A brain tumor is an abnormal mass of tissue in which some cells grow and multiply uncontrollably, apparently unregulated by the mechanisms that control normal cells. The growth of a tumor takes up space within the skull and interferes with normal brain activity. A tumor can cause damage by increasing pressure in the brain, by shifting the brain or pushing against the skull, and by invading and damaging nerves and healthy brain tissue. The location of a brain tumor influences the type of symptoms that occur. This is because different functions are controlled by different parts of the brain.

Brain tumors rarely metastasize (spread) to other parts of the body outside of the central nervous system (CNS). The CNS includes the brain and spinal cord.

Some tumor types are more common in children than in adults. When childhood brain tumors occur in adults, they often occur in a different part of the brain than in children. Brain tumors in children are often located in the brain stem and cerebellum, while in adults they often occur in the cerebral hemispheres. Although most primary tumors attack member of both sexes with equal frequency, some, such as meningiomas, occur more frequently in women, while others, such as medulloblastomas, more commonly afflict boys and young men.

The prognosis for brain tumor patients is as individual as the patients themselves. Your child’s doctors will help you understand the possible consequences of your child’s specific tumor.

For more information about brain tumor types, see chapter 4 of The Essential Guide to Brain Tumors.

Some of the general symptoms of brain tumors in children are headaches; vomiting (usually in the morning and without nausea); unsteadiness or loss of balance; seizures; double vision or vision problems; decreased coordination; fatigue or sleepiness; weakness on one side of the body; increased size of the head; uncontrolled eye movements; irritability; and behavioral changes.

Symptoms are often vague in children, especially in very young children who are not able to fully describe their symptoms. Some of these symptoms can occur in a variety of more common childhood illnesses. The difference with brain tumors is that these symptoms persist and get worse over time. If your child is experiencing any of the above-mentioned symptoms, it is important to see a doctor and get a definitive diagnosis.

Pediatric brain tumors are not contagious. Their causes are unknown.

Benign brain tumors are slow-growing tumors that can be removed or destroyed if in an accessible location. Malignant tumors (brain cancer) are rapidly growing tumors that invade or infiltrate and destroy normal brain tissue. No one is certain why, but some benign brain tumors may change over time to become malignant.

Tumors are graded to indicate how quickly they are growing. Most medical institutions use the World Health Organization (WHO) classification system to identify brain tumors. The WHO classifies brain tumors by cell origin and how the cells behave, from the least aggressive (benign) to the most aggressive (malignant).Some tumor types are assigned a grade, which signifies the rate of growth. There are variations in grading systems, depending on the tumor type. The classification and grade of an individual tumor help predict its likely behavior.

The distinction between benign and malignant can be ambiguous. Some benign tumors can be as dangerous as malignant ones if in a dangerous or inaccessible location, such as the brain stem. Conversely, some malignant tumors can be cured.

Although they may fall into a specific classification or category, brain tumors are specific to each individual. Brain tumors have vastly different characteristics and patterns of growth due to the molecular makeup of the individual tumor.

For more information about brain tumor types, see chapter 4 of The Essential Guide to Brain Tumors.

A brain tumor diagnosis usually involves several steps, which can include a neurological examination, brain scan(s) and/or a biopsy.

Should a child’s symptoms lead the doctor to suspect a tumor, a neurological examination will be given. This is a series of tests to measure the function of the child’s nervous system and physical and mental alertness. If responses to the neurological exam are not normal, the doctor may order a scan.

A brain scan is a picture of the internal structures in the brain. A specialized machine takes a scan in much the same way a digital camera takes a photograph. Using computer technology, a scan compiles an image of the brain by photographing it from various angles. Magnetic Resonance Imaging (MRI) and computerized tomography (CT) scans may confirm the presence and location of a tumor. Other studies, such as an electroencephalogram (EEG) or examination of cerebrospinal fluid (CSF) may also be done.

