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Epidemiology of central nervous system tumors in children

Ching Lau, MD, PhD
Section Editor
David G Poplack, MD
Deputy Editor
Carrie Armsby, MD, MPH


Central nervous system (CNS) tumors include both non-malignant and malignant tumors of the brain and spinal cord. Primary malignant CNS tumors are the second most common childhood malignancies, after hematological malignancies, and are the most common pediatric solid organ tumor (table 1) [1]. Although progress has been made in the treatment of childhood tumors, significant mortality and morbidity are still associated with malignant brain tumors.

The epidemiology of CNS tumors in children will be reviewed here. The clinical manifestations, diagnosis, and overview of the management of CNS tumors and outcome in children are discussed separately. (See "Clinical manifestations and diagnosis of central nervous system tumors in children" and "Overview of the management of central nervous system tumors in children".)


In the United States (US), based upon data from the Central Brain Tumor Registry of the US (CBTRUS), the estimated incidence of primary non-malignant and malignant CNS tumors is 5.4 cases per 100,000 person-years for children and adolescents ≤19 years of age [2]. The estimated five-year survival rate for all primary CNS tumors is about 66 percent in patients ≤19 years of age, resulting in about 26,000 children living in the United States with a CNS tumor.

In the US, CNS tumors are the most common solid tumors in children (table 1) and are the leading cause of cancer death in children 0 through 14 years [1,3]. In the CBTRUS report, CNS tumors accounted for 29 percent of all malignancies in children <15 years of age [3].

In Germany, the incidence of childhood CNS tumors is lower, with a reported rate of 2.6 per 100,000 children <15 years based on epidemiologic data from 1990 to 1999 [4].


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Literature review current through: Jul 2017. | This topic last updated: Dec 07, 2015.
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  1. Linabery AM, Ross JA. Trends in childhood cancer incidence in the U.S. (1992-2004). Cancer 2008; 112:416.
  2. Ostrom QT, Gittleman H, Liao P, et al. CBTRUS statistical report: primary brain and central nervous system tumors diagnosed in the United States in 2007-2011. Neuro Oncol 2014; 16 Suppl 4:iv1.
  3. Ostrom QT, de Blank PM, Kruchko C, et al. Alex's Lemonade Stand Foundation Infant and Childhood Primary Brain and Central Nervous System Tumors Diagnosed in the United States in 2007-2011. Neuro Oncol 2015; 16 Suppl 10:x1.
  4. Kaatsch P, Rickert CH, Kühl J, et al. Population-based epidemiologic data on brain tumors in German children. Cancer 2001; 92:3155.
  5. Wilson PE, Oleszek JL, Clayton GH. Pediatric spinal cord tumors and masses. J Spinal Cord Med 2007; 30 Suppl 1:S15.
  6. Zhou D, Zhang Y, Liu H, et al. Epidemiology of nervous system tumors in children: a survey of 1,485 cases in Beijing Tiantan Hospital from 2001 to 2005. Pediatr Neurosurg 2008; 44:97.
  7. Bleyer A, O’Leary M, Barr R, Ries LAG (eds): Cancer Epidemiology in Older Adolescents and Young Adults 15 to 29 Years of Age, Including SEER Incidence and Survival: 1975-2000. National Cancer Institute, NIH Pub. No. 06-5767. Bethesda, MD 2006.
  8. Louis DN, Ohgaki H, Wiestler OD, Cavenee WK. Classification of Tumours of the Nervous System, IARC Press, Lyon, France 2007.
  9. Allen JC, Siffert J, Hukin J. Clinical manifestations of childhood ependymoma: a multitude of syndromes. Pediatr Neurosurg 1998; 28:49.
  10. Taylor AJ, Little MP, Winter DL, et al. Population-based risks of CNS tumors in survivors of childhood cancer: the British Childhood Cancer Survivor Study. J Clin Oncol 2010; 28:5287.
  11. Zhang J, Walsh MF, Wu G, et al. Germline Mutations in Predisposition Genes in Pediatric Cancer. N Engl J Med 2015; 373:2336.