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%.  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 important factors that affect treatment and prognosis. Immunohistochemical analysis, cytogenetic and molecular genetic findings, and measures of mitotic activity are increasingly used in tumor diagnosis and classification.
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.
The classification of childhood central nervous system (CNS) tumors is based on histology and location.  Tumors are classically categorized as infratentorial, supratentorial, parasellar, or spinal. 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.
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.   
Infratentorial (posterior fossa) tumors include the following:
Supratentorial tumors include the following:
Parasellar tumors include the following:
Spinal cord tumors include the following:
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:
For more information about possible long-term or late effects, refer to the PDQ summary on Late Effects of Treatment for Childhood Cancer.
There is no uniformly accepted staging system for childhood brain tumors. These tumors are classified and treated based on their histology and location within the brain (Table 1).
|Tumor Type||Pathologic Subtype||Staging and Treatment of Newly Diagnosed and Recurrent Disease|
|Astrocytomas and Other Tumors of Glial Origin|
|– Low-Grade Astrocytomas||Diffuse fibrillary astrocytoma||Childhood Astrocytomas Treatment|
|Subependymal giant cell astrocytoma|
|– High-Grade Astrocytomas||Anaplastic astrocytoma||Childhood Astrocytomas Treatment|
|Giant cell glioblastoma|
|Brain Stem Glioma|
|Diffuse intrinsic pontine gliomas||Childhood Brain Stem Glioma Treatment|
|Focal or low-grade brain stem gliomas|
|CNS Embryonal Tumors|
|– Medulloblastoma||Anaplastic||Childhood CNS Embryonal Tumors Treatment|
|Medulloblastoma with extensive nodularity|
|– CNS Primitive Neuroectodermal Tumors (PNETs)||CNS ganglioneuroblastoma|
|– Tumors of the Pineal Region||Pineal parenchymal tumor of intermediate differentiation|
|Papillary tumor of the pineal region|
|– 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 teratomas|
|Teratomas with malignant transformation|
|– Non-Germinomatous Germ Cell Tumors||Choriocarcinoma|
|Mixed germ cell tumors|
|Yolk sac tumor|
|Craniopharyngioma||Childhood Craniopharyngioma Treatment|
|Ependymoma||Childhood Ependymoma Treatment|
|Tumors of the Choroid Plexus|
|CNS = central nervous system.|
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 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.
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 one of the following PDQ summaries for more information on the staging and treatment of newly diagnosed and recurrent childhood spinal cord tumors:
In general, 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 that cannot be re-resected can be treated on protocols designed for histologically similar brain tumors.
The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.
General Approach to Care for Children with Brain and Spinal Cord Tumors
Added Iuvone et al. as reference 6.
This summary is written and maintained by the PDQ Pediatric Treatment Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ NCI's Comprehensive Cancer Database pages.
This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of childhood brain and spinal cord tumors. It is intended as a resource to inform and assist clinicians who care for cancer patients. It does not provide formal guidelines or recommendations for making health care decisions.
This summary is reviewed regularly and updated as necessary by the PDQ Pediatric Treatment Editorial Board, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).
Board members review recently published articles each month to determine whether an article should:
Changes to the summaries are made through a consensus process in which Board members evaluate the strength of the evidence in the published articles and determine how the article should be included in the summary.
The lead reviewers for Childhood Brain and Spinal Cord Tumors Treatment Overview are:
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National Cancer Institute: PDQ® Childhood Brain and Spinal Cord Tumors Treatment Overview. Bethesda, MD: National Cancer Institute. Date last modified <MM/DD/YYYY>. Available at: http://www.cancer.gov/cancertopics/pdq/treatment/childbrain/healthprofessional. Accessed <MM/DD/YYYY>.
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Date last modified: 2012-05-10
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