Astrocytomas are childhood central nervous system tumors that develop from astrocytes. Diagnosis is based on MRI. Treatment is a combination of surgical resection and chemotherapy. Radiation therapy is rarely used to treat low-grade tumors but is commonly used to treat high-grade tumors.
Astrocytes are star-shaped glial cells that form a large portion of the brain parenchyma and serve various functions depending on their location in the central nervous system (CNS).
Astrocytomas range from low-grade indolent tumors (the most prevalent, including pilocytic astrocytoma and diffuse low-grade glioma) to malignant high-grade tumors (including anaplastic astrocytoma and glioblastoma).
As a group, astrocytomas are the most common CNS tumor in children, representing approximately 40% of pediatric CNS tumors (1, 2). Most cases occur in children younger than 9 years, with a peak incidence between 6 years and 8 years (3–5). These tumors can occur anywhere in the brain or spinal cord but are common in the cerebellum (6).
Despite similarities in appearance, astrocytomas in children and adults have different biological backgrounds with different molecular findings, leading to differences in clinical features such as where the tumors arise, how they are treated, and overall outcomes (7, 8).
General references
1. 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
2. Lamba N, Groves A, Torre M, Yeo KK, Iorgulescu JB. The epidemiology of primary and metastatic brain tumors in infancy through childhood. J Neurooncol. 2022;156(2):419-429. doi:10.1007/s11060-021-03927-z
3. Schmidt LS, Schmiegelow K, Lahteenmaki P, et al. Incidence of childhood central nervous system tumors in the Nordic countries. Pediatr Blood Cancer. 2011;56(1):65-69. doi:10.1002/pbc.22585
4. Helligsoe ASL, Kenborg L, Henriksen LT, Udupi A, Hasle H, Winther JF. Incidence and survival of childhood central nervous system tumors in Denmark, 1997-2019. Cancer Med. 2022;11(1):245-256. doi:10.1002/cam4.4429
5. Wellbrock M, Voigt M, Ronckers C, Grabow D, Spix C, Erdmann F. Registration, incidence patterns, and survival trends of central nervous system tumors among children in Germany 1980-2019: An analysis of 40 years based on data from the German Childhood Cancer Registry. Pediatr Blood Cancer. 2024;71(6):e30954. doi:10.1002/pbc.30954
6. Cohen AR. Brain Tumors in Children. N Engl J Med. 2022;386(20):1922-1931. doi:10.1056/NEJMra2116344
7. Jones C, Perryman L, Hargrave D. Paediatric and adult malignant glioma: Close relatives or distant cousins? Nat Rev Clin Oncol. 2012;9(7):400–413. doi:10.1038/nrclinonc.2012.87
8. Greuter L, Guzman R, Soleman J: Pediatric and adult low-grade gliomas: Where do the differences lie? Children (Basel) 8(11):1075, 2021. doi: 10.3390/children8111075
Symptoms and Signs of Astrocytomas in Children
Symptoms and signs are typically associated with the location of the tumor. Many patients have symptoms consistent with increased intracranial pressure (eg, morning headaches, vomiting, lethargy). Other common tumor locations and associated symptoms and signs include:
Cerebellum: Weakness, tremor, or ataxia
Cerebral hemispheres: Weakness, numbness, or seizures
Visual pathway: Visual loss, proptosis, or nystagmus
Spinal cord: Pain, weakness, changes in bowel/bladder function, or gait disturbance
Diagnosis of Astrocytomas in Children
Contrast-enhanced MRI
Biopsy with tumor molecular profiling
Contrast-enhanced MRI is the imaging test of choice for diagnosing the tumor, determining extent of disease, and detecting recurrence (1, 2). Contrast-enhanced CT can also be used, but it is less accurate.
The T2-FLAIR MRI scan (top) shows a large, bilateral white (hyperdensity) signal around a glioblastoma, which is the highest grade and most malignant astrocytoma. This particular glioblastoma is called a butterfly glioma because the white signal around the tumor forms the wings of the butterfly; it is caused by cerebral edema. On the T1-weighted scan (bottom), contrast outlines the edge of the tumor (ring enhancement). The edema appears as a darkened area (hypointensity) on T1.
Images courtesy of William R. Shapiro, MD.
This contrast-enhanced axial MRI through the posterior fossa shows a well circumscribed, partially cystic mass with a solid region of pathologic enhancement along the back wall of the mass. This mass is located in the left cerebellum. There is mass effect with compression of the fourth ventricle.
LIVING ART ENTERPRISES, LLC/SCIENCE PHOTO LIBRARY
With exceptions, biopsy is needed for determining tumor type, grade, and genetics (2). These tumors are typically classified as low grade (eg, pilocytic astrocytoma, diffuse low-grade glioma) or high grade (eg, anaplastic astrocytoma, glioblastoma) (3, 4). Many pathologists designate grades 1 and 2 tumors as low grade and grades 3 and 4 tumors as high grade.