MRI (Magnetic Resonance Imaging) is a scanning device that uses magnetic fields and computers to capture images of the brain on film. It does not use x-rays. It provides pictures from various planes, which permits doctors to create a three-dimensional image of the tumor. The MRI detects signals emitted from normal and abnormal tissue, providing clear images of most tumors.

CT or CAT Scan (Computed Tomography) combines sophisticated x-ray and computer technology. CT can show a combination of soft tissue, bone, and blood vessels. CT images can determine some types of tumors, as well as help detect swelling, bleeding, and bone and tissue calcification.

CT is a valuable diagnostic tool and its use has been increasing rapidly. However, CT scans involve exposure to ionizing radiation, which is known to cause cancer. This is a concern for children, because they are more sensitive to radiation than adults. It is wise for parents of children who have brain tumors to keep a record of their x-ray history. This information can help doctors make informed decisions and minimize radiation overexposure.

Some types of scans use a contrast agent, which helps the doctor to see the difference between normal and abnormal brain tissue. The contrast agent is injected into a vein and flows into brain tissue. Abnormal brain tissue absorbs more dye than normal tissue. Contrast agents may cause allergic reactions in some patients. Gadolinium, the contrast agent used with an MRI, may cause temporary headaches but has no other known side effects. Usually, iodine is the contrast agent used during a CT scan. If your child is allergic to iodine, tell your doctor beforehand.

The most accurate diagnosis of a brain tumor is made with surgery, which permits the neurosurgeon to see the tumor and obtain a specimen for a pathological examination.  

A biopsy is a surgical procedure in which a sample of tissue is taken from the tumor site and examined under a microscope. The biopsy will provide information on types of abnormal cells present in the tumor. The purpose of a biopsy is to discover the type and grade of a tumor. An open biopsy is done during a craniotomy. A craniotomy involves removing a piece of the skull in order to get access to the brain. After the tumor is resected (completely removed)or debulked (partially removed), the bone is usually put back into place. A closed biopsy (also called stereotactic or needle biopsy) may be performed when the tumor is in an area of the brain that is difficult to reach. In a closed biopsy, the neurosurgeon drills a small hole into the skull and passes a narrow, hollow needle into the tumor to remove a sample of tissue.

Once a sample is obtained, a pathologist examines the tissue under a microscope and writes a pathology report containing an analysis of the brain tissue. Sometimes the pathologist may not be able to make an exact diagnosis. This may be because more than one grade of tumor cells exists within the same tumor. In some cases, the tissue may be sent to another institution for additional analysis.

The standard treatments for brain tumors are surgery, radiation therapy, and chemotherapy. In some cases when the tumor is slow growing, the treatment team may delay surgery and use frequent scans to monitor the tumor's growth.

Sometimes surgery alone will cure a brain tumor. In general, radiation and chemotherapy treatments are used as secondary or adjuvant treatments for tumors that cannot be managed using only surgery. However, radiation and chemotherapy may be used without surgery if the tumor is inoperable. Use of radiation therapy is avoided in children below the age of three because it causes significant long-term damage to the developing brain.

In many cases, at the time of diagnosis, treatment decisions must be made quickly, especially with regard to surgery. When possible, the doctor and family will discuss a treatment plan based on the type and location of the tumor. In treating brain tumors, a multi-disciplinary treatment team, made up of various specialists, is generally considered the preferred approach. Neurosurgeons, neurologists, radiation oncologists, pathologists, and other medical professionals may be part of the treatment team.

The goal of brain surgery, or resection, is to remove as much of a tumor as possible without causing damage to critical neurological functions. When only part of a tumor can be resected, it is possible that the tumor will recur (grow back). However, a partial resection can sometimes relieve symptoms and improve the effectiveness of other therapies.

