Local and systemic therapy of recurrent ependymoma in children and adolescents: short- and long-term result of the E-HIT-REZ 2005 study
| Autor: | Jonas E. Adolph, Denise Obrecht, Martin Mynarek, Julia Zeller, Katja von Hoff, Andreas Faldum, Monika Warmuth-Metz, Beate Timmermann, Stephan Tippelt, Torsten Pietsch, Robert Kwiecien, Michael C. Frühwald, Stefan M. Pfister, Udo Bode, Jürgen Kraus, Stefan Rutkowski, Ruth Mikasch, Rolf-Dieter Kortmann, Ulrich Schüller, Olaf Witt, Gudrun Fleischhack, Kristian W. Pajtler, Hendrik Witt, Brigitte Bison |
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| Jazyk: | angličtina |
| Rok vydání: | 2021 |
| Předmět: |
Re-Irradiation
Ependymoma Cancer Research medicine.medical_specialty Chemotherapy Temozolomide business.industry medicine.medical_treatment Medizin medicine.disease Chemotherapy regimen Systemic therapy Gastroenterology Trofosfamide Radiation therapy chemistry.chemical_compound Oncology chemistry Internal medicine Medicine Neurology (clinical) business medicine.drug |
| Popis: | Background 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. Methods 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. Results 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%. Conclusion 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%). |
| Databáze: | OpenAIRE |
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