Glioma, Low Grade

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    A low-grade glioma is a slow-growing cancer of the brain that begins in glial cells, which surround and support nerve cells. Learn about low-grade gliomas and find information on how we support and care for children and teens with low-grade gliomas 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

Low-Grade Gliomas

Overview

Having a tumor in the brain is always a very serious matter, and a low-grade glioma is no exception. Low-grade gliomas are a class of slow-growing, less aggressive tumors that can occur in a number of places in the brain and spinal cord. In general, low-grade gliomas have a more positive prognosis than malignant, high-grade types of brain cancer.

  • There are more 1,000 cases of pediatric low-grade gliomas each year in the United States.
  • Low-grade gliomas are the most common types of pediatric brain tumors.
  • They most commonly arise from a specific type of cell known as a glial cell, or astrocyte.
  • Astrocytes make up the supportive network of the brain, and get their name from their star-like shape.
  • Astrocytomas (tumors that begin in astrocytes) are the most common central nervous system (brain and spinal cord) tumor found in children.

As you read on, you’ll find detailed information about low-grade gliomas.

How Dana-Farber/Boston Children's Cancer and Blood Disorders Center approaches low-grade gliomas

Patients with low-grade gliomas are treated at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center through our Glioma Program, the world’s largest pediatric glioma treatment program.

Dana-Farber/Boston Children's is also home to the world’s largest pediatric low-grade glioma research program, the Pediatric Low-Grade Astrocytoma (PLGA) Program. Through the PLGA Program, we conduct advanced research on the genetic and molecular causes of low-grade glioma.

After treatment, your child will receive expert follow-up brain tumor survivorship care through the Stop & Shop Family Pediatric Neuro-Oncology Outcomes Clinic, where he 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 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.

In-depth

We understand how overwhelming a diagnosis of a glioma can be. Right now, you probably have a lot of questions. How dangerous is this condition? What is the very best treatment? What do we do next?

We’ve tried to provide some answers to those questions here, and our experts can explain your child’s condition fully when you meet with us.

What is a low-grade glioma?

Low-grade gliomas are a class of slow-growing, less aggressive tumors of the central nervous system. They most commonly arise from a specific type of cell known as a glial cell, or astrocyte.

How are low-grade gliomas classified?

An important part of diagnosing a brain tumor involves staging and classifying the disease, which will help your child’s doctor determine treatment options and prognosis. Staging is the process of determining whether the tumor has spread and, if so, how far.

There are four major types of astrocytomas, classified based on what they look like under a microscope.

  • grade I (pilocytic astrocytoma - benign)
  • grade II (fibrillary astrocytoma)
  • grade III (anaplastic astrocytoma – refers to lack of structure in the cell)
  • grade IV (glioblastoma multiforme – the most serious kind of tumor)

Low-grade gliomas are usually either grade I or grade II.

Low-grade gliomas may also be classified according to their location in the brain. This includes:

  • cerebellar pilocytic astrocytoma (the part of the brain known as the cerebellum) cervico-medullary astrocytoma (the brainstem)
  • optic pathway glioma (the optic nerve)
  • tectal glioma (the “roof” of the brainstem) thalamic/hypothalamic astrocytoma (the parts of the brain known as the thalamus or hypothalamus)

Other types of low-grade gliomas include:

  • oligodendroglioma (very rare—account for only 2 percent of all pediatric brain tumors)
  • ganglioglioma (tumors have properties of both glial cells and neuronal cells)
  • pleomorphic xanthoastrocytoma (associated with a higher seizure rate than other low-grade gliomas)

The other two types of astrocytomas are classified as high-grade gliomas:

  • grade III or anaplastic astrocytoma
  • grade IV or glioblastoma multiforme
What causes low-grade gliomas?

As a parent, you undoubtedly want to know what may have caused your child’s tumor. It’s important to understand that low-grade gliomas most often occur with no known cause. There’s nothing that you could have done or avoided doing that would have prevented the tumor from developing.

Research has shown that there is a link between some types of low-grade gliomas and certain genetic diseases, specifically:

  • neurofibromatosis
  • tuberous sclerosis
What are the symptoms of low-grade gliomas?

The symptoms of low-grade gliomas can vary greatly depending on the size and location of the tumor and whether it has infiltrated into other areas of the brain or spine.

Due to the relative slow growth rate of low-grade gliomas, your child’s symptoms may have begun many months or years before they see the doctor. While each child may experience symptoms differently, some of the most common include:

  • change in / loss of vision due to low-grade gliomas of the visual pathway
  • weight gain or loss and/or premature puberty due to low-grade gliomas in the hormone center of the brain
  • problems with movement or bowel/bladder control due to low-grade gliomas in the spine
  • vomiting, headache, fatigue and motor control problems due to fluid build-up in and increased pressure on the brain
  • seizures, due to irritation of the normal brain cells

Keep in mind that these symptoms may resemble other, more common conditions or medical problems. If you don’t have a diagnosis and are concerned, always consult your child's physician.

