Brain Tumors

  • Dana-Farber / Children's Hospital Cancer Center logo

    A brain tumor refers to the growth of abnormal cells in the tissues of the brain. Brain tumors can be benign or malignant. Learn about brain tumors and find information on how we support and care for children and teens with brain tumors before, during, and after treatment.

The Brain Tumor Center at Dana-Farber/Boston Children's Cancer and Blood Disorders Center cares for children with many different types of common and rare brain and spinal tumors, including astrocytomas, medulloblastomas, ependymoma, glioblastomas, and primitive neuroectodermal tumors (PNET).

Your child will receive care from some of the world’s most experienced pediatric brain tumor doctors and internationally recognized pediatric subspecialists.

Our team works closely together to develop a care plan that offers your child the highest possible quality of life after treatment, and takes the needs of your child and your family into account.

Children treated at the Brain Tumor Center have access to some of the most advanced diagnostics and therapies, including:

  • Quick and accurate diagnosis from our dedicated pediatric neuropathologist
  • Access to advanced technologies like the intraoperative MRI, which allows our neurosurgeons to see detailed images of the brain during surgery
  • Advanced pediatric radiation oncology services, including targeted radiosurgery and low-dose radiation therapy that minimize exposure to radiation
  • Outpatient and oral chemotherapy, which may minimize the number of times your child will need to visit the hospital
  • Innovative therapies offered through clinical trials at Dana-Farber, Boston Children's Hospital, and nationally
  • Specialized programs for the treatment of low- and high-grade gliomas, and medulloblastoma

Thanks to refined surgical techniques and improved chemotherapy and radiation therapy, the majority of children with brain and spinal cord tumors are now long-term survivors. However, they may face physical, social, and intellectual challenges that require specialized care.

Learn more about our Brain Tumor Center.

Information for: Patients | Healthcare Professionals

Brain Tumors

Overview

Having a tumor in the brain is always a very serious matter, but today, more than half of all children diagnosed with a brain tumor will be cured of the disease. Tumors are masses of abnormal cells that grow out of control. When these tumors originate in the brain, they can be very complicated to treat because of the delicate surrounding tissue.

While all brain tumors are life-threatening, most children and adolescents who have been diagnosed with one survive into adulthood. Many of them face physical, psychological, social and intellectual challenges related to their treatment, and require ongoing care to help with school and with skills they will use throughout adulthood.

  • Brain tumors in children are relatively rare, occurring in only five of every 100,000 children.
  • About 2,200 children and adolescents in the United States are diagnosed with a brain tumor each year.
  • Brain tumors are commonly treated with surgery and/or other therapies including chemotherapy and radiation.

As you read on, you’ll find an overview of pediatric brain tumors. If you would like to read information about a specific type of brain tumor, click here for a list of pediatric brain tumors we treat.

How Dana-Farber/Boston Children's Cancer and Blood Disorders Center Approaches Pediatric Brain Tumor

Children with brain tumors are treated through the Pediatric Brain Tumor Center at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center, an integrated pediatric hematology and oncology partnership between Dana-Farber Cancer Institute and Boston Children’s Hospital – and a world-renowned destination for children with malignant and non-malignant brain and spinal cord tumors.

Our pediatric neuro-oncology, neurosurgical and neurology specialists at Dana-Farber/Boston Children's offer:

  • technological advances, such as the intra-operative MRI, which allow our pediatric neurosurgeons to “see” the tumor as they operate with MRI scans. This allows them to remove as much of the tumor as possible.
  • treatment with the best standard of care, including neurosurgery, radiation therapy and chemotherapy
  • access to unique Phase I clinical trials run by our own investigators, Children’s Oncology Group, Department of Defense and the Pediatric Oncology Experimental Therapeutics Consortium

Through the Stop & Shop Neuro-Oncology Outcomes Clinic at Dana-Farber Cancer Institute, your child will be able to meet with his entire care team at the same follow-up visit.

  • Our pediatric brain tumor survivorship clinic is held weekly.
  • In addition to meeting with your pediatric neuro-oncologists, neurologist and neurosurgeon, your child may also see one of our endocrinologists or alternative/complementary therapy specialists.
  • School liaisons and psychosocial personnel from the pediatric brain tumor team are also available.
  • If your child needs rehabilitation, he may also meet with speech, physical, and occupational therapists during and after treatments.

We understand how scary and overwhelming a diagnosis of a brain tumor can be. Right now, you probably have a lot of questions. What is the very best treatment for my child? What do we do next? 

We’ve tried to provide some answers to those questions in the following pages, and our expert pediatric subspecialists can explain your child’s condition fully when you meet with us.

