Local and systemic therapy of recurrent ependymoma in children and adolescents

• Verfasst von: Adolph, Jonas E. [VerfasserIn]
• Verfasst von: Fleischhack, Gudrun [VerfasserIn]
• Verfasst von: Mikasch, Ruth [VerfasserIn]
• Verfasst von: Zeller, Julia [VerfasserIn]
• Verfasst von: Warmuth-Metz, Monika [VerfasserIn]
• Verfasst von: Bison, Brigitte [VerfasserIn]
• Verfasst von: Mynarek, Martin [VerfasserIn]
• Verfasst von: Rutkowski, Stefan [VerfasserIn]
• Verfasst von: Schüller, Ulrich [VerfasserIn]
• Verfasst von: von Hoff, Katja [VerfasserIn]
• Verfasst von: Obrecht, Denise [VerfasserIn]
• Verfasst von: Pietsch, Torsten [VerfasserIn]
• Verfasst von: Pfister, Stefan [VerfasserIn]
• Verfasst von: Pajtler, Kristian Wilfried [VerfasserIn]
• Verfasst von: Witt, Olaf [VerfasserIn]
• Verfasst von: Witt, Hendrik [VerfasserIn]
• Verfasst von: Kortmann, Rolf-Dieter [VerfasserIn]
• Verfasst von: Timmermann, Beate [VerfasserIn]
• Verfasst von: Krauß, Jürgen [VerfasserIn]
• Verfasst von: Frühwald, Michael C [VerfasserIn]
• Verfasst von: Faldum, Andreas [VerfasserIn]
• Verfasst von: Kwiecien, Robert [VerfasserIn]
• Verfasst von: Bode, Udo [VerfasserIn]
• Verfasst von: Tippelt, Stephan [VerfasserIn]
• Titel: Local and systemic therapy of recurrent ependymoma in children and adolescents
• Titelzusatz: short- and long-term results of the E-HIT-REZ 2005 study
• Verf.angabe: Jonas E. Adolph, Gudrun Fleischhack, Ruth Mikasch, Julia Zeller, Monika Warmuth-Metz, Brigitte Bison, Martin Mynarek, Stefan Rutkowski, Ulrich Schüller, Katja von Hoff, Denise Obrecht, Torsten Pietsch, Stefan M. Pfister, Kristian W. Pajtler, Olaf Witt, Hendrik Witt, Rolf-Dieter Kortmann, Beate Timmermann, Jürgen Krauß, Michael C. Frühwald, Andreas Faldum, Robert Kwiecien, Udo Bode, Stephan Tippelt; for the German GPOH HIT-Network
• Jahr: 2021
• Umfang: 12 S.
• Fussnoten: Advance Access date 17 December 2020 ; Gesehen am 25.11.2021
• Titel Quelle: Enthalten in: Neuro-Oncology
• Ort Quelle: Oxford : Oxford Univ. Press, 1999
• Jahr Quelle: 2021
• Band/Heft Quelle: 23(2021), 6, Seite 1012-1023
• ISSN Quelle: 1523-5866
• DOI: doi:10.1093/neuonc/noaa276
• Datenträger: Online-Ressource
• Sprache: eng
• K10plus-PPN: 1779059175

Abstract

Survival in recurrent ependymomas in children and adolescents mainly depends on the extent of resection. Studies on repeated radiotherapy and chemotherapy at relapse have shown conflicting results.Using data from the German multi-center E-HIT-REZ-2005 study, we examined the role of local therapy and the efficacy of chemotherapy with blockwise temozolomide (TMZ) in children and adolescents with recurrent ependymomas.Fifty-three patients with a median age of 6.9 years (1.25-25.4) at first recurrence and a median follow-up time of 36 months (2-115) were recruited. Gross- and near-total resection (GTR/NTR) were achieved in 34 (64.2%) patients and associated with a markedly improved 5-year overall survival (OS) of 48.7% vs. 5.3% in less than GTR/NTR. Radiotherapy showed no improvement in OS following complete resection (OS: 70 (CI: 19.9-120.1) vs. 95 (CI: 20.7-169.4) months), but an advantage was found in less than GTR/NTR (OS: 22 (CI: 12.7-31.3) vs. 7 (CI: 0-15.8) months). Following the application of TMZ, disease progression was observed in most evaluable cases (18/21). A subsequent change to oral etoposide and trofosfamide showed no improved response. PF-A EPN were most abundant in relapses (n = 27). RELA-positive EPN (n = 5) had a 5-year OS of 0%.The extent of resection is the most important predictor of survival at relapse. Focal re-irradiation is a useful approach if complete resection cannot be achieved, but no additional benefit was seen after GTR/NTR. Longer-term disease stabilization (>6 months) mediated by TMZ occurred in a small number of cases (14.3%).