Some childhood brain tumors block the flow of cerebrospinal fluid (CSF) in the ventricles (spaces within the brain), leading to hydrocephalus (water on the brain). This condition causes increased intracranial pressure, leading to neurological problems. In such cases, the neurosurgeon will implant a ventriculoperitoneal (VP) shunt to drain the excess fluid from the ventricles into the abdominal cavity and relieve the pressure in the brain. This type of surgery is performed under general anesthesia. A small hole is drilled into the skull and a small catheter (tube) is passed into a ventricle of the brain. A valve which controls the flow of fluid is attached to the catheter to keep the fluid away from the brain. Another catheter is attached to the valve and tunneled under the skin, behind the ear, down the neck and chest, and into the peritoneal (abdominal) cavity. The excess fluid is reabsorbed by the body. Ask your neurosurgeon about the type of shunt, potential problems and what symptoms to look out for that might indicate a problem, and the expected duration of shunt placement.

Radiation therapy uses high-energy x-rays or other types of ionizing radiation to stop cancer cells from dividing. Radiation therapy may be used when surgery is not advised, for tumors that cannot be completely resected, or after surgery to prevent or delay tumor recurrence. Radiation therapy can stop or slow the growth of inoperable tumors.

Conventional radiation therapy delivers radiation to an entire region of the brain. The radiation is fractionated into many small doses and given over a period of time (usually five to seven weeks, excluding weekends). Depending on the location, type, and size of the tumor(s), the treatment may be either focused or whole brain radiation therapy (WBRT). Focused radiation therapy aims x-rays at the tumor and area surrounding it. WBRT aims radiation at the entire brain.

Stereotactic Radiosurgery (SRS) delivers a single, high dose of radiation in a one-day session. a head frame or mask is used to hold the head in position, then CT or MRI scans are taken. With the aid of computer imaging, the location of the tumor is accurately calculated. The radiation is delivered directly to the tumor, often from several different directions. Size and location of the tumor are important eligibility criteria for SRS.

The goal of radiation therapy is to give the maximum amount of radiation to tumor cells while sparing healthy brain tissue.

Chemotherapy uses chemicals (drugs) that have a toxic effect on tumor cells as they divide. Chemotherapy is usually taken orally or by injection, and may be given alone or in combination with other treatments. Chemotherapy is given in cycles, which consist of “on” and “off” phases – days of treatment followed by periods of time between treatments. Cycles vary depending on the drug or drugs used. Chemotherapy is usually a secondary therapy. It is also used to delay or replace radiation treatment in young children.

Most chemotherapy drugs enter and affect the entire body, causing a range of side effects. Side effects are caused when the drugs damage normal cells that are dividing. Reactions can range from mild to severe. All side effects should be reported to the doctor. There are treatments available to alleviate many of these problems. Antiemetics (antinausea drugs) have greatly reduced the nausea chemotherapy patients have experienced in the past.Your child’s doctors will work with you to minimize or prevent anticipated side effects. In some cases, the type of chemotherapy drug may be changed.

For more information about brain tumor treatments, see chapter 5 of The Essential Guide to Brain Tumors.

More information on shunts can be obtained from the Hydrocephalus Association at 888.598.3780 or www.hydroassoc.org.

There are many medical centers with teams that specialize in treating pediatric brain tumor patients. There is no one “best” doctor, but there are issues to consider that can help guide you in choosing the doctor that is best for your child. Good communication is key to a good relationship with your doctors. If you ask your questions and get them answered, the experience will be easier for you and your family. If you are not comfortable with your doctor, consider changing doctors.

Getting a second opinion is important if your child’s doctor does not have experience with brain tumors or if you want to confirm your primary physician’s recommendation. Ask your doctor for a referral to a specialist. You can also consult the information in our Treatment Center Database to research different treatment centers, or call NBTF. We can provide you with phone numbers of comprehensive cancer centers and medical centers of excellence specific to pediatric brain tumors.

There are many important questions to ask when choosing a treatment center. Does the center have specialists in neurosurgery, neurology, neuro-oncology and radiation oncology? What types of imaging technology is available? How many patients does the medical center diagnose and treat per year?

Many people are unsure of what to look for. Our fact sheet, Issues to Consider When Choosing a Treatment Center, may be helpful in this process.

Click here for the fact sheet How and Why to Get a Second Opinion.