About the brain

As you know, the brain is an important organ that controls a lot of what we do — voluntarily (like thinking) or involuntarily (like breathing). The brain controls thought, memory, emotion, touch, motor skills, vision, respiration, temperature, hunger and processes that regulate the body.

The brain can be divided into three main parts:

  • the cerebrum
  • the brainstem
  • the cerebellum

1. The cerebrum is composed of the right and left hemispheres. Its functions include:

  • initiation of movement
  • coordination of movement
  • temperature sensitivity
  • touch
  • vision
  • hearing
  • judgment
  • reasoning
  • problem-solving
  • emotions
  • learning

2. The brainstem includes the midbrain, the pons, and the medulla. This area is responsible for:

  • movement of the eyes and mouth
  • relaying sensory messages (i.e., heat, pain, sound)
  • hunger
  • respiration
  • consciousness
  • cardiac function
  • body temperature
  • involuntary muscle movements
  • sneezing
  • coughing
  • vomiting
  • swallowing

3. The cerebellum is located at the back of the head. Its functions are to:

  • coordinate voluntary muscle movements
  • maintain posture, balance and equilibrium

4. The spinal cord contains bundles of nerve fibers that emanate from the brain and spread out to all parts of the body. These fibers allow signals from the brain to travel to and communicate with different parts of the body.

5. To protect all of these structures, the brain and spinal cord are encased in solid bones wrapped in a layer of tissue called meninges.

6. In addition, the brain floats in a liquid called cerebral spinal fluid, which acts as a shock absorber to protect the brain. This fluid is made in small spaces within the brain, called ventricles.

7. The brain is connected to the face by twelve specific cranial nerves that control most eye, face and tongue movements.

What causes brain tumors?

Although we do not know for certain why one child gets brain cancer while another does not, we know that a small number of brain tumors can be related to genetics. Two things are true of all genetic conditions:

  • Their root cause is located in the body, on the cellular level.
  • They are (most likely) inherited, meaning that defective genes are passed on from parent to child.

Because of this, we can look at what “causes” brain tumors in two different ways—how they occur in the body on the cellular level, and how a child comes to inherit the gene.

(Remember: your child may have a brain tumor because he has inherited certain genes from his parents, or because his genes have mutated on their own.)

On the cellular level 

In each cell are long molecules called chromosomes. These contain genes that determine such things as height, hair color and the shape of our face. They also contain “control signals” that tell a cell when to divide, when to stop dividing, or when to die. Sometimes, these control signals become damaged. This damage is thought to create a mutant gene.

A mutant gene could do (at least) two things wrong:

  • it actively tells a cell to keep dividing (like having your foot stuck on the gas pedal)
  • it lacks the ability to tell the cell to stop dividing (like having no brakes)

If you have your foot stuck on the gas but your brakes still work, it’s okay because the brakes will let you stop. Similarly, cells have safeguards so that in case something goes wrong, mutant genes are not created. For most pediatric brain tumors, several safeguards must have failed, and created mutations in several genes.

Inheriting the gene 

Again, these damaged genes can be created spontaneously or inherited. Everyone has two copies of each gene (one from each parent). In order for a tumor to grow, both copies of the gene must become damaged within the child’s body, or be already damaged and inherited—one from each parent.

Trying to find which genes have been mutated to allow a tumor to grow is like finding a needle in a haystack, but researchers all over the world are devoted to this task.

Why do brain tumors sometimes have more than one name?

Tumors have been classified (named) according to a number of different principles.

The old way: Tumors used to be classified according to how they looked under the microscope (and often their location in the brain).

The new way: As researchers have come to understand more about the cellular and molecular differences between groups of tumors, the names have changed.

This is important because it means that many tumors have more than one name. To make it even more confusing, different tumors can have the same name. Make sure you know both:

  • the name that the pathologist (a doctor who specializes in analyzing body tissues on a cellular level) gave to your child’s tumor
  • which staging (or naming) system was used

How are brain tumors classified? 

Dana-Farber/Boston Children's and Brigham and Women's Hospital use the World Health Organization (WHO) classification system for most pediatric brain tumors. This system incorporates:

  • different aspects of the appearance of the cells, which indicate what type of brain cell the tumor arose from and how "aggressive" (likely to spread) the cells are likely to be
  • the tumor's location within the brain
What is meant by the “grade” of the tumor?  

Many pediatric brain tumors have a second important component to them after their name, which is the grade. This is an estimate of how aggressive or malignant a particular type of tumor is.

We can use glial tumors (which are also known as astrocytomas) as an example. Glial tumors come in four grades. Grade I is the lowest, meaning that these tumors tend to, on average, be less aggressive then their grade IV counterparts, which are usually highly malignant and very difficult to treat.

