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 conditions we treat.
How Dana-Farber/Boston Children's Cancer and Blood Disorders Center Approaches Pediatric Brain Tumor
Your child will be seen through Dana-Farber/Boston Children's Cancer and Blood Disorders Center, an integrated pediatric oncology program through Dana-Farber Cancer Institute and Boston Children's Hospital that provides — in one specialized program — all the services of both a leading cancer center and a pediatric hospital.
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 and 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?
The different types of brain tumors include those listed below. For more information on each, including diagnosis and treatment, click on the type of tumor.
- astrocytoma
- anaplastic astrocytoma
- cerebellar pilocytic astrocytoma
- cervicomedullary astrocytoma
- choroid plexus tumors
- craniopharyngioma
- diffuse pontine glioma
- dysembryoplastic neuroepithelial tumor (DNT)
- ependymoma
- ganglioglioma
- germ cell tumors of the brain
- germinoma
- glioblastoma multiforme
- gliomas
- gliomatosis cerebri
- high-grade gliomas
- low-grade gliomas
- medulloblastoma
- meningioma
- non-germinomatous germ cell tumor
- oligodendroglioma
- optic pathway glioma
- pleomorphic xanthoastrocytoma
- primitive neuroectodermal tumors (PNET)
- atypical teratoid rhabdoid tumor (ATRT)
- tectal gliomas
- thalamic tumors
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 will 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 on the ninth floor of the Berthiaume Building.
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 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
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.