Neuro-Oncology Compendium for the Boards and Clinical Practice, p. 240-C16.P236, 2023
DOI: 10.1093/med/9780197573778.003.0016
Abstract This chapter on pediatric embryonal tumors encompasses the diagnoses of medulloblastoma, atypical teratoid/rhabdoid tumor (ATRT), pineoblastoma, embryonal tumor with multilayered rosettes (ETMR), and other embryonal tumors as delineated by the World Health Organization’s 2016 central nervous system tumor classification system. Each section reviews the epidemiology, clinical presentation, imaging, and pathological characteristics as well as principles of management informed by the most recent available medical literature. Additionally, the chapter describes the evolving understanding of each tumor that has been enabled by modern molecular characterization, including the latest consensus on medulloblastoma subtypes wingless ([WNT]-activated, sonic hedgehog [SHH]-activated, Group 3, and Group 4) and the emerging insights in other entities.