The grade is based on a number of factors, such as how many cells are dividing at any one time or how different the cells look from their normal counterparts.

What does it mean to say that a tumor is “benign” or “malignant”?

Tumors can also be classified as benign or malignant:

  • Benign tumors usually remain localized, without the ability to spread. Complete removal of the tumor is usually all that is required for treatment.
  • Malignant tumors can spread and invade other areas. This means that even if the tumor is surgically removed, other cells will grow back, continuing to invade your child’s body.

Remember that all tumors of the central nervous system are dangerous. Even benign tumors can be fatal if they press on certain vital areas, or if they cannot be completely removed and continue to grow.

Symptoms

What are the symptoms of a brain tumor?

Each child may experience symptoms differently, and symptoms vary depending on size and location of the tumor — both in the brain and elsewhere in the central nervous system.

There is no space in the skull for anything except for the brain and its fluid. This means that any tumor, extra tissue or fluid can cause pressure on the brain.

Symptoms related to pressure on the brain can include:

  • headache
  • vomiting (usually in the morning)
  • nausea
  • personality changes
  • irritability
  • drowsiness
  • depression
  • decreased cardiac and respiratory function, and even eventually coma if left untreated

Symptoms of brain tumors in the cerebellum may include:

  • vomiting (usually occurs in the morning without nausea)
  • headache
  • uncoordinated muscle movements
  • problems walking

Brain tumors in the lower part of the brain often press on the cerebellum. This may cause symptoms including:

  • problems walking
  • loss of control of the nerves and/or muscles of the face

Brain tumors in the brainstem may compress nerves and cause symptoms including:

  • visual changes such as double vision
  • paralysis of nerves and/or muscles of the face, or half of the body
  • respiratory changes
  • clumsy, uncoordinated walk

Symptoms of brain tumors in the cerebrum may include:

  • seizures
  • visual changes
  • slurred speech
  • paralysis or weakness on half of the body or face
  • drowsiness and/or confusion
  • personality changes and/or impaired judgment
  • short-term memory loss
  • ataxia (problems walking)
  • communication problems

Symptoms of brain tumors in the optic pathway (eyes) may include:

  • visual problems
  • puberty or growth abnormalities
  • excessive urination

Symptoms of tumors in the spine (usually spreading from a tumor at a higher point on the spinal cord) may include:

  • bowel or bladder dysfunction
  • back pain
  • focal weakness or sensory loss, depending on where in the spine the disease is located

It’s important to remember that the symptoms of a brain tumor may resemble other, more common conditions or medical problems. Always consult your child's physician for a diagnosis.

Who’s at risk

What risk factors are associated with brain tumors?
  • Children with certain genetic conditions (such as neurofibromatosis, von Hippel-Lindau disease, Li-Frameni syndrome, Gorlin’s Syndrome and retinoblastoma) have an increased risk of developing tumors of the central nervous system.
  • If your child has received radiation therapy to the head as part of previous treatment for other malignancies, he may also be at an increased risk for new brain tumors.
  • Research has been looking into the relationship between past exposure to certain chemicals and having a child with a brain tumor. It is currently thought that some chemicals may change the structure of a gene that protects the body from diseases including cancer.

Types of pediatric brain tumors

What are the different types of brain tumors?

There are many different types of brain tumors. Learn more about types of pediatric brain tumors, including diagnosis and treatment options.

Questions to ask your child’s doctor

After your child is diagnosed with a brain tumor, you may feel overwhelmed with information. It can be easy to lose track of the questions that occur to you.

Lots of parents find it helpful to jot down questions as they arise – that way, when you talk to your child’s doctors, you can be sure that all of your concerns are addressed.

If your child is old enough, you may want to suggest that he write down what he wants to ask his health care provider, too.

Some of the questions you may want to ask include:

  • What type of brain tumor does my child have?
  • Where in the brain is the tumor located? How might this affect my child?
  • Has my child’s brain tumor spread?
  • Can the tumor be treated with surgery?
  • How long will my child need to be in the hospital?
  • What are the possible short and long-term complications of treatment? How will they be addressed?
  • What is the likelihood of cure?
  • What services are available to help my child and my family cope?

FAQ

Q: Will my child be OK? 

A: Today, more than half of all children diagnosed with a brain tumor will be cured of the disease. However, prognosis can vary widely based on the type of tumor, its location and whether it has spread. Your care team will talk to you in depth about your child’s unique situation.

Q: Where will my child be treated? 

A: Children treated through Dana-Farber/Boston Children's receive outpatient care at the Jimmy Fund Clinic on the third floor of Dana Farber Cancer Institute. If your child needs to be admitted to the hospital, he will stay at Boston Children's Hospital. Learn more about our treatment facilities.

