Phys 18 (4): 773-81, 1990.
Linstadt D, Wara WM, Edwards MS, et al.: Radiotherapy of primary intracranial germinomas: the case against routine craniospinal irradiation. Int J Radiat Oncol Biol Phys 15 (2): 291-7, 1988.
Balmaceda C, Heller G, Rosenblum M, et al.: Chemotherapy without irradiation--a novel approach for newly diagnosed CNS germ cell tumors: results of an international cooperative trial. The First International Central Nervous System Germ Cell Tumor Study. J Clin Oncol 14 (11): 2908-15, 1996.
Bouffet E, Baranzelli MC, Patte C, et al.: Combined treatment modality for intracranial germinomas: results of a multicentre SFOP experience. Société Française d'Oncologie Pédiatrique. Br J Cancer 79 (7-8): 1199-204, 1999.
Robertson PL, DaRosso RC, Allen JC: Improved prognosis of intracranial non-germinoma germ cell tumors with multimodality therapy. J Neurooncol 32 (1): 71-80, 1997.
Constantini S, Miller DC, Allen JC, et al.: Radical excision of intramedullary spinal cord tumors: surgical morbidity and long-term follow-up evaluation in 164 children and young adults. J Neurosurg 93 (2 Suppl): 183-93, 2000.
Bouffet E, Pierre-Kahn A, Marchal JC, et al.: Prognostic factors in pediatric spinal cord astrocytoma. Cancer 83 (11): 2391-9, 1998.
Hardison HH, Packer RJ, Rorke LB, et al.: Outcome of children with primary intramedullary spinal cord tumors. Childs Nerv Syst 3 (2): 89-92, 1987.
O'Sullivan C, Jenkin RD, Doherty MA, et al.: Spinal cord tumors in children: long-term results of combined surgical and radiation treatment. J Neurosurg 81 (4): 507-12, 1994.
Hassall TE, Mitchell AE, Ashley DM: Carboplatin chemotherapy for progressive intramedullary spinal cord low-grade gliomas in children: three case studies and a review of the literature. Neuro-oncol 3 (4): 251-7, 2001.
Top
Treatment of Recurrent Childhood Brain and Spinal Cord Tumors
Recurrence is not uncommon in both low-grade and malignant childhood brain tumors
and may occur many years after initial treatment.[1] Disease may occur at the
primary tumor site or, especially in malignant tumors, at noncontiguous central
nervous system (CNS) sites. Systemic relapse is rare but may occur. At time of
recurrence, a complete evaluation for extent of relapse is indicated for all
malignant tumors and, at times, for lower-grade lesions. Biopsy or surgical
re-resection may be necessary for confirmation of relapse; other entities,
such as secondary tumor and treatment-related brain necrosis, may be clinically
indistinguishable from tumor recurrence. The need for surgical intervention
must be individualized based on the initial tumor type, the length of time
between initial treatment and the reappearance of the lesion, and the clinical
picture.
Childhood Astrocytoma
Childhood astrocytomas are classified as low-grade or high-grade.
Childhood Low-Grade Astrocytomas:
- Pilocytic astrocytoma.
- Diffuse fibrillary astrocytoma.
Childhood High-Grade Astrocytomas:
- Anaplastic astrocytoma.
- Glioblastoma multiforme.
Refer to the PDQ summary on Childhood Astrocytomas Treatment for more information.
Childhood Central Nervous System (CNS) Atypical Teratoid/Rhabdoid Tumor
Refer to the PDQ summary on Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor Treatment for more information.
Childhood Brain Stem Glioma
Childhood brain stem gliomas include:
- Diffuse intrinsic pontine gliomas.
- Focal or low-grade brain stem gliomas.
Refer to the PDQ summary on Childhood Brain Stem Glioma Treatment for more
information.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with
recurrent childhood brain stem glioma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
CNS Tumors in Children Aged 3 Years and Younger
Studies have addressed the treatment of infants who have progressive disease in
spite of chemotherapy. Approaches that have been used include further surgery,
chemotherapy, local and/or craniospinal radiation therapy, high-dose chemotherapy
supported by autologous stem cell rescue, or combinations of chemotherapy and
radiation therapy. Overall salvage rates have been less than optimal, but a
subgroup of children, primarily those with localized disease at the time of
relapse, may experience prolonged disease control and possible cure with
treatment after recurrence.[2][3][4][5][6][7] For children aged 2 years and younger, the use of high-dose craniospinal irradiation has been associated with poor neurocognitive outcome. Treatment for young children with multiple
recurrent and/or disseminated brain tumors is even more problematic and entry
into phase I and phase II trials is indicated to identify more effective and less
toxic agents.
Childhood CNS Embryonal Tumors
Childhood CNS embryonal tumors include:
- CNS atypical teratoid/rhabdoid tumors. (Refer to the PDQ summary on Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor Treatment for more information.)
- Ependymoblastomas.
- Medulloblastomas.
- Medulloepitheliomas.
- Pineal parenchymal tumors of intermediate differentiation.
- Pineoblastomas.
- Supratentorial primitive neuroectodermal tumors.
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Childhood CNS Germ Cell Tumors
Childhood CNS germ cell tumors include:
- Germinomas.