Many people rely on family and friends or their spiritual community for support. Medical social workers can connect you with local resources. Support groups, including Internet-based groups, can often provide contact with other people in a similar situation. A good support system is important to help families cope with the crisis brought on by a child’s brain tumor diagnosis.

There are organizations with resources available to help patients and their families cope with the disease. Some hold summer camps for children with cancer and their siblings. Wish-fulfillment agencies grant wishes for children with life-threatening illnesses.

You can post a message on the NBTF Message Boards, visit our Link Library for pediatric cancer resources, or call NBTF at 800.934.2873 for more information and referrals.

Frequent or prolonged absence from the classroom may disrupt the learning process. A child with a brain tumor may face the additional challenge of physical changes in the structure of the brain, which affect the thought and learning processes. It is important to identify these changes and to adopt teaching and learning strategies that capitalize on the child's strengths and compensate for the child's weaknesses. These changes can best be identified by neuropsychological testing.

Neuropsychological testing measures cognitive skills (memory, learning, language), motor skills, and social skills. It is used to help schools plan for optimal educational interventions. Child Development Centers associated with major medical centers generally have neuropsychologists who are skilled not only in administering testing but also in making recommendations for optimal functioning of the child. Many insurance companies require a letter of necessity from your child's doctor to cover this service. Once a child's educational needs have been identified, those involved in meeting these needs must decide upon a plan of action (called an Individualized Educational Plan or IEP) to ensure that these needs are met.

Prior to a full return to school, the social worker or school liaison on your child's treatment team can help your child to participate in school activities to the best of his or her current ability and energy. This can be done through partial attendance, hospital-based schooling, or "home-bound" tutoring. When your child is able to return to school more fully, there are several steps you can take to ease that transition.

How a child is feeling on any given day will also affect his or her academic performance. The attitude of the child's teacher and peers toward to child who is ill may also affect his or her behavior and performance in school.

Some children can continue to attend school while in treatment. Many are at risk of problems with their self-image and relationships with peers, especially if educators and classmates misunderstand the side effects of the child's illness and treatments. To make the transition back to school easier, the teacher and school nurse should be encouraged to prepare classmates by supplying them with information and answering their questions. Some medical centers provide a child life worker and health care practitioner who can help prepare the class for your child's return. Ask your child's healthcare team about how they can help.

You can visit our Link Library for other pediatric resources or post a message on the NBTF Message Boards. In addition, you can call NBTF or contact the agencies below for more information and referrals.

Childrens Brain Tumor Foundation
866.228.HOPE (866.228.4683) www.cbtf.org

Brain Tumor Foundation for Children, Inc.
404.252.4107 www.braintumorkids.org

Pediatric Brain Tumor Foundation of the United States
800.253.6530 www.pbtfus.org

For a free copy of A Resource Guide for Parents of Children with Brain or Spinal Cord Tumors available in English and Spanish (Guia de recursos para padres de niños con tumors cerebrales o de médula espinal), contact the Childrens Brain Tumor Foundation at 866.228.HOPE (866.228.4683) or email info@cbtf.org.

Childhood Brain & Spinal Cord Tumors: A Guide for Families, Friends & Caregivers by Tania Shiminski-Maher, Patsy Cullen, and Maria Sansalone is a useful resource available in bookstores.

Some tumor types are more common in children than in adults. The most common types of childhood tumors are brain stem gliomas, craniopharyngiomas, ependymomas, juvenile pilocytic astrocytomas, medulloblastomas, optic nerve gliomas, pineal tumors, primitive neuroectodermal tumors (PNET), and rhabdoid tumors.

Brain Stem Glioma

Characteristics

• Named for its location at the base of the brain
• Can range from low grade to high grade
• Occurs most often in children between three and ten years of age, but can occur in adults

Symptoms

• Headaches
• Nausea
• Speech or balance abnormalities
• Difficulty swallowing
• Weakness or numbness of the arms and/or legs
• Facial weakness
• Double vision

Symptoms can develop slowly and subtly and may go unnoticed for months. In other cases, the symptoms may arise abruptly. A sudden onset of symptoms tends to occur with rapidly growing, high-grade tumors.