Q: What services are available to help my child and my family cope? 

A: Above all, we are committed to designing a treatment plan that fits the individual needs and circumstances of your child, and to providing emotional and educational support for your entire family. We offer a variety of services to help you, your child and your family get through this difficult time.

Q: What kind of supportive or palliative care is available at Dana-Farber/Boston Children's? 

A: When necessary, our Pediatric Advanced Care Team (PACT) is available to provide supportive treatments intended to optimize the quality of life and promote healing and comfort for children with life-threatening illness. In addition, PACT can provide psychosocial support and help arrange end-of-life care when necessary.

Tests

The first step in treating your child is forming an accurate and complete diagnosis, and the chances of treatment being successful are much higher if a brain tumor is caught early. Diagnostic procedures for brain tumors are used to determine the exact type of tumor your child has and whether the tumor has spread. These may include:

  • physical exam, tests of reflexes, muscle strength, eye and mouth movement, coordination and alertness
  • magnetic resonance imaging (MRI) to produce detailed images of the brain and/or spine
  • magnetic resonance spectroscopy (MRS) which is a test done along with an MRI that can detect the presence of particular organic compounds produced by the body's metabolism within sample tissue; this helps us identify tissue as either normal or tumor, and may be able to distinguish between glial tumors and tumors of neuronal (nerve cell) origin.
  • computerized tomography scan (also called a CT or CAT scan) to capture a detailed view of the bones and fluid filled spaces of the brain.
  • biopsy or tissue sample from the tumor to provide definitive information about the type of tumor; this is collected during surgery
  • lumbar puncture (spinal tap) to remove a small sample of cerebrospinal fluid (CSF) and determine if any tumor cells have started to spread. In young children, this procedure is safely performed under sedation, and is less difficult and less painful than placing an intravenous (IV) catheter.

After we complete all necessary tests, our experts meet to review and discuss what they have learned about your child's condition. Then we will meet with you and your family to discuss the results and outline the best treatment options.

Treatment and Care

It’s entirely natural that you might be concerned, right now, about your child’s health; a diagnosis of a brain tumor can be frightening. But you can rest assured that, at Dana-Farber/Boston Children's, your child is in good hands. Our physicians are bright, compassionate, and committed to focusing on the whole child, not just his condition.

The good news is that treatment for brain tumors in children has progressed tremendously in the last decade:

  • New tools are being used to help doctors diagnose tumors sooner and with more accuracy.
  • Radiation therapy and chemotherapy are increasingly targeting tumors more accurately and effectively while keeping clear of healthy brain cells and tissue.
  • A successful new surgical technique is the intra-operative MRI, which gives surgeons a three-dimensional picture of the tumor so they can remove the cancer while leaving other parts of the brain relatively untouched.
What treatments are available for brain tumors?

Your child’s team will determine the best treatment plan based on a number of factors, including but not limited to:

  • your child's age, overall health and medical history
  • your child's tolerance for specific medications, procedures or therapies
  • type, grade, and location of tumor(s)
  • expectations for the course of the cancer
  • your opinion or preference

Surgery 

Surgery has multiple roles in the diagnosis and treatment of brain tumors, including release of pressure on the brain, biopsy and tumor removal.

If your child has a brain tumor, the first treatment is usually surgery to remove as much of the tumor as possible.

  • Tumor specimens are examined by neuropathologists to determine the exact diagnosis.
  • When possible, it’s best to completely remove the entire tumor. In general, the more of the tumor that is removed, the greater the chance for survival.
    • Most high-grade gliomas cannot be completely removed because of the infiltrating fingers of tumor which characterize their growth.
    • Tumors of the cerebral hemispheres are in general more easily removed than those of the midline, more inner-brain structures.
     
  • Using the latest molecular profiling techniques, all pediatric brain tumors are now processed to identify abnormal genes within the tumor (whether due to an inherited condition or a new mutation that occurred to start the tumor)

Radiation therapy 

Our doctors use precisely targeted and dosed radiation to kill cancer cells left behind after your child’s surgery. This is important to control the growth of the tumor. Depending on the type of the tumor, some patients are treated with targeted focal radiation therapy. In those tumors that may have spread, radiation therapy can sometimes be delivered to the entire brain and spine.

While radiotherapy can be quite effective in treating certain cancers, the radiation damages both cancerous and non-cancerous cells. Because of this, there can be many undesirable side effects during and after treatment. Being able to anticipate these side effects can help the care team, parents and child prepare, and, in some cases, prevent these symptoms from occurring.

Chemotherapy 

Chemotherapy (“chemo”) refers to drugs that interfere with the cancer cell’s ability to grow or reproduce. For some kinds of tumors, chemotherapy before surgery may help shrink the tumor, making it possible to remove.

