% IMPORTANT: The following is UTF-8 encoded. This means that in the presence % of non-ASCII characters, it will not work with BibTeX 0.99 or older. % Instead, you should use an up-to-date BibTeX implementation like “bibtex8” or % “biber”. @ARTICLE{ObrechtSturm:292426, author = {D. Obrecht-Sturm and M. Schoof and A. Eckhardt and M. Mynarek and M. R. Gilbert and K. Aldape and T. S. Armstrong and V. Ramaswamy and M. Bockmayr and K. von Hoff and G. Fleischhack and J. E. Adolph and S. Tippelt and S. Pfister$^*$ and K. Pajtler$^*$ and D. Sturm$^*$ and R. Drexler and F. L. Ricklefs and N. Stepien and J. Gojo and T. Pietsch and M. Warmuth-Metz and R. Kortmann and B. Timmermann and C. Haberler and S. Rutkowski and U. Schüller}, title = {{D}istinct relapse pattern across molecular ependymoma types.}, journal = {Neuro-Oncology}, volume = {27}, number = {1}, issn = {1522-8517}, address = {Oxford}, publisher = {Oxford Univ. Press}, reportid = {DKFZ-2024-01709}, pages = {267–276}, year = {2025}, note = {Volume 27, Issue 1, January 2025, Pages 267–276}, abstract = {Ependymoma (EPN) is not a uniform disease but represents different disease types with biological and clinical heterogeneity. However, the pattern of when and where different types of EPN relapse is not yet comprehensively described.We assembled 269 relapsed intracranial EPN from pediatric (n=233) and adult (n=36) patients from European and Northern American cohorts and correlated DNA methylation patterns and copy-number alterations with clinical information.The cohort comprised the following molecular EPN types: PF-EPN-A (n=177), ST-EPN-ZFTA (n=45), PF-EPN-B (n=31), PF-EPN-SE (n=12), and ST-EPN-YAP (n=4). First relapses of PF-EPN-B (PF: posterior-fossa) and PF-EPN-SE (SE: subependymoma) occurred later than of PF-EPN-A, ST-EPN-YAP (ST: supratentorial), or ST-EPN-ZFTA (median time to relapse: 4.3 and 6.0 years vs. 1.9/1.0/2.4 years; p<0.01). Metastatic or combined recurrences in PF-EPN-B and -A more often involved the spinal cord than in ST-EPN-ZFTA $(72.7\%$ and 40.0 vs. $12.5\%;$ p<0.01). No distant relapses were observed in ST-EPN-YAP (n=4) or PF-EPN-SE (n=12). Post-relapse survival (PRS) was poor for PF-EPN-A and ST-EPN-ZFTA (5-year PRS: $44.5±4.4/47.8±9.1\%),$ whereas PF-EPN-B and PF-EPN-SE displayed a 5-year PRS of $89.5±7.1/90.0±9.5\%$ (p=0.03). However, 10-year PRS for PF-EPN-B dropped to $45.8±17.3\%.$ Neither between radiation field and relapse pattern nor between radiation field and spinal involvement at relapse an impact was identified. Notably, all patients with relapsed ST-EPN-YAP did not receive upfront radiotherapy, but were successfully salvaged using irradiation at relapse.Relapse patterns of specific EPN types are different. Future clinical trials, treatment adaptions, duration of surveillance and diagnostics should be planned incorporating entity-specific relapse information.}, keywords = {ependymoma (Other) / metastatic (Other) / recurrence (Other) / relapse (Other)}, cin = {B360 / HD01}, ddc = {610}, cid = {I:(DE-He78)B360-20160331 / I:(DE-He78)HD01-20160331}, pnm = {312 - Funktionelle und strukturelle Genomforschung (POF4-312)}, pid = {G:(DE-HGF)POF4-312}, typ = {PUB:(DE-HGF)16}, pubmed = {pmid:39171767}, doi = {10.1093/neuonc/noae166}, url = {https://inrepo02.dkfz.de/record/292426}, }