Embryonale Tumoren des Zentralnervensystems

Robert Y. Shih; Kelly K. Koeller

doi:10.1055/a-0677-4094

Neuroradiologie Scan, Table of Contents

Neuroradiologie Scan 2019; 09(01): 47-68
DOI: 10.1055/a-0677-4094

CME-Fortbildung

Embryonale Tumoren des Zentralnervensystems

Embryonal Tumors of the Central Nervous System

Robert Y. Shih

,

Kelly K. Koeller

Subject Editor: Wissenschaftlich verantwortlich gemäß Zertifizierungsbestimmungen für diesen Beitrag ist Professor Dr. Michael Forsting, Essen

Abstract

Die Erkenntnisse zu Biologie und Verhalten der hochmalignen embryonalen Tumoren des Zentralnervensystems nehmen stetig zu. Das schlägt sich auch in ihrer sich über die Jahre wandelnden Terminologie und Klassifizierung nieder. Dieser Artikel stellt wichtige Änderungen des neuen Klassifikationsschemas der Weltgesundheitsorganisation 2016 vor, die für die Diagnostik und Tumorforschung in Zukunft den neuen Standard bilden werden.

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.

Schlüsselwörter

embryonale ZNS-Tumoren - WHO-Klassifikation 2016 - molekulare Marker - bildgebende pathologische Befunde

Keywords

embryonal tumors of the CNS - WHO classification 2016 - molecular parameters - radiologic-pathologic features

Full Text

References

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