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Maligne Rundzelltumoren

Das Ewing-Sarkom und jenseits davon

Malignant round cell tumors

The Ewing sarcoma and beyond

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Ewing-Sarkome stellen hochmaligne Tumoren dar, die hauptsächlich bei Kindern und Jugendlichen anzutreffen sind. Neben einer frühen klinischen Diagnostik stellt die korrekte histopathologische und molekulargenetische Klassifizierung den wichtigsten Schritt dar. Zwar stellt die EWSR1-FLI1-Fusion die mit Abstand häufigste nachweisbare Veränderung dar, aber es gibt auch weitere, histopathologisch und immunhistochemisch nicht von klassischen Ewing-Sarkomen zu unterscheidende Vertreter der Ewing-Sarkom-Familie, welche andere molekulargenetische Profile aufweisen. Aus einer genauen molekulargenetischen Differenzierung der verschiedenen Vertreter der klein-rund-blauzelligen Tumoren ergibt sich zum heutigen Zeitpunkt zwar noch keine Änderung der standardmäßig angewandten Chemotherapie und chirurgischen Versorgung, erlaubt aber eine Abschätzung der Prognose und wird in Zukunft mit hoher Wahrscheinlichkeit dazu beitragen, Ewing-Sarkome im Rahmen der personalisierten Medizin noch individueller zu therapieren.


Ewing sarcomas are highly malignant tumors that are mainly found in children and adolescents. In addition to early clinical diagnosis, correct histopathological and molecular genetic classification is the most important step. Although EWSR1-FLI1 fusion is by far the most common detectable change, there are also other representatives of the Ewing sarcoma family that cannot be distinguished histopathologically and immunohistochemically from classical Ewing sarcomas and that have different molecular genetic profiles. Although a precise molecular genetic differentiation of the various representatives of small round blue cell tumors does not yet lead to any change in the standard chemotherapy and surgical treatment applied, it does allow an estimation of the prognosis and will probably contribute in the future to an even more individualized treatment of Ewing sarcomas within the framework of personalized medicine.

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Correspondence to Prof. Dr. Dr. Johannes Haybaeck.

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J. Haybaeck und A. Roessner geben an, dass kein Interessenkonflikt besteht.

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

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A. Roessner, Magdeburg

J. Haybaek, Magdeburg

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Haybaeck, J., Roessner, A. Maligne Rundzelltumoren. Pathologe (2020). https://doi.org/10.1007/s00292-020-00759-y

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  • Ewing-Sarkom
  • Atypische Varianten
  • Molekulare Genetik
  • Neue Therapieansätze
  • Fusionsonkoprotein


  • Ewing sarcoma
  • Atypical variants
  • Molecular genetics
  • New therapy approaches
  • Fusion oncoprotein