Central nervous system tumors are the most common solid cancer in children < 15 years old and are the leading cause of childhood death due to cancer. Diagnosis is typically by imaging (usually MRI) and biopsy. Treatment may include surgical resection, chemotherapy, targeted therapy, and radiation therapy.
Central nervous system (CNS) tumors are the leading cause of cancer mortality in children. In the United States in 2022, brain and nervous system cancers accounted for 40% of cancer deaths (1). The cause of most childhood CNS tumors is unknown, but 2 established risk factors are ionizing radiation (eg, high-dose cranial irradiation) and specific genetic syndromes (eg, neurofibromatosis) (2).
The most common CNS tumors in children are (in order) (2):
Less common CNS tumors in children include craniopharyngiomas, meningioma, choroid plexus tumors, CNS germ cell tumors and other CNS embryonal tumors (eg, pineoblastoma, atypical teratoid/rhabdoid tumors [ATRTs]) (2).
Starting from the top right label:
Astrocytoma (glioma)
Optic nerve glioma
Craniopharyngioma
Pituitary adenoma
Schwann cell tumor (nerve sheath tumor)
Ependymoma (glioma)
Brain stem glioma
Medulloblastoma
Pineal gland tumor
Meningioma
Oligodendroglioma (glioma)
designua/stock.adobe.com
General references
1. Siegel RL, Kratzer TB, Giaquinto AN, Sung H, Jemal A. Cancer statistics, 2025. CA Cancer J Clin. 2025;75(1):10-45. doi: 10.3322/caac.21871
2. Price M, Ballard C, Benedetti J, et al. CBTRUS Statistical Report: Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2017-2021. Neuro Oncol. 2024;26(Supplement_6):vi1-vi85. doi:10.1093/neuonc/noae145
Symptoms and Signs of CNS Tumors in Children
Increased intracranial pressure resulting from obstructive hydrocephalus is the cause of the most common manifestations, which include:
An enlarged head in infants and children
Headache
Nausea and vomiting
Irritability
Lethargy
Changes in behavior
Gait and balance disorders
Other common symptoms are related to tumor location, including vision loss and seizures.
Spinal tumors can cause weakness, paresthesias, ataxia, loss of bowel or bladder control, or other symptoms based on specific location.
Diagnosis of CNS Tumors in Children
MRI
Biopsy or resection, often with tumor molecular profiling
Sometimes lumbar puncture
MRI is the imaging test of choice because it provides more detailed images of parenchymal tumors and can detect tumors within the posterior fossa, subarachnoid spaces, and the arachnoid and pia mater (1). CT may be performed initially for emergencies, particularly if acute intracranial bleeding is suspected, but in general is inferior to MRI for evaluating brain tumors.
Once imaging demonstrates the presence of a brain mass, a biopsy is usually, but not always, needed to confirm the specific cancer diagnosis and to determine tumor type and grade (2). The World Health Organization (WHO) has created a commonly used classification system (3) that includes molecular and histologic information to further classify CNS tumors.
Once a diagnosis is made and tumor histology and molecular profiling (if needed) are determined, staging and risk assessment are then performed. Staging includes an MRI of the entire spine, often a lumbar puncture for cerebrospinal fluid cytology, and a postoperative MRI to assess for any residual tumor (4–6). Risk assessment is generally based on age, degree of residual tumor, molecular findings, and evidence of metastasis.
Diagnosis references
1. Jaju A, Li Y, Dahmoush H, et al. Imaging of pediatric brain tumors: A COG Diagnostic Imaging Committee/SPR Oncology Committee/ASPNR White Paper. Pediatr Blood Cancer. 2023;70 Suppl 4(Suppl 4):e30147. doi:10.1002/pbc.30147
2. Soleman J, Dvir R, Ben-Sira L, et al. MRI-based diagnosis and treatment of pediatric brain tumors: is tissue sample always needed?. Childs Nerv Syst. 2021;37(5):1449-1459. doi:10.1007/s00381-021-05148-1
3. Louis DN, Perry A, Wesseling P, et al. The 2021 WHO Classification of Tumors of the Central Nervous System: a summary. Neuro Oncol. 2021;23(8):1231-1251. doi:10.1093/neuonc/noab106
4. Rogers SN, Udayasankar U, Pruthi S, et al. Imaging of pediatric spine and spinal cord tumors: ACOG Diagnostic Imaging Committee/SPR Oncology Committee/ASPNR White Paper. Pediatr Blood Cancer. 2023;70 Suppl 4(Suppl 4):e30150. doi:10.1002/pbc.30150
5. National Comprehensive Cancer Network. NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines). Pediatric Central Nervous System Cancers, version 1.2026. https://www.nccn.org/professionals/physician_gls/pdf/ped_cns.pdf. Accessed May 19, 2026.
6. Sacerdote C, Mosso ML, Alessi D, et al. An application of the Toronto Childhood Cancer Stage Guidelines in three population-based cancer registries: The case of central nervous tumors. Pediatr Blood Cancer. 2020;67(6):e28303. doi:10.1002/pbc.28303
Treatment of CNS Tumors in Children
Surgical resection
Radiation therapy, chemotherapy, targeted therapy, or a combination
Relief of obstructed cerebrospinal fluid flow if necessary
Treatment approach depends on the diagnosis, grade, stage, and risk assessment. In general, after initial surgery, radiation therapy, chemotherapy, targeted therapy, or a combination may be required.
Entry into a clinical trial, if available, should be considered for all children with a CNS tumor. Optimal treatment requires a multidisciplinary team of pediatric oncologists, pediatric neuro-oncologists, pediatric neurosurgeons, neuropathologists, neuroradiologists, pediatric neurologists, and radiation oncologists who have experience treating CNS tumors in children (1). Because radiation therapy for CNS tumors is technically demanding, children should be sent to centers that have experience in this area if possible.
If the flow of cerebrospinal fluid is obstructed, a shunt or an endoscopic third ventriculostomy (ETV) may be needed.
Treatment reference
1. National Comprehensive Cancer Network. NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines). Pediatric Central Nervous System Cancers, version 1.2026. https://www.nccn.org/professionals/physician_gls/pdf/ped_cns.pdf. Accessed May 19, 2026.
