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Ewing-Sarkom

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Pädiatrische Hämatologie und Onkologie

Part of the book series: Springer Reference Medizin ((SRM))

Zusammenfassung

Ewing-Sarkome sind die zweithäufigsten primären Knochentumoren im Kindes- und Jugendalter. Vordringlich ist eine rechtzeitige Diagnose. Fortbestehende lokalisierte (Knochen-)Schmerzen bei Kindern, Jugendlichen und jungen Erwachsenen müssen innerhalb von 4 Wochen differenzialdiagnostisch abgeklärt sein. Diagnostisch wegweisend ist der Nachweis der EWS-Fli1-Genfusion im Tumorgewebe. Eine intensive Kombinationschemotherapie mit mindestens 4 Medikamenten ist als Standard anzusehen. Den höchsten Stellenwert haben alkylierende Substanzen und Anthrazykline. Eine operative und/oder radiotherapeutische Lokaltherapie der Primärtumorregion ist unverzichtbar. Die Komplexität der Therapiestrategie erfordert die Therapie in spezialisierten Sarkomzentren. Mit multimodaler Therapie liegen die Heilungsraten bei lokalisierter Erkrankung >70 %. Die Prognose für Ewing-Sarkom-Patienten ist bestimmt durch die Größe des Primärtumors, den Metastasierungsstatus und das biologische Ansprechen auf initiale Chemotherapie. Die große Mehrzahl geheilter Ewing-Tumor-Patienten führt ein gesundes Leben ohne beeinträchtigende Spätfolgen.

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Authors and Affiliations

  1. Pädiatrische Hämatologie und Onkologie, Universitätsklinikum Münster, Albert-Schweitzer-Campus 1, 48149, Münster, Deutschland

    Prof. Dr. med. U. Dirksen

  2. Klinik f Pädiatrische Hämatologie u Onkologie, Universitätsklinikum Münster, Albert-Schweitzer-Campus 1, 48149, Münster, Deutschland

    Prof. Dr. med. H. Jürgens

Authors
  1. Prof. Dr. med. U. Dirksen
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  2. Prof. Dr. med. H. Jürgens
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Correspondence to U. Dirksen or H. Jürgens .

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Editors and Affiliations

  1. Universitätsklinik um Freiburg Zentrum für Kinder- und Jugendmedizin, Freiburg, Deutschland

    Charlotte Niemeyer

  2. Charité-Universitätsmedizin Berlin, Berlin, Deutschland

    Angelika Eggert

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Dirksen, U., Jürgens, H. (2018). Ewing-Sarkom. In: Niemeyer, C., Eggert, A. (eds) Pädiatrische Hämatologie und Onkologie. Springer Reference Medizin. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-43686-8_28

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  • DOI: https://doi.org/10.1007/978-3-662-43686-8_28

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-662-43685-1

  • Online ISBN: 978-3-662-43686-8

  • eBook Packages: Medicine (German Language)

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