Journal Article

DKFZ-2020-02949

http://join2-wiki.gsi.de/foswiki/pub/Main/Artwork/join2_logo100x88.png Local and Systemic Therapy of Recurrent Ependymoma in Children and Adolescents: Short- and Long-term Results of the E-HIT-REZ 2005 Study.

Adolph, J. E. ; Fleischhack, G. ; Mikasch, R. ; Zeller, J. ; Warmuth-Metz, M. ; Bison, B. ; Mynarek, M. ; Rutkowski, S. ; Schüller, U. ; von Hoff, K. ; Obrecht, D. ; Pietsch, T. ; Pfister, S. M.DKFZ* ; Pajtler, K. W.DKFZ* ; Witt, O.DKFZ* ; Witt, H.DKFZ* ; Kortmann, R.-D. ; Timmermann, B. ; Krauß, J. ; Frühwald, M. C. ; Faldum, A. ; Kwiecien, R. ; Bode, U. ; Tippelt, S. ; HIT-Network, G. G. (Collaboration Author)

2021
Oxford Univ. Press Oxford

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Please use a persistent id in citations: doi:10.1093/neuonc/noaa276

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.53 patients with a median age of 6.9 years (1.25-25.4) at 1 st 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 application of TMZ, disease progression was observed in most evaluable cases (18/21). 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%).

Keyword(s): chemotherapy ; ependymoma ; radiotherapy ; relapse ; tumor resection

Note: 2021 Jun 1;23(6):1012-1023

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Contributing Institute(s):

1. B062 Pädiatrische Neuroonkologie (B062)
2. KKE Pädiatrische Onkologie (B310)

Research Program(s):

1. 312 - Funktionelle und strukturelle Genomforschung (POF4-312) (POF4-312)

Appears in the scientific report 2021

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Database coverage:
; Clarivate Analytics Master Journal List ; Current Contents - Clinical Medicine ; Essential Science Indicators ; IF >= 10 ; JCR ; Nationallizenz ; PubMed Central ; SCOPUS ; Science Citation Index Expanded ; Web of Science Core Collection

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