Note: Separate PDQ summaries on Childhood Brain Stem Glioma Treatment; Childhood Cerebellar Astrocytoma Treatment; Childhood Cerebral Astrocytoma/Malignant Glioma Treatment; Childhood Medulloblastoma Treatment; Childhood Supratentorial Primitive Neuroectodermal Tumors and PineoblastomaTreatment; and Childhood Visual Pathway and Hypothalamic Glioma Treatment are also available.

This cancer treatment information summary provides an overview of the diagnosis, classification, treatment, and prognosis of childhood brain tumors.

The National Cancer Institute provides the PDQ pediatric cancer treatment information summaries as a public service to increase the availability of evidence-based cancer information to health professionals, patients, and the public. These summaries are updated regularly according to the latest published research findings by an Editorial Board of pediatric oncology specialists.

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.

Approximately 50% of brain tumors in children are infratentorial, with three fourths of these located in the cerebellum or fourth ventricle. Common infratentorial (posterior fossa) tumors include the following:

1. Cerebellar astrocytoma (usually pilocytic but also fibrillary and, less frequently, high-grade).
2. Medulloblastoma (primitive neuroectodermal tumor [PNET]).
3. Ependymoma (cellular, papillary, clear cell, tanycytic or anaplastic).
4. Brain stem glioma (often diagnosed neuroradiologically without biopsy; more frequently high-grade and may be low-grade).
5. Atypical teratoid/rhabdoid tumors.

Supratentorial tumors include those tumors that occur in the sellar or suprasellar region and/or in the cerebrum or diencephalon. Sellar/suprasellar tumors comprise approximately 20% of childhood brain tumors and include the following:

1. Craniopharyngioma.
2. Diencephalic (central tumors involving the chiasm, hypothalamic, and/or thalamic) gliomas generally of low grade (including astrocytoma, grade 1 or 2).
3. Germ cell tumors (germinoma and nongerminomatous).

Other tumors that occur supratentorially include the following:

1. Low-grade cerebral hemispheric astrocytoma or glioma (grade 1 or grade 2).
2. High-grade or malignant astrocytoma (anaplastic astrocytoma, glioblastoma multiforme [grade 3 or grade 4]).
3. Mixed glioma (low-grade or high-grade).
4. Oligodendroglioma (low-grade or high-grade).
5. Primitive neuroectodermal tumor (including cerebral neuroblastoma, pineoblastoma, ependymoblastoma).
6. Ependymoma (cellular or anaplastic).
7. Meningioma.
8. Choroid plexus tumors (papilloma and carcinoma).
9. Pineal parenchymal tumors (pineocytoma, or mixed pineal parenchymal tumor).
10. Neuronal and mixed neuronal glial tumor (ganglioglioma, desmoplastic infantile ganglioglioma, dysembryoplastic neuroepithelial tumor).
11. Metastasis (rare) from extra neural malignancies.

Spinal cord tumors

Primary central nervous system spinal cord tumors comprise approximately 1% to 2% of all childhood nervous system tumors.[1,2,3] As is the case for primary brain tumors, such lesions are histologically heterogeneous. Approximately 70% of all intramedullary spinal cord tumors will be low-grade astrocytomas and/or gangliogliomas. Other tumor types which occur include ependymomas, higher-grade glial tumors, and (rarely) primitive neuroectodermal tumors. Myxopapillary ependymomas have a tendency to develop in the conus and cauda equina regions. Symptoms and signs of spinal cord tumors are highly dependent on the location of the tumor and its extent; some low-grade spinal cord tumors are associated with large cysts that extend rostrally and caudally and have been termed holocord astrocytomas. At times, it is impossible to distinguish a tumor which arises in the medulla from a tumor which arises in the upper cervical cord.