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 she write down what she wants to ask her 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: What causes low-grade gliomas?

A: As a parent, you undoubtedly want to know what may have caused your child’s tumor. The vast majority of children with low-grade gliomas develop these tumors spontaneously, meaning that there is no identifiable cause. A small percentage can be associated with certain genetic syndromes.  Your doctor can easily determine if a genetic cause is associated with the development of your child’s tumor.

Q: Will my child be OK after treatment for low-grade glioma?

A: The outcome after treatment for low-grade glioma can vary significantly depending on the location of the tumor, whether or not the tumor has spread and whether the tumor can be completely removed through surgery. In general, low-grade gliomas have a more positive prognosis than malignant, high-grade types of brain cancer. Your doctor will discuss treatment options with you and your family including clinical trials and supportive care.

Q: Where will my child be treated?

A: Children treated on an outpatient basis through Dana-Farber/Boston Children's are cared for 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: 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 for my child?

When necessary, our Pediatric Advanced Care Team (PACT) offers 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, so your child’s physician may order a number of different tests to determine the type and location of the tumor. Diagnostic procedures for a brain tumor are used to determine the exact type of tumor and whether the tumor has spread. In addition to a physical exam, a medical history and neurological exam (a test of reflexes, muscle strength, eye and mouth movement, coordination and alertness), your child may require tests such as:

  • computerized tomography scan (also called a CT or CAT scan) - a diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images of the body. CT scans are more detailed than general x-rays. If a low-grade glioma is suspected, your child may have a CT scan of the brain.
     
  • magnetic resonance imaging (MRI) - a diagnostic procedure that uses a combination of large magnets, radiofrequencies and a computer to produce detailed images of the brain and spine. For low-grade gliomas, an MRI of the brain is usually done. In the rare event that your child’s low-grade gliomas spread to the spine, an MRI of the spine may also be ordered.
     
  • biopsy - in many cases, a tissue sample from the tumor will be taken through a needle during a simple surgical procedure to confirm the diagnosis. However, with low-grade gliomas of the optic pathway and brain stem, surgery (including biopsy) is generally avoided, due to the very delicate structures in these areas.

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 & care

We know how difficult a diagnosis of a pediatric brain tumor can be, both for your child and for your whole family. That’s why our physicians are focused on family-centered care: From your first visit, you’ll work with a team of professionals who are committed to supporting all of your family’s physical and psychosocial needs. We’ll work with you to create a care plan that’s best for your child.

If your child has been diagnosed with a low-grade glioma, you’ll naturally be eager to know how your child’s physician will treat the tumor. Your child’s physician will determine a specific course of treatment based on several factors, including:

  • your child's age, overall health and medical history
  • type, location, and size of the tumor
  • extent of the disease
  • your child's tolerance for specific medications, procedures or therapies
  • how your child's doctors expects the disease to progress

There are a number of treatments we may recommend. Some of them help to treat the tumor while others are intended to address complications of the disease or side effects of the treatment.

What treatments are available for low-grade gliomas?

If your child has been diagnosed with a low-grade glioma, he may receive one or more of the following treatments:

  • neurosurgery - usually the first step in the treatment of brain tumors. The goal is to remove as much of the tumor as possible without compromising neurological function.
  • radiation therapy - using high-energy rays (radiation) from a specialized machine to damage or kill cancer cells and shrink tumors. Due to the long-term damage that radiation can cause to the developing brain of a child, this treatment is usually only used as a last resort.
  • chemotherapy - a drug treatment that works by interfering with the cancer cell's ability to grow or reproduce. Modern treatments now include biologic (also called smart drugs) that target specific abnormal pathways required by the tumor to grow and spread. A number of these types of drugs are now in clinical trials in children with low-grade gliomas.
What is expected post-treatment for low-grade glioma?

The prognosis for a child with a low-grade glioma depends on tumor grade, location and in some cases, age of the child at diagnosis.

  • Many low-grade gliomas are first treated with surgery and then monitored for regrowth. Grade I astrocytomas, for example, are usually cured with complete surgical removal alone.
     
  • If, due to the tumor’s location, complete surgical resection is not an option (optic pathway or brain stem gliomas, thalamic/hypothalamic or cervico-medullary gliomas) or if a tumor begins to grow back after it has been removed, the doctor may recommend chemotherapy.
     
  • We usually don’t use radiation therapy unless your child’s tumor has grown after chemotherapy. Due to the potential side effects of radiation, including effects on learning and hormone function, it is best avoided if your child is young (especially under age 10).
What is chemotherapy?

Chemotherapy is systemic treatment, meaning it is introduced to the bloodstream and travels throughout the body to kill cancer cells. Different groups of chemotherapy drugs work in different ways to fight cancer cells and shrink tumors.

How is chemotherapy given?