Tumor molecular profiling, including tumor genetics, contributes to integrated diagnosis and grading.
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. 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.
3. Horbinski C, Solomon DA, Lukas RV, et al. Molecular Testing for the World Health Organization Classification of Central Nervous System Tumors: A Review. JAMA Oncol. 2025;11(3):317-328. doi:10.1001/jamaoncol.2024.5506
4. Cohen AR. Brain Tumors in Children. N Engl J Med. 2022;386(20):1922-1931. doi:10.1056/NEJMra2116344
Treatment of Astrocytomas in Children
Surgical resection
Sometimes chemotherapy
Treatment of astrocytoma depends on location and grade of the tumor. As a general rule, the lower the grade of the tumor, the less intensive the therapy and the better the outcome.
Low-grade astrocytomas
Surgical resection is the primary treatment, and total resection is the goal (if safe) (1). Even after local recurrence, a second surgical resection can be beneficial depending on the location of the tumor.
Chemotherapy is commonly used when treatment is needed, especially for children whose tumors are unresectable, cannot be completely excised, or progress/recur after surgery (1).
Radiation therapy is rarely used in children with low-grade astrocytoma, and it is avoided if possible because of the associated long-term cognitive impairment and other adverse effects such as secondary cancer and vasculopathy.
Targeted therapies (eg, tovorafenib, dabrafenib plus trametinib) have been shown to be beneficial in the treatment of tumors with specific genetic mutations (eg, BRAF V600E, KIAA1549-BRAF fusion) in phase 2 trials (2, 3).
Most children with low-grade astrocytomas have an excellent overall outcome and are long-term survivors (1, 4).
High-grade astrocytomas
These tumors are treated with a combination of surgery (unless location precludes it), radiation therapy, and chemotherapy. The most commonly used chemotherapy agents are temozolomide and lomustine (CCNU) (1, 5–7).
Prognosis is poor. One population-based study reported a survival rate of 24% at 5 years and 20% at 10 years; others have reported a 10 to 30% survival rate at just 2 years (4, 8).
IDH-mutant high-grade astrocytoma in children is relatively uncommon (1 to 20%, possibly more in adolescents), and is classified as an adult-type diffuse glioma (9, 10). It has a prognosis generally more favorable than IDH wild-type tumors but is still variable based on molecular features and long-term follow-up care (10). Management is the same as for adults and emphasizes maximal safe resection and risk-adapted use of radiation, chemotherapy, and emerging targeted therapies such as IDH inhibitors while carefully balancing treatment toxicity in the developing brain.
Treatment references
1. Cohen AR. Brain Tumors in Children. N Engl J Med. 2022;386(20):1922-1931. doi:10.1056/NEJMra2116344
2. Kilburn LB, Khuong-Quang DA, Hansford JR, et al. The type II RAF inhibitor tovorafenib in relapsed/refractory pediatric low-grade glioma: the phase 2 FIREFLY-1 trial. Nat Med. 2024;30(1):207-217. doi:10.1038/s41591-023-02668-y
3. Bouffet E, Hansford JR, Garrè ML, et al. Dabrafenib plus Trametinib in Pediatric Glioma with BRAF V600 Mutations. N Engl J Med. 2023;389(12):1108-1120. doi:10.1056/NEJMoa2303815
4. Hoogendijk R, Capocaccia R, van der Lugt J, et al. Long-term survival and cure fraction estimates for paediatric central nervous system tumours in 31 European countries (EUROCARE-6): a population-based study. Lancet Oncol. 2025;26(8):1091-1099. doi:10.1016/S1470-2045(25)00297-9
5. Jakacki RI, Cohen KJ, Buxton A, et al. Phase 2 study of concurrent radiotherapy and temozolomide followed by temozolomide and lomustine in the treatment of children with high-grade glioma: a report of the Children's Oncology Group ACNS0423 study. Neuro Oncol. 2016;18(10):1442-1450. doi:10.1093/neuonc/now038
6. Cohen KJ, Pollack IF, Zhou T, et al. Temozolomide in the treatment of high-grade gliomas in children: A report from the Children's Oncology Group. Neuro Oncol. 2011;13(3):317–323. doi:10.1093/neuonc/noq191
7. 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.
8. Kallappagoudar S, Yadav RK, Lowe BR, Partridge JF. Histone H3 mutations--a special role for H3.3 in tumorigenesis?. Chromosoma. 2015;124(2):177-189. doi:10.1007/s00412-015-0510-4
9. Yeo KK, Alexandrescu S, Cotter JA, et al. Multi-institutional study of the frequency, genomic landscape, and outcome of IDH-mutant glioma in pediatrics. Neuro Oncol. 2023;25(1):199-210. doi:10.1093/neuonc/noac132
10. Evans L, Trinder S, Dodgshun A, et al. IDH-mutant gliomas in children and adolescents - from biology to clinical trials. Front Oncol. 2025;14:1515538. Published 2025 Jan 6. doi:10.3389/fonc.2024.1515538
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