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Epidemiology of Pediatric Central Nervous System Tumors

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Brain Tumors in Children

Abstract

Tumors of the central nervous system (CNS) comprise a broad and diverse collection of neoplasms within pediatric oncology. Yet when taken together pediatric brain and spine tumors represent the most common childhood cancer with an incidence of 5.57 per 100,000 annually and are a leading cause of cancer-related death in patients under 19 years of age (Ostrom et al. 2014; Siegel et al. 2015). Factors such as genetic predisposition, age, and sex play an increasingly significant role in understanding presentation, management, and etiology of childhood brain tumors. Although long-standing observations regarding general patterns of CNS tumors continue to be clinically useful, the introduction of molecular subtypes, such as in medulloblastoma and ependymoma, and the discovery of epigenetic regulators, such as in diffuse intrinsic pontine gliomas (DIPG) and other diffuse midline gliomas with H3K27M mutations, have repurposed epidemiological findings and reconceptualized CNS tumor classification (Louis et al. 2016). The elucidation of the molecular profile of pediatric CNS tumors has made it clear that epidemiology, viewed through a prism of genetics and epigenetics, can offer even greater insights into this incredibly challenging group of tumors. Epidemiology today considers not only environmental, parental, and birth factors that may increase the risk of pediatric CNS tumors, but also germline and molecular features that are causal or pathognomonic of tumor types and subtypes.

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Vitanza, N.A., Campen, C.J., Fisher, P.G. (2018). Epidemiology of Pediatric Central Nervous System Tumors. In: Gajjar, A., Reaman, G., Racadio, J., Smith, F. (eds) Brain Tumors in Children. Springer, Cham. https://doi.org/10.1007/978-3-319-43205-2_1

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