Glioma, Diffuse Pontine

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    A diffuse pontine glioma or brain stem glioma is a malignant tumor that arises in the supportive tissue of the part of the brain that controls many vital functions. Learn about diffuse pontine glioma and find information on how we support and care for children and teens with brain stem glioma 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

Diffuse Pontine Glioma    

Overview

Having a tumor in the brain is always a very serious matter, and a diffuse pontine glioma – also called a brainstem glioma – is no exception. Diffuse pontine glioma is a highly aggressive and difficult to treat brain tumor and is found at the base of the brain in the pons, which controls vital body functions, such as breathing.

  • Diffuse pontine glioma is a glial tumor, meaning that it arises in the glial (supportive) tissue of the lowest, stem-like part of the brain, which controls many of the body’s most vital functions.
  • Diffuse pontine gliomas account for 10 to 15 percent of all childhood central nervous system tumors.
  • The median age at diagnosis is 5 to 9 years old but they can occur at any age in childhood.
  • These tumors occur with equal frequency in boys and girls and do not generally appear in adults.

As you read on, you’ll find detailed information about diffuse pontine gliomas.

How Dana-Farber/Boston Children's Cancer and Blood Disorders Center approaches diffuse pontine glioma

Children with diffuse pontine glioma are treated through the Glioma Program at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center, an integrated pediatric hematology and oncology partnership between Dana-Farber Cancer Institute and Boston Children’s Hospital. Working together, we provide more specialists, more programs, and more clinical trials than almost any other pediatric center treating cancer and blood disorders in the world.

In-depth

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

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

What causes diffuse pontine glioma?

As a parent, you undoubtedly want to know what may have caused your child’s tumor. However, the cause of diffuse pontine glioma is not currently understood.

There are no known factors or conditions that make your child more or less likely to develop this type of tumor. It might be reassuring to know that there’s nothing that you could have done or avoided doing that would have prevented the tumor from developing.

What are the symptoms of a diffuse pontine glioma?

Symptoms usually develop very rapidly prior to diagnosis, reflecting the fast growth of these tumors. Most patients have less than three months and many less than three weeks of symptoms prior to diagnosis. While each child may experience symptoms of a diffuse pontine glioma differently, some of the most common include:

  • rapidly developing problems controlling eye movements, facial expressions, speech, chewing and swallowing (due to problems in the cranial nerves)
  • weakness in the arms and legs
  • problems with walking and coordination

Keep in mind 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.

How are diffuse pontine gliomas classified?

The World Health Organization classification scheme includes 4 grades of glioma, according to how the cells look under a microscope.grade I (benign)

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

Since diffuse pontine gliomas are not generally biopsied, it can be difficult to assign a grade to one of these tumors. When biopsied, they are usually grade III or grade IV; occasionally they are grade II.

That being said, diffuse pontine gliomas usually progress like grade IV glioblastoma multiforme tumors. These are the most aggressive kind of astrocytic tumor, and they usually have the following characteristics:

  • an increased number of cells
  • abnormal cells and nuclei
  • the cells reproduce rapidly
  • the cells die quickly
  • increased growth of blood vessels

These tumors are aggressive, and will invade normal brain tissue.

Frequently Asked Questions

Q: What is the expected outcome after treatment?

A: Unfortunately, the prognosis for diffuse pontine glioma tumors remains poor. Experimental chemotherapy delivered concurrent to radiation therapy is actively being investigated in the treatment of diffuse pontine gliomas.

Q: Where will my child be treated?

A: Children treated through Dana-Farber/Boston Children's Cancer and Blood Disorders Center 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, she 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 many 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?

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.

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:  

  • Has my child’s brain tumor spread?
  • 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?

Tests

The first step in treating your child is forming an accurate and complete diagnosis. Diffuse pontine glioma is most commonly diagnosed from radiologic studies such as a CT scan or more commonly MRI. The location of these tumors and their tendency to invade into normal tissue make biopsies complicated, although a biopsy may be performed if your child’s symptoms and other tests do not seem typical for the condition. Certain clinical trials require a biopsy as part of the therapy for diffuse pontine glioma.

Your child’s doctor will perform a complete medical and physical examination. In addition, your child’s physician may order some of the following tests: 

  • 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 (often called slices), both horizontally and vertically, of the body. CT scans are more detailed than general x-rays.
     
