Primary brain tumors are a diverse group of diseases that together constitute the most common solid tumor of childhood. The Central Brain Tumor Registry of the United States (CBTRUS) estimates that approximately 4,300 U.S. children are diagnosed each year.
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.
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.
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.
The classification of childhood central nervous system (CNS) tumors is based on histology, location, and extent of spread (refer to the Table below). Immunohistochemical analysis, cytogenetic and molecular genetic findings, and measures of proliferative activity are increasingly used in tumor diagnosis and classification. With advances in molecular data, it is conceivable that genomic factors will refine classification approaches and will be increasingly used to stratify patients entered on clinical trials.
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.
|Tumor Type (Based on the 2016 WHO Classification)||Pathologic Subtype (Based on the 2016 WHO Classification)||Related PDQ Treatment Summary|
|CNS = central nervous system; WHO = World Health Organization.|
|Diffuse astrocytic tumors||Diffuse astrocytoma,IDH-mutant andIDH-wildtype||Childhood Astrocytomas Treatment|
|Anaplastic astrocytoma,IDH-mutant andIDH-wildtype|
|Diffuse midline glioma, H3K27M-mutant||Childhood Astrocytomas Treatment|
|Childhood Brain Stem Glioma Treatment|
|Other astrocytic tumors||Pilocytic astrocytoma||Childhood Astrocytomas Treatment|
|Childhood Brain Stem Glioma Treatment|
|Subependymal giant cell astrocytoma||Childhood Astrocytomas Treatment|
|Anaplastic pleomorphic xanthoastrocytoma|
|Ependymal tumors||Subependymoma||Childhood Ependymoma Treatment|
|Other gliomas||Angiocentric glioma||Childhood Astrocytomas Treatment|
|Neuronal and mixed neuronal-glial tumors||Dysembryoplastic neuroepithelial tumor||Childhood Astrocytomas Treatment|
|Desmoplastic infantile astrocytoma and ganglioglioma|
|Papillary glioneuronal tumor|
|Rosette-forming glioneuronal tumor|
|Diffuse leptomeningeal glioneuronal tumor|
|Tumors of the pineal region||Pineoblastoma||Childhood Medulloblastoma and Other Central Nervous System Embryonal Tumors Treatment|
|Embryonal tumors||Medulloblastoma,WNT-activated||Childhood Medulloblastoma and Other Central Nervous System Embryonal Tumors Treatment|
|Medulloblastoma, SHH-activated andTP53-mutant; Medulloblastoma, SHH-activated andTP53-wildtype|
|Medulloblastoma, non-WNT/non-SHH group 3|
|Medulloblastoma, non-WNT/non-SHH group 4|
|Medulloblastoma with extensive nodularity|
|Medulloblastoma, large cell/anaplastic|
|Embryonal tumor with multilayered rosettes,C19MC-altered|
|Atypical teratoid/rhabdoid tumor||Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor Treatment|
|CNS embryonal tumor with rhabdoid features||Childhood Medulloblastoma and Other Central Nervous System Embryonal Tumors Treatment|
|Germ cell tumors||Germinoma||Childhood Central Nervous System Germ Cell Tumors Treatment|
|Yolk sac tumor|
|Teratoma with malignant transformation|
|Mixed germ cell tumor|
|Tumors of the sellar region||Adamantinomatous craniopharyngioma||Childhood Craniopharyngioma Treatment|
Relapse is not uncommon in both low-grade and malignant childhood brain tumors and may occur many years after initial treatment. Disease relapse may occur at the primary tumor site or, especially in malignant tumors, at noncontiguous CNS sites. Systemic relapse is rare but may occur in some tumor types. At 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 or the diagnosis of tumor transformation, which can include a change in grade and molecular makeup.[2,3] Other entities, such as secondary tumor and treatment-related intratumoral necrosis or frank brain necrosis, may be clinically indistinguishable from tumor recurrence. Determining 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.
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This summary was reformatted.
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Purpose of This Summary
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.
Reviewers and Updates
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).
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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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Levels of Evidence
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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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Last Revised: 2020-10-09
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