Treatment

Surgery may not be an option because the brain stem controls vital life functions and can easily be damaged. Radiation therapy can reduce symptoms and help slow the tumor’s growth. Low-grade brain stem gliomas can have very long periods of remission.  

Craniopharyngioma

Characteristics

• Most common in the parasellar region, an area at the base of the brain and near the optic nerves
• Also grows in the regions of the optic nerves and the hypothalamus, near the pituitary gland
• Tends to be low grade
• Often accompanied by a cyst
• Originates in cells left over from early fetal development
• Occurs in children and men and women in their 50s and 60s

Symptoms

• Headaches
• Visual changes
• Weight gain
• Delayed development in children

Treatment

Surgery is the most common treatment. Radiation therapy may be used.

Ependymoma

Ependymal tumors begin in the ependyma, cells that line the passageways in the brain where cerebral spinal fluid (CSF) is produced and stored. Ependymomas are classified as either supratentorial (in the cerebral hemispheres) or infratentorial (in the back of the brain). Variations of this tumor type include subependymoma, subependymal giant-cell astrocytoma, and malignant ependymoma. Ependymoblastoma, which occurs in infants and children under three years, is no longer considered a subtype of ependymoma. For ependymoblastoma, see primitive neuroectodermal tumor (PNET) below.

Characteristics

• Usually localized to one area of the brain
• Develops from cells that line the hollow cavities at the bottom of the brain and the canal containing the spinal cord
• Can be slow growing or fast growing
• May be located in the ventricles
• May block the ventricles, causing hydrocephalus (water on the brain)
• Sometimes extends to the spinal cord
• Common in children, and among men and women in their 40s and 50s
• Occurrence peaks at age five and again at age 34
• Accounts for two percent of all brain tumors

Symptoms

• Severe headaches
• Nausea and vomiting
• Difficulty walking
• Fatigue and sleepiness
• Problems with coordination
• Neck pain or stiffness
• Visual problems

Treatment

The doctor will perform tests to determine if it has spread to the spinal cord. Surgery followed by radiation therapy is the usual course of treatment. A shunt may be needed to treat hydrocephalus caused by blockage of the ventricles.

Juvenile Pilocytic Astrocytoma (JPA)

This tumor is also known as a pilocytic astrocytoma, or by the initials JPA. Astrocytoma tumors develop from star-shaped glial cells (astrocytes) that support nerve cells. Astrocytomas are the most common primary CNS tumor. Astrocytomas are generally classified as low or high grade. Low-grade astrocytomas are slow growing. High-grade astrocytomas (grades three and four) grow more quickly. Juvenile Pilocytic Astrocytoma is a low-grade (grade one) tumor. Symptoms tend to be subtle and may take one to two years to diagnose. This is because the brain can often adapt to a slow-growing tumor for a period of time. High-grade tumors may present with changes that are sudden and dramatic.

Characteristics

• Slow growing, with relatively well-defined borders
• Grows in the cerebrum, optic nerve pathways, brain stem and cerebellum
• Occurs most often in children and teens
• Accounts for two percent of all brain tumors

Symptoms

• Headaches
• Seizures or convulsions
• Difficulty thinking or speaking
• Behavioral or cognitive changes (related to thinking, reasoning, and memory)
• Weakness or paralysis in one part or one side of the body
• Loss of balance
• Vision changes
• Nausea or vomiting

Treatment

Surgery is the standard treatment. If the tumor cannot be completely resected, radiation or chemotherapy may be given. Chemotherapy may be given to very young children instead of radiation therapy to avoid damage to the developing brain. Some of these tumors can progress to a higher grade, so it is important to be diligent about following up with the medical team after treatment.

