The brain and spinal cord make up the central nervous system (CNS). Tumors of the central nervous system are the second most common cancer in children under 15 years of age (after leukemia) and the leading cause of death in children from cancer.
The most common central nervous system tumors in children are (in order) astrocytomas, medulloblastomas, and ependymomas.
Less common central nervous system tumors in children include craniopharyngiomas, meningioma, choroid plexus tumors, CNS germ cell tumors, and other CNS embryonal tumors (for example, pineoblastomas and atypical teratoid/rhabdoid tumors).
Brain tumors can cause various symptoms, including headaches, nausea, vomiting, vision problems, listlessness, and loss of coordination or balance.
The diagnosis is usually based on results of magnetic resonance imaging and a biopsy.
Treatment may involve surgery, radiation therapy, chemotherapy, targeted therapy, or a combination.
This illustration shows the nervous system of an infant. Note that the central nervous system is made up of the brain and spinal cord.
Image Copyright © Nucleus Medical Media. All rights reserved.
The cause of most central nervous system tumors is unknown. However, high doses of radiation to the brain and spinal cord and certain genetic disorders (for example, neurofibromatosis) can cause central nervous system tumors.
(See also brain tumors in adults.)
Starting from the top right label:
Astrocytoma (glioma)
Optic nerve glioma
Craniopharyngioma
Pituitary adenoma
Schwann cell tumor (nerve sheath tumor)
Ependymoma (glioma)
Brain stem glioma
Medulloblastoma
Pineal gland tumor
Meningioma
Oligodendroglioma (glioma)
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Symptoms of Brain and Spinal Cord Tumors in Children
Common brain tumor symptoms result from increased pressure within the skull (intracranial pressure). Pressure may be increased because the tumor blocks the flow of cerebrospinal fluid within the brain (a condition called hydrocephalus) or because the tumor takes up space. Increased pressure can cause the following symptoms:
An enlarged head (macrocephaly) in infants and very young children
Headaches
Nausea and vomiting (often when the child first awakens)
Vision problems, such as double vision or vision loss
Difficulty turning the eyes upward
Changes in behavior or consciousness level, making the child irritable, listless, confused, or drowsy
Seizures
When the tumor involves the spinal cord, symptoms can include the following:
Back pain
Muscle weakness in arms or legs
Loss of sensation in arms or legs
Loss of bladder or bowel control
Difficulty walking or maintaining balance
Other symptoms vary depending on where the tumor is located in the brain or the spine.
Diagnosis of Brain and Spinal Cord Tumors in Children
Imaging tests
Sometimes biopsy or sometimes surgery to remove some or all of the tissue or tumor
Microscopic and molecular (genetic) analysis of the tissue or tumor
Sometimes a spinal tap
Doctors suspect a brain or spinal cord tumor based on symptoms.
Doctors typically do an imaging test such as magnetic resonance imaging (MRI) of the brain, which can usually detect a tumor. Computed tomography (CT) may also be done. Before MRI or CT is done, a contrast agent is usually injected into a vein (intravenously). Contrast agents are substances that make organs and other structures clearer to see on imaging tests.
Once a brain or spinal cord tumor is confirmed by an imaging test, doctors usually remove the tumor, if possible. If removal is not possible, they may do a biopsy to remove a small piece of tissue. Regardless of whether the tumor is removed or biopsied, the tumor tissue is analyzed under a microscope. Based on its appearance under the microscope, doctors can sometimes determine how quickly the tumor is likely to grow or spread. In some cases, the biopsy is not necessary before doctors do the surgery to remove some or all of the tumor.
After biopsy is done or the tumor is removed, doctors look at the tissue or tumor under a microscope and do tests to further analyze the tissue or tumor for specific gene mutations or other molecular features that can occur in that specific tumor type. This information can help doctors make the best treatment decisions and estimate the likelihood of successful treatment and survival.
Sometimes a spinal tap (lumbar puncture) is done to obtain cerebrospinal fluid for examination under a microscope. This procedure is often done to help doctors determine whether tumor cells have spread to the cerebrospinal fluid.
All of this diagnostic information about the tumor's type, appearance, size, spread, and genetic profile is combined to determine the grade and stage, which helps determine the most appropriate treatment (see also Staging Cancer).
Treatment of Brain and Spinal Cord Tumors in Children
Surgical removal of the tumor
Chemotherapy, radiation therapy, targeted therapy, or a combination
Drainage of cerebrospinal fluid
(See also Cancer Treatment Principles.)
Usually, treatment of brain and spinal cord tumors involves surgically removing the tumor. Then, chemotherapy, radiation therapy, targeted therapy, or a combination is used.
Treatment should be planned by a team of experts who have experience treating these tumors in children. The care team may include doctors who specialize in the care and treatment of babies, children, and adolescents, such as pediatric cancer specialists (oncologists), pediatric neurologists, pediatric neurosurgeons, and radiation oncologists.
When possible, doctors remove the tumor surgically. Brain tumors are removed by opening the skull (called a craniotomy), whereas spinal cord tumors are removed through the spine and the outer covering of the spinal cord. Some, but not all, tumors can be removed with little or no damage to the brain or spinal cord. After surgery, doctors do an MRI scan of the brain or spinal cord to determine whether any of the tumor is left and, if so, how much.
After surgery, radiation therapy, chemotherapy, targeted therapy, or a combination of these treatments may be required. In children younger than 5 years of age, depending on the tumor type, chemotherapy may be used first because radiation therapy can interfere with growth and brain development. If needed, radiation therapy may be added when children are older. Chemotherapy may also have serious side effects.
If the tumor is blocking the flow of cerebrospinal fluid, a small tube (catheter) may be used to drain the cerebrospinal fluid before the tumor is surgically removed. After a local or general anesthetic is given, the tube is inserted through a tiny opening drilled in the skull, and fluid is withdrawn to reduce the pressure within the skull. The tube is connected to a gauge that measures the pressure within the skull. After a few days, the tube is removed or may be converted to a permanent drain called a shunt (see Treatment of Hydrocephalus).
Because cancer is relatively rare in children, the doctor may speak with the parents about entry into a clinical trial, if available. In such trials, some children receive the standard treatment, and others receive the treatment being tested (called experimental treatment). The experimental treatment may involve new chemotherapies, new combinations of older medications, or new surgical or radiation techniques. However, experimental treatments are not always effective, and side effects or complications may not be known.
More Information
The following English-language resources may be useful. Please note that The Manual is not responsible for the content of these resources.
American Cancer Society: How to Cope if Your Child Has Cancer
The brain tumors organizations here provide information about types of and treatments for brain tumors as well as information for caregivers about support resources:
American Brain Tumor Association: Pediatric Caregiver Resources Center
