PRIMITIVE NEUROECTODERMAL TUMOR

Course overview

IN 2016, THE WORLD HEALTH ORGANIZATION PUBLISHED A REVISED CLASSIFICATION OF CENTRAL NERVOUS SYSTEM TUMORS USING MOLECULAR PARAMETERS. IN THIS CLASSIFICATION, SOME TUMORS PREVIOUSLY RECOGNIZED IN THE 2007 CLASSIFICATION HAD BEEN RENAMED OR ELIMINATED. THE PRIMITIVE NEUROECTODERMAL TUMOR IS NO LONGER RECOGNIZED AS SUCH AND THOSE TUMORS ARE NOW UNDER THE CLASSIFICATION OF EMBRYONAL TUMORS. MEDULLOBLASTOMAS ARE EMBRYONAL TUMORS BUT HAVE THEIR OWN GENETICALLY (MOLECULAR) AND HISTOLOGICALLY DEFINED GROUPS. THE OTHER EMBRYONAL TUMORS ARE CLASSIFIED DEPENDING IF THEY HAVE ON CHROMOSOME 19 AN AMPLIFICATION OF THE C19MC REGION (19Q13.42). THOSE EMBRYONAL TUMORS THAT NO AMPLIFICATION OF THE C19MC, NO ALTERATIONS OF INI1 OR BRG1, OR NO ROSETTES ARE IDENTIFIED, ARE CALLED EMBRYONAL TUMOR, NOS (WHO 9473/3). THIS ACTIVITY REVIEWS THE EVALUATION, AND MANAGEMENT OF EMBRYONAL TUMORS, AND HIGHLIGHTS THE ROLE OF THE INTERPROFESSIONAL TEAM IN MANAGING PATIENTS WITH THIS CONDITION.