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SHR Neuro Krebs Kardio Lipid Stoffw Microb

Ng, CH; Obrecht, D; Wells, O; Zapotocky, M; Sumerauer, D; Coltin, H; Khuong-Quang, DA; Eisenstat, DD; Kinross, KM; White, CL; Algar, EM; Luck, A; Witt, H; Schueller, U; Mynarek, M; Pietsch, T; Gerber, NU; Benesch, M; Warmuth-Metz, M; Kortmann, R; Bison, B; Taylor, MD; Rutkowski, S; Pfister, SM; Jones, DT; Gottardo, NG; von Hoff, K; Pajtler, KW; Ramaswamy, V; Hansford, JR.
A multi-institutional retrospective pooled outcome analysis of molecularly annotated pediatric supratentorial ZFTA-fused ependymoma
NEURO-ONCOL ADV. 2023; 5(1): vdad057 Doi: 10.1093/noajnl/vdad057 [OPEN ACCESS]
Web of Science PubMed FullText FullText_MUG

Co-Autor*innen der Med Uni Graz: Benesch Martin
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Abstract:: Background ZFTA-RELA (formerly known as c11orf-RELA) fused supratentorial ependymoma (ZFTAfus ST-EPN) has been recognized as a novel entity in the 2016 WHO classification of CNS tumors and further defined in the recent 2021 edition. ZFTAfus ST-EPN was reported to portend poorer prognosis when compared to its counterpart, YAP1 ST-EPN in some previously published series. The aim of this study was to determine the treatment outcome of molecularly confirmed and conventionally treated ZFTAfus ST-EPN patients treated in multiple institutions. Methods We conducted a retrospective analysis of all pediatric patients with molecularly confirmed ZFTAfus ST-EPN patients treated in multiple institutions in 5 different countries (Australia, Canada, Germany, Switzerland, and Czechia). Survival outcomes were analyzed and correlated with clinical characteristics and treatment approaches. Results A total of 108 patients were collated from multiple institutions in 5 different countries across three continents. We found across the entire cohort that the 5- and 10-year PFS were 65% and 63%, respectively. The 5- and 10-year OS of this cohort of patients were 87% and 73%. The rates of gross total resection (GTR) were high with 84 out of 108 (77.8%) patients achieving GTR. The vast majority of patients also received post-operative radiotherapy, 98 out of 108 (90.7%). Chemotherapy did not appear to provide any survival benefit in our patient cohort. Conclusion This is the largest study to date of contemporaneously treated molecularly confirmed ZFTAfus ST-EPN patients which identified markedly improved survival outcomes compared to previously published series. This study also re-emphasizes the importance of maximal surgical resection in achieving optimal outcomes in pediatric patients with supratentorial ependymoma.
Find related publications in this database (Keywords): central nervous system tumors; molecular; pediatric; supratentorial ependymoma; ZFTA-RELA