Dramatic improvements in survival have been achieved for children and adolescents with cancer. Between 1975 and 2010, childhood cancer mortality decreased by more than 50%.  Childhood and adolescent cancer survivors require close monitoring 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 by 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. 
The Central Brain Tumor Registry of the United States (CBTRUS) estimates that approximately 4,300 U.S. children are diagnosed 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 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. 
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:
(Refer to the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information about possible long-term or late effects.)
Presently, there is no uniformly accepted staging system for most childhood brain tumors. These tumors are classified and treated on the basis of their histology and location within the brain (refer to the Table below). With advances in molecular data, it is conceivable that genomic factors will refine classification approaches and will be used progressively more to stratify patients entered on clinical trials.
|Tumor Type||Pathologic Subtype||Related PDQ Treatment Summary|
|Astrocytomas and Other Tumors of Glial Origin|
|– Low-Grade Astrocytomas||Diffuse astrocytoma, IDH-mutant, IDH-wild type, or NOS||Childhood Astrocytomas Treatment|
|Subependymal giant cell astrocytoma|
|– High-Grade Astrocytomas||Anaplastic astrocytoma, IDH-mutant or IDH-wild type||Childhood Astrocytomas Treatment|
|Anaplastic pleomorphic xanthoastrocytoma|
|Diffuse midline glioma, H3 K27M-mutant|
|– Other Astrocytomas or Gliomas||Angiocentric glioma||Childhood Astrocytomas Treatment|
|Choroid glioma of the third ventricle|
|Brain Stem Glioma|
|Diffuse intrinsic pontine glioma, H3 K27M-mutant||Childhood Brain Stem Glioma Treatment|
|Focal or low-grade brain stem glioma|
|CNS Embryonal Tumors|
|– Medulloblastomas||Medulloblastoma, WNT-activated||Childhood CNS Embryonal Tumors Treatment|
|Medulloblastoma, SHH-activated and TP53-mutant|
|Medulloblastoma, SHH-activated and TP53-wildtype|
|– Nonmedulloblastomas||CNS ganglioneuroblastoma|
|Embryonal tumor with multilayered rosettes (ETMR), C19MC-altered or NOS|
|– CNS Atypical Teratoid/Rhabdoid Tumor||Childhood CNS Atypical Teratoid/Rhabdoid Tumor Treatment|
|Pineal Parenchymal Tumors||Pineoblastoma||Childhood CNS Embryonal Tumors Treatment|
|CNS Germ Cell Tumors|
|– Germinomas||Childhood CNS Germ Cell Tumors Treatment|
|– Teratomas||Immature teratoma|
|Teratoma with malignant transformation|
|– Non-Germinomatous Germ Cell Tumors||Choriocarcinoma|
|Mixed germ cell tumor|
|Yolk sac tumor|
|Craniopharyngioma||Childhood Craniopharyngioma Treatment|
|Subependymoma (WHO grade I)||Childhood Ependymoma Treatment|
|Myxopapillary ependymoma (WHO grade I)|
|Ependymoma (WHO grade II)|
|Ependymoma, RELA fusion–positive (WHO grade II or grade III)|
|Anaplastic ependymoma (WHO grade III)|
|Tumors of the Choroid Plexus|
|CNS = central nervous system; NOS = not otherwise specified; 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 and/or tumor transformation over time, including change in grade and molecular makeup.   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 on the basis of the initial tumor type, the length of time between initial treatment and the reappearance of the lesion, and other clinical parameters.
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, tumor extent at diagnosis, 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:
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.
This summary was comprehensively reviewed.
General Information About Childhood Brain and Spinal Cord Tumors
Added Louis et al. as reference 2.
Classification of Central Nervous System Tumors
This section was extensively revised.
General Approach to Care for Children with Brain and Spinal Cord Tumors
Revised text to state that the cause of most childhood brain tumors remains unknown; however, germline mutations are becoming increasingly recognized as cancer-predisposing, as they are identified in up to 8% of children with cancer (cited Zhang et al. as reference 2).
Treatment of Newly Diagnosed and Recurrent Childhood Brain Tumors
Revised text to state that with advances in molecular data, it is conceivable that genomic factors will refine classification approaches and will be used progressively more to stratify patients entered on clinical trials.
Revised the Table to reflect the recent changes to the World Health Organization classification of central nervous system tumors.
Revised text to state that biopsy or surgical re-resection may be necessary for confirmation of relapse and/or tumor transformation over time, including change in grade and molecular makeup (cited Morrissy et al. and Mistry et al. as references 1 and 2, respectively). Also added Packer et al. as reference 3.
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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The preferred citation for this PDQ summary is:
PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Brain and Spinal Cord Tumors Treatment Overview. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/brain/hp/child-brain-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389453]
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Date last modified: 2017-08-02
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