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Childhood Brain and Spinal Cord Tumors Summary Index (PDQ®)–Health Professional Version

General Information About Childhood Brain and Spinal Cord Tumors

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.[1]

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.[2]

References
  1. Ostrom QT, Gittleman H, Farah P, et al.: CBTRUS statistical report: Primary brain and central nervous system tumors diagnosed in the United States in 2006-2010. Neuro Oncol 15 (Suppl 2): ii1-56, 2013. [PUBMED Abstract]
  2. Louis DN, Perry A, Reifenberger G, et al.: The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary. Acta Neuropathol 131 (6): 803-20, 2016. [PUBMED Abstract]

Type of Childhood Brain and Spinal Cord Tumors

For information about the type of childhood brain and spinal cord tumor and its related PDQ summary, see the table below. If a tumor type is not listed, a corresponding PDQ treatment summary is not available.

CNS Tumor Type, Pathological Subtype, and Its Related PDQ Treatment Summary
Tumor Type (Based on the 2021 WHO Classification)aPathological Subtype (Based on the 2021 WHO Classification)aRelated PDQ Treatment Summary
CNS = central nervous system; NEC = not elsewhere classified; NOS = not otherwise specified; WHO = World Health Organization.
aWHO classification adapted from Louis et al.[1]
Pediatric-type diffuse high-grade gliomasDiffuse pediatric-type high-grade glioma, H3-wild type and IDH-wild typeChildhood Astrocytomas, Other Gliomas, and Glioneuronal/Neuronal Tumors Treatment 
Diffuse midline glioma, H3 K27-altered
Diffuse hemispheric glioma, H3 G34-mutant
Infant-type hemispheric glioma
Circumscribed astrocytic gliomasPilocytic astrocytomaChildhood Astrocytomas, Other Gliomas, and Glioneuronal/Neuronal Tumors Treatment
High-grade astrocytoma with piloid features
Subependymal giant cell astrocytoma
Pleomorphic xanthoastrocytoma
Choroid glioma
Astroblastoma, MN1-altered
Ependymal tumorsSubependymomaChildhood Ependymoma Treatment
Myxopapillary ependymoma
Supratentorial ependymoma, NOS
Supratentorial ependymoma, ZFTA fusion–positive
Supratentorial ependymoma, YAP1 fusion–positive
Posterior fossa ependymoma, NOS
Posterior fossa group A (PFA) ependymoma
Posterior fossa group B (PFB) ependymoma
Spinal ependymoma, NOS
Spinal ependymoma, MYCN-amplified
Pediatric-type diffuse low-grade gliomaDiffuse astrocytoma, MYB- or MYBL1-alteredChildhood Astrocytomas, Other Gliomas, and Glioneuronal/Neuronal Tumors Treatment
Angiocentric glioma
Polymorphous low-grade neuroepithelial tumor of the young
Diffuse low-grade glioma, MAPK pathway-altered
Glioneuronal and neuronal tumorsDysembryoplastic neuroepithelial tumorChildhood Astrocytomas, Other Gliomas, and Glioneuronal/Neuronal Tumors Treatment
Ganglioglioma
Gangliocytoma
Desmoplastic infantile ganglioglioma
Desmoplastic infantile astrocytoma
Diffuse glioneuronal tumor with oligodendroglioma-like features and nuclear clusters (provisional entity)
Papillary glioneuronal tumor
Rosette-forming glioneuronal tumor
Myxoid glioneuronal tumor
Diffuse leptomeningeal glioneuronal tumor
Multinodular and vacuolating neuronal tumor
Dysplastic cerebellar gangliocytoma (Lhermitte-Duclos disease)
Extraventricular neurocytoma
Cerebellar liponeurocytoma
Central neurocytoma
Tumors of the pineal regionPineoblastomaChildhood Medulloblastoma and Other Central Nervous System Embryonal Tumors Treatment
Embryonal tumorsMedulloblastoma, WNT-activatedChildhood Medulloblastoma and Other Central Nervous System Embryonal Tumors Treatment
Medulloblastoma, SHH-activated and TP53-mutant; Medulloblastoma, SHH-activated and TP53-wildtype
Medulloblastoma, non-WNT/non-SHH
Medulloblastoma, histologically defined
Desmoplastic nodular medulloblastoma
Medulloblastoma with extensive nodularity
Large cell medulloblastoma
Anaplastic medulloblastoma
Embryonal tumor with multilayered rosettes, C19MC-altered
CNS tumor with BCOR internal tandem duplication
CNS neuroblastoma, FOXR2-activated
CNS embryonal tumor, NEC/NOS
Atypical teratoid/rhabdoid tumorChildhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor Treatment
Germ cell tumorsGerminomaChildhood Central Nervous System Germ Cell Tumors Treatment
Embryonal carcinoma
Yolk sac tumor
Choriocarcinoma
Mature teratoma
Immature teratoma
Teratoma with somatic-type malignancy
Mixed germ cell tumor
Tumors of the sellar regionAdamantinomatous craniopharyngiomaChildhood Craniopharyngioma Treatment
Papillary craniopharyngioma
References
  1. Louis DN, Perry A, Wesseling P, et al.: The 2021 WHO Classification of Tumors of the Central Nervous System: a summary. Neuro Oncol 23 (8): 1231-1251, 2021. [PUBMED Abstract]

Latest Updates to This Summary (12/19/2023)

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 and extensively revised.

This summary was renamed from Childhood Brain and Spinal Cord Tumors Treatment Overview.

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® Cancer Information for Health Professionals pages.

About This PDQ Summary

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 in the care of their 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).

Board members review recently published articles each month to determine whether an article should:

  • be discussed at a meeting,
  • be cited with text, or
  • replace or update an existing article that is already cited.

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 Summary Index are:

  • Kenneth J. Cohen, MD, MBA (Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins Hospital)
  • Louis S. Constine, MD (James P. Wilmot Cancer Center at University of Rochester Medical Center)
  • Roger J. Packer, MD (Children's National Hospital)
  • D. Williams Parsons, MD, PhD (Texas Children's Hospital)
  • Malcolm A. Smith, MD, PhD (National Cancer Institute)

Any comments or questions about the summary content should be submitted to Cancer.gov through the NCI website's Email Us. Do not contact the individual Board Members with questions or comments about the summaries. Board members will not respond to individual inquiries.

Levels of Evidence

Some of the reference citations in this summary are accompanied by a level-of-evidence designation. These designations are intended to help readers assess the strength of the evidence supporting the use of specific interventions or approaches. The PDQ Pediatric Treatment Editorial Board uses a formal evidence ranking system in developing its level-of-evidence designations.

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The preferred citation for this PDQ summary is:

PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Brain and Spinal Cord Tumors Summary Index. 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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