  • Different groups of chemotherapy drugs work in different ways to fight cancer cells and shrink tumors.
  • Often, a combination of chemotherapy drugs is used.
  • Certain chemotherapy drugs may be given in a specific order depending on the type of cancer it is being used to treat.

While chemotherapy can be quite effective in treating certain cancers, the drugs treat cancerous and non-cancerous cells the same. Because of this, there can be many undesirable side effects during treatment. Being able to anticipate these side effects can help the care team, parents and child prepare, and, in some cases, prevent these symptoms from occurring.

Chemotherapy is systemic treatment, meaning it is introduced to the bloodstream and travels throughout the body to kill cancer cells. It may be given:

  • orally, as a pill to swallow
  • intramuscularly, as an injection into the muscle or fat tissue
  • intravenously, directly to the bloodstream (also called IV)
  • intrathecally, directly into the spinal fluid with a needle

Chemotherapy also refers to the use of drugs that are made to specifically inhibit a molecular pathway required to keep the tumor going. These drugs are often referred to as “targeted” therapy. Rather than the standard side effects associated with radiation and chemotherapy (loss of hair, nausea and vomiting, damage of the blood producing cells), these drugs tend to be much better tolerated.

What is the long-term outlook for a child with a brain tumor?

As with other tumors in both children and adults, surgery is the primary treatment, usually followed by radiation treatment and/or chemotherapy. Unfortunately, because your child’s brain is still developing, these treatments can result in more substantial and permanent side effects than they would for an adult.

Many children who are treated for brain tumors experience significant long-term problems, such as changes in intellectual and motor function. They require ongoing assessment and specialized care to help them function at school and throughout life as best as possible.

Your child’s prognosis greatly depends on:

  • the type of tumor
  • the extent of the disease
  • size and location of the tumor
  • presence or absence of metastasis (spreading)
  • the tumor's response to therapy
  • your child’s age and overall health
  • your child’s tolerance of specific medications, procedures or therapies
  • new developments in treatment

Today, more than half of all children diagnosed with a brain tumor will be cured of the disease. But with any cancer, prognosis and long-term survival varies greatly. Prompt medical attention and aggressive therapy are very important, as is continuous follow-up care.

Your child may also need a lot of rehabilitation for lost motor skill and muscle strength. If appropriate, he may also see speech therapists and physical and occupational therapists.

New methods are continually being discovered to improve treatment and to decrease side effects.

What kind of long-term follow-up care should my child get? 

Because of the possible long-term problems and the risk of a tumor returning, assessments and care usually continue for years after the tumor is removed.

One of the major goals of the Dana-Farber/Boston Children's Pediatric Brain Tumor Program is to maximize the long-term function of your child. This is achieved through multidisciplinary monitoring and interventions, as needed, in a wide range of areas, including:

  • intellectual function and school performance
  • endocrine evaluation and treatment
  • neurologic assessment
  • psychosocial care
  • hearing, vision
  • ovarian dysfunction in girls
  • motor function

Through the Stop and Shop Neuro-Oncology Outcomes Clinic at Dana-Farber Cancer Institute, your child will be able to meet with his neurosurgeon, radiation oncologist, pediatric neuro-oncologist and neurologists at the same follow-up visit.

  • Our pediatric brain tumor survivorship clinic is held weekly.
  • In addition to meeting with your pediatric neuro-oncologists, neurologist and neurosurgeon, your child may also see one of our endocrinologists and/or alternative/complementary therapy specialists.
  • School liaisons and psychosocial personnel from the pediatric brain tumor team are also available.
  • If your child needs rehabilitation, he may also meet with speech, physical, and occupational therapists during and after treatments

Resources and Support

We understand that you may have a lot of questions if your child is diagnosed with a brain tumor. Will it affect my child long-term? What do we do next? We’ve tried to provide some answers to those questions in these pages, but there are also a number of resources and support services to help you and your family through this difficult time.

Research and Innovation

A Hopeful Future 

Treatment for brain tumors in children has progressed tremendously in the last decade:

  • New tools are being used to help doctors diagnose tumors sooner and with more accuracy.
  • Radiation therapy and chemotherapy are increasingly targeting tumors more accurately and effectively while keeping clear of healthy brain cells and tissue.
  • A successful new surgical technique is the intra-operative MRI, which gives surgeons a three-dimensional picture of the tumor so they can remove the cancer while leaving other parts of the brain relatively untouched.

Research and clinical trials 

Our program offers unique access to a range of brain tumor clinical trials in which your child can receive the newest brain tumor treatments. Through this research, our physicians work to improve current therapeutic approaches and outcomes for many hard-to-treat pediatric brain tumors.

