Purpose or Case Report: The 2016 World Health Organization Classification of Tumors of the Central Nervous System has now incorporated molecular and genetic parameters in addition to histology to define many tumor entities. Significant restructuring has occurred for pediatric CNS tumors. For example, medulloblastomas are classified into four genetic subtypes. Other embryonal tumors such as embryonal tumor with multilayered rosettes (ETMR) and atypical teratoid/rhabdoid tumor (ATRT) are further defined by their molecular features. Also new entities have been added defined by both histology and molecular signatures including H3 K27M-mutant diffuse midline glioma, RELA fusion-positive ependymoma and diffuse leptomeningeal glioneuronal tumor (DLGNT).
These more homogeneous and narrowly defined entities are expected to facilitate better classification, prognostication and patient stratification for precision therapy. This also improves the design of clinical trials and experimental models.
In this presentation, we will review the new WHO classification scheme and review the imaging and as well as molecular/genetic features of pediatric CNS tumors.
Radiologists must keep up to date with updates to the WHO classification scheme to be able to better communicate with clinicians ensure optimal patient care and relevant research collaboration.

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