Your child may receive chemotherapy:

  • orally, as a pill to swallow
  • intramuscularly, as an injection into the muscle or fat tissue
  • intravenously, directly to the bloodstream (intravenously or IV)
  • intrathecally, directly into the spinal fluid with a needle (intrathecally)
Does chemotherapy cause side effects?

While chemotherapy can be quite effective in treating certain cancers, the agents do not differentiate normal healthy cells from cancer cells. Because of this, your child may experience adverse side effects during treatment. Being able to anticipate these side effects can help you, your child and your care team prepare for, and, in some cases, prevent these symptoms from occurring, if possible.

How are side effects managed?

Side effects in the treatment of low-grade gliomas can arise from surgery, radiation and chemotherapy.

  • Procedures should be performed in specialized centers where experienced neurosurgeons, working in the most technologically advanced settings, can provide the most extensive resections while preserving normal brain tissue.
     
  • Radiation therapy often produces inflammation, which can temporarily exacerbate symptoms and dysfunction. To control this, inflammation steroids are sometimes necessary.

     
  • Some of the chemotherapy agents are associated with fatigue, diarrhea, constipation and headache. These side effects can be effectively managed under most circumstances with standard medical approaches.

Our Pediatric Brain Tumor Program also has access to specialists who deliver complementary or alternative medicines. These treatments, which may help control pain and side effects of therapy include the following.

  • acupuncture/acupressure
  • therapeutic touch
  • massage
  • herbs
  • dietary recommendations

Talk to your child’s physician about whether complementary or alternative medicine might be a viable option.

What about progressive or recurrent disease?

There are numerous standard and experimental treatment options for children with progressive or recurrent low-grade gliomas.

Dana-Farber Cancer Institute is one of nine institutes in the nation belonging to the Pediatric Oncology Experimental Therapeutic Investigators Consortium. The consortium is dedicated to the development of new and innovative treatments for children with newly diagnosed as well as progressive or recurrent brain tumors. We are also home to the world’s largest pediatric low-grade astrocytoma research program and the Department of Defense Neurofibromatosis Clinical Trial Consortium.

Long-term follow-up

Today, the majority of children and adolescents diagnosed with pediatric brain tumors will survive into adulthood. However, many of them will face physical, psychological, social and intellectual challenges related to their treatment and will require ongoing assessment and specialized care.

To address the needs of this growing community of brain tumor survivors, Dana-Farber/Boston Children's established the Stop & Shop Family Pediatric Neuro-Oncology Outcomes Clinic.

Today, more than 1,000 pediatric brain tumor survivors of all ages are followed by the Outcomes Clinic, a multi-disciplinary program designed to address long-term health and social issues for families and survivors of childhood brain tumors. Some of the post-treatment services provided by the Outcomes Clinic include:

  • MRI scans to monitor for tumor recurrences
  • intellectual function evaluation
  • endocrine evaluation and treatment
  • neurologic assessment
  • psychosocial care
  • hearing, vision monitoring
  • ovarian dysfunction evaluation and treatment
  • motor function evaluation and physical therapy
  • complementary medicine

As a result of treatment, children may experience changes in intellectual and motor function. Among several programs addressing these needs are the School Liaison and Back to School Programs, which provide individualized services to ease children's return to school and maximize their ability to learn. In addition to providing thorough and compassionate care, our Outcomes Clinic specialists conduct innovative survivorship research and provide continuing education for staff, patients and families

Research and Innovations

Dana-Farber/Boston Children's is a member of the Pediatric Oncology Therapeutic Experimental Investigators Consortium (POETIC), a collaborative clinical research group offering experimental therapies to patients with relapsed or refractory disease. It is also the New England Phase I Center of the Children's Oncology Group and a member of the Department of Defense Neurofibromatosis Clinical Trial Consortium.

We are home to the only dedicated pediatric low-grade glioma program, the Pediatric Low-Grade Astrocytoma (PLGA) Program. In addition to the discovery of a number of novel targets, this program has initiated a number of phase II protocols using molecular inhibitors for children with progressive/recurrent low-grade glioma.

Through the PLGA Research Program, we have pioneered strategies for analyzing the genetic and molecular characteristics of pediatric low-grade astrocytomas. In collaboration with the Broad Institute of Harvard and MIT, we have made strides towards a better understanding of these conditions. Through the program, we’ve also established a patient registry and multiple international research projects.

Clinical trials 

For many children with rare or hard-to-treat conditions, clinical trials provide new options.

 


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 2002, 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. Between 2,500 and 3,500 children are diagnosed in the United States each year.

References:

  1. 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.

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.[7][8][9] Secondary tumors have increasingly been diagnosed in long-term survivors.[10] 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.[11][12][13] 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.[14]

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. 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.

  8. 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.

  9. 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.

  10. Jenkin D: Long-term survival of children with brain tumors. Oncology (Huntingt) 10 (5): 715-9; discussion 720, 722, 728, 1996.

  11. 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.

  12. 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.

  13. 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.

  14. 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 January 28, 2014.

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