Dana-Farber/Boston Children's oversees New England’s most active pediatric oncology clinical research program; it provides access to unique clinical trials for patients with newly diagnosed, relapsed or refractory brain tumors. We are:

  • New England’s Phase I Children’s Oncology Group referral center, a Pediatric Oncology Experimental Therapeutics Investigator Consortium member (POETIC), a Department of Defense Neurofibromatosis Clinical Trial Consortium member and a founding institution of the Pediatric Brain Tumor Consortium. We are also members of the Pediatric Blood and Marrow Transplant Consortium (PBMTC).
  • Skilled at improving current therapeutic approaches and outcomes of hard to treat pediatric brain tumors.

Rapidly translating scientific discoveries to the bedside is a major focus of the program. Members of our brain tumor team:

  • played a key role in the identification and application of anti-angiogenic treatments for pediatric brain tumors
  • launched new studies investigating gene profiling of patients’ tumors and the development of personalized treatment approaches
  • pioneered the development of new treatments for specific highly malignant tumors such as ATRT development of new targeted therapies for diffuse intrinsic pontine gliomas
  • creation of the only pediatric low-grade astrocytoma program
  • molecular profiling of pediatric brain tumors for any patient
  • are developing tissue registries to better classify and treat certain types of brain tumors

General Information About Childhood Brain and Spinal Cord Tumors

Dramatic improvements in survival have been achieved for children and adolescents with cancer. Between 1975 and 2010, childhood cancer mortality decreased by more than 50%.[1] Childhood and adolescent cancer survivors require close follow-up because cancer therapy side effects may persist or develop months or years after treatment. Refer to the PDQ summary on Late Effects of Treatment for Childhood Cancer for specific information about the incidence, type, and monitoring of late effects in childhood and adolescent cancer survivors.

Primary brain tumors are a diverse group of diseases that together constitute the most common solid tumor of childhood. Brain tumors are classified according to histology, but tumor location and extent of spread are also important factors that affect treatment and prognosis. Immunohistochemical analysis, cytogenetic and molecular genetic findings, and measures of proliferative activity are increasingly used in tumor diagnosis and classification.

Incidence

Primary central nervous system tumors are a diverse group of diseases that together constitute the most common solid tumor in childhood. The Central Brain Tumor Registry of the United States (CBTRUS) estimates that approximately 4,300 U.S. children are diagnosed each year.[2]

References:

  1. Smith MA, Altekruse SF, Adamson PC, et al.: Declining childhood and adolescent cancer mortality. Cancer 120 (16): 2497-506, 2014.

  2. Ostrom QT, Gittleman H, Farah P, et al.: CBTRUS statistical report: Primary brain and central nervous system tumors diagnosed in the United States in 2006-2010. Neuro Oncol 15 (Suppl 2): ii1-56, 2013.

Classification of Central Nervous System Tumors

The classification of childhood central nervous system (CNS) tumors is based on histology and location.[1] Tumors are classically categorized as infratentorial, supratentorial, parasellar, or spinal. Immunohistochemical analysis, cytogenetic and molecular genetic findings, and measures of proliferative activity are increasingly used in tumor diagnosis and classification and will likely affect classification and nomenclature in the future.

Primary CNS spinal cord tumors comprise approximately 1% to 2% of all childhood CNS tumors. 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]

Infratentorial (posterior fossa) tumors include the following:

  1. Cerebellar astrocytomas (most commonly pilocytic, but also fibrillary and less frequently, high grade).
  2. Medulloblastomas (including classic, desmoplastic/nodular, extensive nodularity, anaplastic, or large cell variants).
  3. Ependymomas (cellular, papillary, clear cell, tanycytic, or anaplastic).
  4. Brain stem gliomas (typically diffuse intrinsic pontine gliomas and focal, tectal, and exophytic cervicomedullary gliomas are most frequently pilocytic astrocytomas).
  5. Atypical teratoid/rhabdoid tumors.
  6. Choroid plexus tumors (papillomas and carcinomas).
  7. Rosette-forming glioneuronal tumors of the fourth ventricle.