Medulloblastoma

Characteristics

• A type of primitive neuroectodermal tumor (PNET) (see below)
• Often located in the cerebellum or near the brain stem
• Can spread to the spinal cord through the CSF
• May obstruct the fourth ventricle, causing hydrocephalus
• Occurs most often in children under the age of ten, but may occur in adults
• Slightly more common in males than females

Symptoms

• Headaches
• Early morning vomiting
• Lethargy or sleepiness
• Lack of coordination
• Double vision
• Behavioral or personality changes
• Signs of pressure seenbehind the eye when examined with an ophthalmoscope

Treatment

Surgery is the standard treatment when possible. Chemotherapy is usually part of the treatment plan. Radiation of the brain and spine is often recommended in adults and children over three years of age. A shunt may be needed to treat hydrocephalus. This tumor may recur years later if not totally resected.

Optic Nerve Glioma

Characteristics

• Named for its location on or near the nerve pathways between the eyes and the brain
• Can range from low grade to high grade
• Occurs most often in infants and children, but can occur in adults

Symptoms

• Headaches
• Progressive loss of vision
• Double vision

Treatment

Surgery is standard treatment, usually followed by radiation therapy or chemotherapy. Chemotherapy may be given to very young children instead of radiation therapy to avoid damage to the developing brain.

Pineal Tumor

A malignant form of pineal tumor is called pineoblastoma.

Characteristics

• Named for its location in or around the pineal gland (near the center of the brain)
• Can range from low grade to high grade
• Can produce an excess of melatonin, a hormone that controls the sleep/wake cycle
• Can block the ventricles, causing hydrocephalus 
• High-grade pineal tumors can spread to the spinal cord through the CSF
• Common types include germ cell tumors, pineal parenchymal tumors, and gliomas
• Occurs most often in children and young adults

Symptoms

• Headaches
• Nausea and vomiting
• Fatigue
• Double vision
• Memory problems

Treatment

Surgery is standard treatment when possible. Radiation therapy may be used as primary treatment in adults and children over three. Chemotherapy may be given to delay the use of radiation therapy in very young patients. Clinical trials using chemotherapy drugs are available for pineal tumors. A shunt may be needed to treat hydrocephalus caused by blockage of the ventricles. Treatment for high-grade (malignant) pineal tumors such as a pineoblastoma may involve radiation to the brain and spine to control spread through the CSF. Clinical trials using chemotherapy or biological therapy following radiation therapy are being investigated.

Primitive Neuroectodermal Tumors (PNET)

There are several tumor types in this category. Names of specific PNETs may be based on the tumor location. Examples include pineoblastoma (located in the pineal region), medulloblastoma (located in the cerebellum), and cerebral cortex PNET (located in the cerebral cortex).

Characteristics

• Highly aggressive and tend to spread throughout the CNS
• Grow from undeveloped brain cells
• Commonly include cysts and calcification (calcium deposits)
• Tend to be large
• Occur most often in young children

Symptoms

• Can vary depending on location of tumor
• Weakness or change in sensation on one side of the body
• Morning headache or headache that goes away after vomiting
• Nausea and vomiting
• Seizures
• Unusual sleepiness or lethargy
• Behavioral or personality changes
• Unexplained weight loss or weight gain

Treatment

Surgery is the standard treatment when possible. In adults and children over three years of age, surgery may be followed by radiation therapy to the whole brain and spinal cord, and chemotherapy. In children under three years of age, surgery may be followed by chemotherapy or a clinical trial of chemotherapy to delay or reduce the need for radiation therapy.

Rhabdoid Tumor

Characteristics

• Rare
• Highly aggressive and tends to spread throughout the CNS
• Often appears in multiple sites in the body, especially the kidneys
• Difficult to classify; may be confused with medulloblastoma or PNETs
• Occurs most often in young children but can also occur in adults

Symptoms

• Vary depending on location of tumor in the brain or body
• An orbital tumor may cause the eye to protrude
• Balance problems may occur
• External tumors cause noticeable lumps; internal tumor symptoms vary based on location

Treatment

Whenever possible, surgery is performed to remove as much of the tumor as possible. This is usually followed by chemotherapy and radiation therapy. In children under three years of age, surgery may be followed by chemotherapy alone. Clinical trials are being studied using autologous bone marrow transplantation after high-dose chemotherapy for recurrent or multiple rhabdoid tumors.