  • magnetic resonance imaging (MRI) - a diagnostic procedure that uses a combination of large magnets, radiofrequencies, and a computer to produce detailed images of organs and structures within the body. MRI provides greater anatomical detail than CT scan and does a better job of distinguishing between tumors, tumor-related swelling and normal tissue.
     
  • magnetic resonance spectroscopy (MRS) - a test done along with an MRI. It can detect the presence of organic compounds within sample tissue that can identify the tissue as normal or tumor, and may also be able to tell if the tumor is a glial tumor or if it is of neuronal origin (originating in a neuron, instead of an astrocytic cell).

Surgical biopsies are not routinely performed in cases of diffuse pontine glioma because of the location of the tumor. Diffuse pontine gliomas occur in the brainstem, which controls the body’s vital functions.

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

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 diffuse pontine 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.

Learn more about our pediatric Glioma Program.

What are the treatments for a diffuse pontine glioma?

If your child has been diagnosed with a diffuse pontine glioma, treatment may include:

  • Radiation therapy – This is the primary therapy for newly diagnosed diffuse pontine glioma. It uses high-energy rays (radiation) from a specialized machine to damage or kill cancer cells and shrink tumors.Conventional limited-field radiation produces responses in over 90 percent of children with diffuse pontine gliomas. These responses are short-lived however, with a median duration of about 6 months. Several trials to increase the dose of radiation therapy have been performed and none has improved survival.
  • Experimental chemotherapy – Chemotherapy and biologic therapy in combination with radiation therapy is actively being investigated as a treatment of this condition. Several trials evaluating new agents are either underway or have been recently completed.

In addition, there are trials evaluating whether new ways of delivering the traditional drugs might improve responses. We should know more about the results of these tests soon. Unfortunately, no currently available chemotherapy regimen has been shown to increase survival rates in this condition.

Unfortunately, complete surgical resection is not an option in the treatment of these tumors because of where the tumor is located. Diffuse pontine gliomas occur in the brainstem, which controls the body’s most vital functions. Surgery in this part of the brain can cause severe neurological damage.

How are side effects managed?

Side effects in the treatment of diffuse pontine glioma can arise from radiation and chemotherapy. 

  • Procedures should be performed in specialized centers where experienced specialists work in the most technologically advanced settings.
  • 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.

Many specialized brain tumor treatment centers have now 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.

In the event that end-of-life care is necessary, our Pediatric Advanced Care Team is available to ease symptoms and help your child maintain quality-of-life as much as possible.

What is the expected outcome after treatment for diffuse pontine glioma?

Unfortunately, the prognosis for diffuse pontine gliomas remains very poor although a small percentage of patients can survive this disease. Your child’s physician will discuss treatment options with you, including experimental clinical trials, and supportive care.

What about progressive or recurrent disease?

Clinical trials and experimental therapies are available for patients with relapsed diffuse pontine glioma. Current trials include novel medications as well as new methods for the delivery of more traditional agents.

Resources and support

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

Research and innovation

Clinical and basic scientists at Dana-Farber/Boston Children's are conducting numerous research studies to help clinicians better understand and treat diffuse pontine gliomas.

What is the latest research on diffuse pontine gliomas and malignant gliomas?
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 (COG) and the Department of Defense (DOD) Neurofibromatosis Clinical Trial Consortium.

Through these groups, a number of novel therapies are available for children with both newly diagnosed and current brain tumors. Two new protocols include a phase II trial of radiation therapy, cetuximab and irinotecan for children with newly diagnosed malignant glioma and diffuse intrinsic pontine glioma.

A second trial for newly diagnosed malignant gliomas is a Phase I gene therapy immunotherapy trial combined with radiation therapy and temozolomide. This combination is toxic to malignant glioma cells and thus stops their growth. More importantly, it can induce an immune response to malignant cells located outside of the tumor's primary site, thus targeting the infiltrative boundary of the tumor that typically results in recurrence.

We are also leading an international phase II clinical trial on the genetics of diffuse pontine glioma. Using advanced surgical techniques, a surgical biopsy of the tumor is performed on participating patients. Samples are then analyzed at the Broad Institute of Harvard and MIT in order to understand the unique molecular characteristics of each tumor. This research will allow us to tailor treatment to each individual patient and hopefully improve outcomes for children with diffuse pontine glioma.

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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