Supratentorial tumors include the following:

  1. Low-grade cerebral hemispheric astrocytomas (grade I [pilocytic] astrocytomas or grade II [diffuse] astrocytomas).
  2. High-grade or malignant astrocytomas (anaplastic astrocytomas and glioblastoma [grade III or grade IV]).
  3. Mixed gliomas (low- or high-grade).
  4. Oligodendrogliomas (low- or high-grade).
  5. Primitive neuroectodermal tumors (PNETs) (cerebral neuroblastomas, pineoblastomas, and ependymoblastomas).
  6. Atypical teratoid/rhabdoid tumors.
  7. Ependymomas (cellular or anaplastic).
  8. Meningiomas (grades I, II, and III).
  9. Choroid plexus tumors (papillomas and carcinomas).
  10. Tumors of the pineal region (pineocytomas, pineoblastomas, pineal parenchymal tumors of intermediate differentiation, and papillary tumors of the pineal region), and germ cell tumors.
  11. Neuronal and mixed neuronal glial tumors (gangliogliomas, desmoplastic infantile astrocytoma/gangliogliomas, dysembryoplastic neuroepithelial tumors, and papillary glioneuronal tumors).
  12. Other low-grade gliomas (including subependymal giant cell tumors and pleomorphic xanthoastrocytoma).
  13. Metastasis (rare) from extraneural malignancies.

Parasellar tumors include the following:

  1. Craniopharyngiomas.
  2. Diencephalic astrocytomas (central tumors involving the chiasm, hypothalamus, and/or thalamus) that are generally low-grade (including astrocytomas, grade I [pilocytic] or grade II [diffuse]).
  3. Germ cell tumors (germinomas or nongerminomatous).

Spinal cord tumors include the following:

  1. Low-grade cerebral hemispheric astrocytomas (grade I [pilocytic] astrocytomas or grade II [diffuse] astrocytomas).
  2. High-grade or malignant astrocytomas (anaplastic astrocytomas and glioblastoma [grade III or grade IV]).
  3. Gangliogliomas.
  4. Ependymomas (often myxopapillary).

References:

  1. Louis DN, Ohgaki H, Wiestler OD, et al., eds.: WHO Classification of Tumours of the Central Nervous System. 4th ed. Lyon, France: IARC Press, 2007.

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 tumor or spinal cord tumor include the following:

  1. The cause of most childhood brain tumors remains unknown.[1]
  2. Selection of an appropriate therapy can only occur if the correct diagnosis is made and the stage of the disease is accurately determined.
  3. 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.[2][3] For example, radiation therapy of pediatric brain tumors is technically demanding and should be performed in centers that have experience in this area.
  4. 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. Survival of childhood cancer has advanced as a result of clinical trials that have attempted to improve upon 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 then comparing the results with those previously obtained from existing therapy. Information about ongoing clinical trials is available from the NCI Web site.
  5. While more than 70% of children diagnosed with brain tumors will survive for more than 5 years after 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.[4][5][6] Debilitating effects on growth and neurologic development have frequently been observed after radiation therapy, especially in younger children. Secondary tumors have increasingly been diagnosed in long-term survivors.[7] 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.[8][9][10] Long-term management of these patients is complex and requires a multidisciplinary approach.

    (Refer to the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information about possible long-term or late effects.)

  6. 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.[11]

References:

  1. Fisher JL, Schwartzbaum JA, Wrensch M, et al.: Epidemiology of brain tumors. Neurol Clin 25 (4): 867-90, vii, 2007.

  2. Blaney SM, Haas-Kogan D, Young Poussaint T, et al.: Gliomas, ependymomas, and other nonembryonal tumors of the central nervous system. In: Pizzo PA, Poplack DG, eds.: Principles and Practice of Pediatric Oncology. 6th ed. Philadelphia, Pa: Lippincott Williams and Wilkins, 2011, pp 717-771.

  3. Pollack IF: Brain tumors in children. N Engl J Med 331 (22): 1500-7, 1994.

  4. Smith MA, Seibel NL, Altekruse SF, et al.: Outcomes for children and adolescents with cancer: challenges for the twenty-first century. J Clin Oncol 28 (15): 2625-34, 2010.

  5. Reimers TS, Mortensen EL, Nysom K, et al.: Health-related quality of life in long-term survivors of childhood brain tumors. Pediatr Blood Cancer 53 (6): 1086-91, 2009.

  6. Iuvone L, Peruzzi L, Colosimo C, et al.: Pretreatment neuropsychological deficits in children with brain tumors. Neuro Oncol 13 (5): 517-24, 2011.

  7. Armstrong GT: Long-term survivors of childhood central nervous system malignancies: the experience of the Childhood Cancer Survivor Study. Eur J Paediatr Neurol 14 (4): 298-303, 2010.

  8. 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.

  9. 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.

  10. 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.

  11. 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.

