1.Brigham and Women's Hospital, Harvard Medical School, Boston , MA (USA)
Among embryonal tumors of the central nervous system (CNS), we primarily recognize the relatively common medulloblastoma which, by definition, arise from the cerebellum or dorsal brainstem. However, several rare “other embryonal tumors” which arise across the neuroaxis, are also contained within this group. In the 2021 World Health Organization’s (WHO) classification of CNS tumors, these “other embryonal tumors” include: i) atypical teratoid/rhabdoid tumor (AT/RT), ii) embryonal tumor with multilayered rosettes (ETMR), iii) CNS neuroblastoma, FOXR2-activated, iv) CNS tumor with BCOR internal tandem duplication, and v) CNS embryonal tumor NEC/NOS. 1 Previously, many of these entities were considered “CNS primitive neuroectodermal tumors (PNET),” a classification that has since been retired due to an increased understanding of the tumor's biology and molecular features. 2,3
Scott Ryall
Other CNS Embryonal Tumors
Atlas Genet Cytogenet Oncol Haematol. 2023-08-10
Online version: http://atlasgeneticsoncology.org/solid-tumor/209202