Embryonal Tumors of the Central Nervous System: From the Radiologic Pathology Archives

Radiographics. 2018 Mar-Apr;38(2):525-541. doi: 10.1148/rg.2018170182.

Abstract

Embryonal tumors of the central nervous system (CNS) are highly malignant undifferentiated or poorly differentiated tumors of neuroepithelial origin and have been defined as a category in the World Health Organization (WHO) classification since the first edition of the "Blue Book" in 1979. This category has evolved over time to reflect our ever-improving understanding of tumor biology and behavior. With the most recent update in 2016, many previous histologic diagnoses incorporate molecular parameters for the first time (genetically defined entities). While medulloblastoma and atypical teratoid/rhabdoid tumor are familiar carryovers from the 2007 CNS WHO classification, there are major changes to the embryonal tumor category: for example, elimination of the term CNS primitive neuroectodermal tumor and addition of a new genetically defined entity, embryonal tumor with multilayered rosettes, C19MC-altered. The purpose of this article is to discuss both the radiologic-pathologic features of CNS embryonal tumors and the new molecularly defined types/subtypes that will become the standard classification/terminology for future diagnoses and tumor research.

Publication types

  • Review

MeSH terms

  • Biomarkers, Tumor / analysis
  • Central Nervous System Neoplasms / diagnostic imaging*
  • Central Nervous System Neoplasms / pathology
  • Contrast Media
  • Diagnosis, Differential
  • Humans
  • Medulloblastoma / diagnostic imaging*
  • Medulloblastoma / pathology
  • Neoplasms, Germ Cell and Embryonal / diagnostic imaging*
  • Neoplasms, Germ Cell and Embryonal / pathology
  • Neuroendocrine Tumors / diagnostic imaging*
  • Neuroendocrine Tumors / pathology
  • Rhabdoid Tumor / diagnostic imaging*
  • Rhabdoid Tumor / pathology
  • Terminology as Topic

Substances

  • Biomarkers, Tumor
  • Contrast Media