{"doi":"10.1093/neuonc/noae166","title":"Distinct relapse pattern across molecular ependymoma types","abstract":"BACKGROUND: 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. METHODS: 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. RESULTS: 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 < .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 < .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 = .03). However, 10-year PRS for PF-EPN-B dropped to 45.8% ± 17.3%. Neither between the radiation field and relapse pattern nor between the 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. CONCLUSIONS: Relapse patterns of specific EPN types are different. Future clinical trials, treatment adaptions, duration of surveillance, and diagnostics should be planned to incorporate entity-specific relapse information.","journal":"Neuro-Oncology","year":2024,"id":445349,"datarank":0.31191623125197543,"base_score":2.0794415416798357,"endowment":2.0794415416798357,"self_citation_contribution":0.31191623125197543,"citation_network_contribution":0.0,"self_endowment_contribution":0.31191623125197543,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":7,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.6034,"is_data_producer":false,"deposit_databanks":null,"is_oa":true,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2024-01-01","fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":1261273,"name":"Melanie Schoof","orcid":"0000-0002-1373-8130","position":1,"is_corresponding":false},{"id":1261775,"name":"Alicia Eckhardt","orcid":null,"position":2,"is_corresponding":false},{"id":607796,"name":"Martin Mynarek","orcid":"0000-0003-3302-2719","position":3,"is_corresponding":false},{"id":109755,"name":"Mark R. Gilbert","orcid":"0000-0003-2556-9722","position":4,"is_corresponding":false},{"id":13200,"name":"Kenneth Aldape","orcid":"0000-0001-5119-7550","position":5,"is_corresponding":false},{"id":292546,"name":"Terri S. Armstrong","orcid":"0000-0002-2414-0492","position":6,"is_corresponding":false},{"id":240488,"name":"Vijay Ramaswamy","orcid":"0000-0002-6557-895X","position":7,"is_corresponding":false},{"id":1261274,"name":"Michael Bockmayr","orcid":"0000-0002-9249-4292","position":8,"is_corresponding":false},{"id":552511,"name":"Katja von Hoff","orcid":"0000-0002-5669-8546","position":9,"is_corresponding":false},{"id":552513,"name":"Gudrun Fleischhack","orcid":"0000-0001-5714-007X","position":10,"is_corresponding":false},{"id":1261275,"name":"Jonas E Adolph","orcid":"0000-0003-4446-8257","position":11,"is_corresponding":false},{"id":662197,"name":"Stephan Tippelt","orcid":"0000-0001-8791-9914","position":12,"is_corresponding":false},{"id":5562,"name":"Stefan M. Pfister","orcid":"0000-0002-5447-5322","position":13,"is_corresponding":false},{"id":25334,"name":"Kristian W. Pajtler","orcid":"0000-0002-3562-6121","position":14,"is_corresponding":false},{"id":236626,"name":"Dominik Sturm","orcid":"0000-0003-0250-1696","position":15,"is_corresponding":false},{"id":1010912,"name":"Richard Drexler","orcid":"0000-0002-4830-3671","position":16,"is_corresponding":false},{"id":459309,"name":"Franz Ricklefs","orcid":"0000-0002-1321-6930","position":17,"is_corresponding":false},{"id":1261276,"name":"Natalia Stepien","orcid":"0000-0002-6658-8367","position":18,"is_corresponding":false},{"id":5535,"name":"Johannes Gojo","orcid":"0000-0002-8113-3416","position":19,"is_corresponding":false},{"id":236623,"name":"Torsten Pietsch","orcid":"0000-0003-0763-6506","position":20,"is_corresponding":false},{"id":1261277,"name":"Monika Warmuth‐Metz","orcid":"0000-0002-3544-319X","position":21,"is_corresponding":false},{"id":1261776,"name":"Rolf D. Kortmann","orcid":null,"position":22,"is_corresponding":false},{"id":620549,"name":"Beate Timmermann","orcid":"0000-0003-2274-2008","position":23,"is_corresponding":false},{"id":5540,"name":"Christine Haberler","orcid":"0000-0003-1016-0545","position":24,"is_corresponding":false},{"id":1061845,"name":"Stefan Rutkowski","orcid":"0000-0002-5446-9571","position":25,"is_corresponding":false},{"id":236593,"name":"Ulrich Schüller","orcid":"0000-0002-8731-1121","position":26,"is_corresponding":false},{"id":1061832,"name":"Denise Obrecht","orcid":"0000-0002-3216-8452","position":0,"is_corresponding":true}],"reference_count":31,"raw_metadata":{"citation_network_status":"fetched"},"created_at":"2026-07-19T02:01:42.089235Z","pmid":"39171767","pmcid":null,"fwci":null,"citation_percentile":null,"influential_citations":0,"oa_status":null,"license":null,"views":0,"total_file_size_bytes":0,"version_count":0,"fair_f":null,"fair_a":null,"fair_i":null,"fair_r":null,"fair_zscore":null,"fair_rationale":null,"fair_model":null,"fair_agent_version":null,"fair_fulltext_source":null,"fair_has_llm":null,"fair_computed_at":null,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}