- Embryonal yolk sac tumors.
- Choriocarcinomas.
- Immature teratomas.
- Mature teratomas.
- Teratomas with malignant transformation.
- Mixed germ cell tumors.
- Nongerminomatous germ cell tumors.
Germ cell tumors may be chemoresponsive. Patients may benefit from the types
of agents that are used to treat germ cell tumors in other locations; these agents include cisplatin, etoposide, and cyclophosphamide. Patients with recurrent germ cell tumors for whom the
standard chemotherapy options have failed may be entered into phase I and phase
II studies that are designed to determine the activity and toxic effects of
agents new to the treatment of this tumor.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with
childhood central nervous system germ cell tumor. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Childhood Craniopharyngioma
Refer to the PDQ summary on Childhood Craniopharyngioma Treatment for more information.
Childhood Ependymoma
Refer to the PDQ summary on Childhood Ependymoma Treatment for more information.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with
recurrent childhood ependymoma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Childhood Ependymoblastoma
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Childhood Low-Grade Glial Tumors
Surgical resection, radiation therapy (especially if not previously given),
and chemotherapy may result in prolonged disease stabilization for children
with recurrent low-grade tumors. Resection is an option for those patients
with a surgically accessible lesion and has the advantage of documenting the
histology of the recurrent tumor. Radiation therapy, if not previously given,
may result in tumor shrinkage and long-term disease control.
Chemotherapy with drugs such as carboplatin and vincristine has recently been
shown to result in tumor shrinkage and disease control for children with
low-grade glial neoplasms.[8] Similar results have been demonstrated for
hypothalamic and chiasmatic tumors treated with etoposide.[9] Entry into phase
I and phase II trials is indicated to identify more effective and less toxic
agents.
Childhood Medulloblastoma
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with
recurrent childhood medulloblastoma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Childhood Medulloepithelioma
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with
childhood medulloepithelioma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Childhood Pineal Parenchymal Tumors
Childhood pineal parenchymal tumors include:
- Pineoblastomas.
- Pineocytomas.
- Pineal parenchymal tumors of intermediate differentiation.
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with
recurrent childhood pineoblastoma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Childhood Spinal Cord Tumors
At the time of recurrence, low-grade spinal cord glial tumors can be
treated with re-resection with or without the use of radiation therapy.
Recurrent low-grade and high-grade tumors which cannot be re-resected can be
treated on protocols designed for histologically similar brain tumors.
For more information, refer to the PDQ summaries on Childhood Ependymoma Treatment and Childhood Central Nervous System Embryonal Tumors Treatment.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with
recurrent childhood spinal cord neoplasm. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Childhood Supratentorial Primitive Neuroectodermal Tumors
Childhood suprantentorial primitive neuroectodermal tumors include:
- Primitive neuroectodermal tumors.
- Cerebral neuroblastomas.
Refer to the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with
recurrent childhood supratentorial primitive neuroectodermal tumor. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
References:
Jenkin D, Greenberg M, Hoffman H, et al.: Brain tumors in children: long-term survival after radiation treatment. Int J Radiat Oncol Biol Phys 31 (3): 445-51, 1995.
Fisher PG, Needle MN, Cnaan A, et al.: Salvage therapy after postoperative chemotherapy for primary brain tumors in infants and very young children. Cancer 83 (3): 566-74, 1998.
Walter AW, Mulhern RK, Gajjar A, et al.: Survival and neurodevelopmental outcome of young children with medulloblastoma at St Jude Children's Research Hospital. J Clin Oncol 17 (12): 3720-8, 1999.
Goldwein JW, Glauser TA, Packer RJ, et al.: Recurrent intracranial ependymomas in children. Survival, patterns of failure, and prognostic factors. Cancer 66 (3): 557-63, 1990.
Dupuis-Girod S, Hartmann O, Benhamou E, et al.: Will high dose chemotherapy followed by autologous bone marrow transplantation supplant cranio-spinal irradiation in young children treated for medulloblastoma? J Neurooncol 27 (1): 87-98, 1996.
Dunkel IJ, Boyett JM, Yates A, et al.: High-dose carboplatin, thiotepa, and etoposide with autologous stem-cell rescue for patients with recurrent medulloblastoma. Children's Cancer Group. J Clin Oncol 16 (1): 222-8, 1998.
Guruangan S, Dunkel IJ, Goldman S, et al.: Myeloablative chemotherapy with autologous bone marrow rescue in young children with recurrent malignant brain tumors. J Clin Oncol 16 (7): 2486-93, 1998.
Packer RJ, Lange B, Ater J, et al.: Carboplatin and vincristine for recurrent and newly diagnosed low-grade gliomas of childhood. J Clin Oncol 11 (5): 850-6, 1993.
Chamberlain MC, Grafe MR: Recurrent chiasmatic-hypothalamic glioma treated with oral etoposide. J Clin Oncol 13 (8): 2072-6, 1995.
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Important:
This information is intended mainly for use by doctors and other health care professionals. If you have questions about this topic, you can ask your doctor, or call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237).
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This information is provided by the National Cancer Institute.
This information was last updated on October 14, 2009.