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Obrecht, Denise; Mynarek, Martin; von Hoff, Katja; Witt, Hendrik; Pajtler, Kristian W; Juhnke, B Ole; Warmuth-Metz, Monika; Bison, Brigitte; Kortmann, Rolf-Dieter; Timmermann, Beate; Pfister, Stefan M; Sahm, Felix; Sturm, Dominik; von Deimling, Andreas; Schüller, Ulrich; Pietsch, Torsten; Benesch, Martin; Gerber, Nicolas U; Rutkowski, Stefan

EPEN-09. IMPACT OF MOLECULAR SUBGROUP ON OUTCOME FOR INFANTS <12 MONTHS WITH INTRACRANIAL EPENDYMOMA - GERMAN EXPERIENCE FROM HIT2000, INTERIM-2000-REGISTRY AND I-HIT-MED REGISTRY

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  • Medientyp: E-Artikel
  • Titel: EPEN-09. IMPACT OF MOLECULAR SUBGROUP ON OUTCOME FOR INFANTS <12 MONTHS WITH INTRACRANIAL EPENDYMOMA - GERMAN EXPERIENCE FROM HIT2000, INTERIM-2000-REGISTRY AND I-HIT-MED REGISTRY
  • Beteiligte: Obrecht, Denise; Mynarek, Martin; von Hoff, Katja; Witt, Hendrik; Pajtler, Kristian W; Juhnke, B Ole; Warmuth-Metz, Monika; Bison, Brigitte; Kortmann, Rolf-Dieter; Timmermann, Beate; Pfister, Stefan M; Sahm, Felix; Sturm, Dominik; von Deimling, Andreas; Schüller, Ulrich; Pietsch, Torsten; Benesch, Martin; Gerber, Nicolas U; Rutkowski, Stefan
  • Erschienen: Oxford University Press (OUP), 2020
  • Erschienen in: Neuro-Oncology
  • Sprache: Englisch
  • DOI: 10.1093/neuonc/noaa222.149
  • ISSN: 1522-8517; 1523-5866
  • Schlagwörter: Cancer Research ; Neurology (clinical) ; Oncology
  • Entstehung:
  • Anmerkungen:
  • Beschreibung: <jats:title>Abstract</jats:title> <jats:sec> <jats:title>BACKGROUND</jats:title> <jats:p>For infant ependymoma (EP), decision for radiotherapy during first-line therapy is a dilemma. We analyzed therapy outcomes of EP patients younger than 12 months at diagnosis according to molecular subgroup.</jats:p> </jats:sec> <jats:sec> <jats:title>PATIENTS AND METHODS</jats:title> <jats:p>Between 2001 and 2017, 30 patients with histological diagnosis of intracranial EP &amp;lt;12 months at diagnosis with DNA-methylation profiling available were registered in HIT-MED-studies/-registries.</jats:p> </jats:sec> <jats:sec> <jats:title>RESULTS</jats:title> <jats:p>In 3/30, DNA methylation-based CNS tumor classification suggested a diagnosis other than EP or could not be assigned to a reference class. Of the remaining 27 tumors, 16 were classified as PF-A, 8 as RELA-fusion positive and 3 as YAP-fusion positive. Median age at diagnosis was 0.73 (0.30–0.99) years. After a median follow-up time of 5.36 (0.20–12.90) years, 59.3% experienced progressive disease (PD). 5y-PFS and -OS for the whole cohort were 38.2% and 73.1%. RELA- and YAP-fusion positive EP had significantly better OS than PF-A (5y-OS for PF-A: 55.9%; RELA 100%; YAP 100%; p=0.023). PFS was not significantly different. All but one patient with relapsed PF-A died despite multimodal salvage strategies. In contrast, patients with relapsing RELA- and YAP-fusion positive EP (n=5), survived with a combination of re-surgery and first or second local radiotherapy.</jats:p> </jats:sec> <jats:sec> <jats:title>CONCLUSION</jats:title> <jats:p>In this cohort of infants &amp;lt;12 months, patients with PF-A had a significantly inferior OS compared to patients with RELA- and YAP-fusion positive EP. Salvage therapy was ineffective for patients with PF-A, whereas patients with can RELA- and YAP-fusion positive EP can be long-term survivors after PD. Therefore, subgroups-specific therapy should be discussed.</jats:p> </jats:sec>
  • Zugangsstatus: Freier Zugang