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Neuropathologie pädiatrischer Hirntumore

Implikationen der 5. Edition der WHO-Klassifikation der Hirntumore

Neuropathology of pediatric brain tumors

Implications of the 5th edition of the WHO classification of central nervous system tumors

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Zusammenfassung

Hintergrund

Schon in der Fortschreibung der 4. Edition der WHO-Klassifikation der Hirntumore wurde darauf hingewiesen, dass diffuse pädiatrische Gliome nicht denselben molekularen Mechanismen folgen wie Gliome im Erwachsenenalter.

Fragestellung

Welche Änderungen ergeben sich aus der Fortschreibung der Klassifikation der Hirntumore?

Material und Methoden

Mit der 5. Edition der WHO-Klassifikation der Hirntumore wird neben der histologischen Charakterisierung und Gradierung eine zweite, molekularpathologische Informationsebene etabliert.

Ergebnisse

Es wird eine Klassifikation der pädiatrischen diffusen Gliome anhand molekularpathologischer Tumorveränderungen neu erstellt. Wesentliche molekularpathologische Pfade sind Aktivierungen verschiedener Rezeptortyrosinkinasen und epigenetische Störungen der Translation durch Histon H3-Veränderungen.

Schlussfolgerung

Zunehmend besser verstandene Mechanismen der Entstehung pädiatrischer Tumore lassen auf mögliche spezifischere Therapieansätze hoffen.

Abstract

Background

Already with the update of the 4th edition of the World Health Organization (WHO) classification of tumors of the central nervous system, it was pointed out that pediatric diffuse glioma do not follow the same molecular mechanisms used to characterize adult diffuse glioma.

Objectives

What changes result from the update of the classification of tumors of the central nervous system?

Methods

With the 5th edition of the WHO classification of tumors of the central nervous system, a second level of information containing molecular changes besides the histological characterization and grading of tumors was established.

Results

A new classification of diffuse pediatric brain tumors based on molecular tumor pathways was established. The most important tumor pathways, considered for the new classification, were the activation of receptor tyrosine kinases and histone H3 alterations that cause epigenetic changes.

Conclusions

Increasingly better understanding of mechanisms in the development of pediatric brain tumors gives hope for more specific therapeutic approaches.

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Authors and Affiliations

  1. Institut für Neuropathologie, Medizinische Fakultät, Universität des Saarlandes und Universitätsklinikum des Saarlandes, Kirrberger Str. 100, Gebäude 90.3, 66421, Homburg, Deutschland

    Bernardo Reyes Medina, Arne Wrede &  Walter J. Schulz-Schaeffer

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  1. Bernardo Reyes Medina
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  2. Arne Wrede
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  3. Walter J. Schulz-Schaeffer
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Corresponding author

Correspondence to Walter J. Schulz-Schaeffer.

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Interessenkonflikt

B. Reyes Medina, A. Wrede und W.J. Schulz-Schaeffer geben an, dass kein Interessenkonflikt besteht.

Für diesen Beitrag wurden von den Autor/-innen keine Studien an Menschen oder Tieren durchgeführt. Für die aufgeführten Studien gelten die jeweils dort angegebenen ethischen Richtlinien.

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Reyes Medina, B., Wrede, A. & Schulz-Schaeffer, W.J. Neuropathologie pädiatrischer Hirntumore. Radiologie 63, 577–582 (2023). https://doi.org/10.1007/s00117-023-01171-2

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