Childhood Brain and Spinal Cord Tumors Summary Index (PDQ®): Treatment - Health Professional Information [NCI]

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.
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.

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)a	Pathological Subtype (Based on the 2021 WHO Classification)a	Related PDQ Treatment Summary
CNS = central nervous system; NEC = not elsewhere classified; NOS = not otherwise specified; WHO = World Health Organization.
a WHO classification adapted from Louis et al.[1]
Pediatric-type diffuse high-grade gliomas	Diffuse pediatric-type high-grade glioma, H3-wild type andIDH-wild type	Childhood 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 gliomas	Pilocytic astrocytoma	Childhood 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 tumors	Subependymoma	Childhood Ependymoma Treatment
	Myxopapillary ependymoma
	Supratentorial ependymoma, NOS
	Supratentorial ependymoma,ZFTAfusion–positive
	Supratentorial ependymoma,YAP1fusion–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 glioma	Diffuse astrocytoma,MYB- orMYBL1-altered	Childhood 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 tumors	Dysembryoplastic neuroepithelial tumor	Childhood 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 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
	Medulloblastoma, histologically defined
	Desmoplastic nodular medulloblastoma
	Medulloblastoma with extensive nodularity
	Large cell medulloblastoma
	Anaplastic medulloblastoma
	Embryonal tumor with multilayered rosettes,C19MC-altered
	CNS tumor withBCORinternal tandem duplication
	CNS neuroblastoma,FOXR2-activated
	CNS embryonal tumor, NEC/NOS
	Atypical teratoid/rhabdoid tumor	Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor Treatment
Germ cell tumors	Germinoma	Childhood 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 region	Adamantinomatous craniopharyngioma	Childhood 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.

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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Last Revised: 2023-12-19