Stage Information and Treatment of Newly Diagnosed and Recurrent Childhood Brain Tumors

Presently, there is no uniformly accepted staging system for most childhood brain tumors. These tumors are classified and treated based on their histology and location within the brain (see Table below). However, with advances in molecular data, it is conceivable that genomic factors will refine classification approaches for certain groups of tumors, such as medulloblastomas [1][2] and low-grade gliomas.[3][4]

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

Tumor Type

Pathologic Subtype

Related PDQ Treatment Summary

Astrocytomas and Other Tumors of Glial Origin

Low-Grade Astrocytomas

Diffuse fibrillary astrocytoma

Childhood Astrocytomas Treatment

Gemistocytic astrocytoma

Oligoastrocytoma

Oligodendroglioma

Pilocytic astrocytoma

Pilomyxoid astrocytoma

Pleomorphic xanthoastrocytoma

Protoplasmic astrocytoma

Subependymal giant cell astrocytoma

High-Grade Astrocytomas

Anaplastic astrocytoma

Childhood Astrocytomas Treatment

Anaplastic oligoastrocytoma

Anaplastic oligodendroglioma

Giant cell glioblastoma

Glioblastoma

Gliomatosis cerebri

Gliosarcoma

Brain Stem Glioma

Diffuse intrinsic pontine glioma

Childhood Brain Stem Glioma Treatment

Focal or low-grade brain stem glioma

CNS Embryonal Tumors

Medulloblastomas

Anaplastic

Childhood CNS Embryonal Tumors Treatment

Classic

Desmoplastic/nodular

Large cell

Medulloblastoma with extensive nodularity

CNS Primitive Neuroectodermal Tumors (PNETs)

CNS ganglioneuroblastoma

CNS neuroblastoma

Ependymoblastoma

Medulloepithelioma

Pineal Parenchymal Tumors

Pineoblastoma

CNS Atypical Teratoid/Rhabdoid Tumor

Childhood CNS Atypical Teratoid/Rhabdoid Tumor Treatment

CNS Germ Cell Tumors

Germinomas

Childhood CNS Germ Cell Tumors Treatment

Teratomas

Immature teratoma

Mature teratoma

Teratoma with malignant transformation

Non-Germinomatous Germ Cell Tumors

Choriocarcinoma

Embryonal carcinoma

Mixed germ cell tumor

Yolk sac tumor

Craniopharyngioma

Childhood Craniopharyngioma Treatment

Ependymoma

Subependymoma (WHO Grade I)

Childhood Ependymoma Treatment

Myxopapillary ependymoma (WHO Grade I)

Ependymoma (WHO Grade II)

Anaplastic ependymoma (WHO Grade III)

Tumors of the Choroid Plexus

CNS = central nervous system; WHO = World Health Organization.

Recurrence is not uncommon in both low-grade and malignant childhood brain tumors and may occur many years after initial treatment. 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 determination of 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.

Early-phase therapeutic trials may be available for selected patients via Children's Oncology Group phase I institutions, the Pediatric Brain Tumor Consortium, or other entities.

References:

  1. Northcott PA, Shih DJ, Peacock J, et al.: Subgroup-specific structural variation across 1,000 medulloblastoma genomes. Nature 488 (7409): 49-56, 2012.

  2. Kool M, Korshunov A, Remke M, et al.: Molecular subgroups of medulloblastoma: an international meta-analysis of transcriptome, genetic aberrations, and clinical data of WNT, SHH, Group 3, and Group 4 medulloblastomas. Acta Neuropathol 123 (4): 473-84, 2012.

  3. Jones DT, Kocialkowski S, Liu L, et al.: Tandem duplication producing a novel oncogenic BRAF fusion gene defines the majority of pilocytic astrocytomas. Cancer Res 68 (21): 8673-7, 2008.

  4. Pfister S, Janzarik WG, Remke M, et al.: BRAF gene duplication constitutes a mechanism of MAPK pathway activation in low-grade astrocytomas. J Clin Invest 118 (5): 1739-49, 2008.

Stage Information and Treatment of Newly Diagnosed and Recurrent Childhood Spinal Cord Tumors

There is no uniformly accepted staging system for childhood primary spinal cord tumors. These tumors are classified and treated based on their location within the spinal cord and histology. Refer to the following PDQ summaries for more information on the staging and treatment of newly diagnosed and recurrent childhood spinal cord tumors:

  • Childhood Astrocytomas Treatment.
  • Childhood Central Nervous System Embryonal Tumors Treatment.
  • Childhood Ependymoma Treatment.

This information is provided by the National Cancer Institute.

This information was last updated on August 12, 2014.

  • Email
  • Print
  • Share
  • Text
Highlight Glossary Terms
  • Make an Appointment

    • For adults:
      877-442-3324 (877-442-DFCI)
    • For children:
      888-733-4662 (888-PEDI-ONC)
    • Or complete the online form.
  • Find a Clinical Trial

  • Ranked #1

    • U.S. News and World Report Best Children's Hospital Cancer logo Dana-Farber/
      Boston Children's is ranked the #1 pediatric cancer hospital by the U.S. News & World Report 2014-15 Best Children's Hospitals guide.