Home > Publications database > SIOP Ependymoma I: Final results, long term follow-up and molecular analysis of the trial cohort: A BIOMECA Consortium Study. > print

001     178484
005     20240229143548.0
024 7 _ |a 10.1093/neuonc/noac012
|2 doi
024 7 _ |a pmid:35018471
|2 pmid
024 7 _ |a 1522-8517
|2 ISSN
024 7 _ |a 1523-5866
|2 ISSN
024 7 _ |a altmetric:120648421
|2 altmetric
037 _ _ |a DKFZ-2022-00102
041 _ _ |a English
082 _ _ |a 610
100 1 _ |a Ritzmann, Timothy A
|b 0
245 _ _ |a SIOP Ependymoma I: Final results, long term follow-up and molecular analysis of the trial cohort: A BIOMECA Consortium Study.
260 _ _ |a Oxford
|c 2022
|b Oxford Univ. Press
336 7 _ |a article
|2 DRIVER
336 7 _ |a Output Types/Journal article
|2 DataCite
336 7 _ |a Journal Article
|b journal
|m journal
|0 PUB:(DE-HGF)16
|s 1655974183_11776
|2 PUB:(DE-HGF)
336 7 _ |a ARTICLE
|2 BibTeX
336 7 _ |a JOURNAL_ARTICLE
|2 ORCID
336 7 _ |a Journal Article
|0 0
|2 EndNote
500 _ _ |a 2022 Jun 1;24(6):936-948
520 _ _ |a SIOP Ependymoma I was a non-randomised trial assessing event free and overall survival (EFS/OS) of non-metastatic intracranial ependymoma in children aged 3 to 21 years treated with a staged management strategy. A further aim was to assess the response rate (RR) of subtotally resected (STR) ependymoma to vincristine, etoposide and cyclophosphamide (VEC). We report final results with 12-year follow-up and post hoc analyses of recently described biomarkers.74 participants were eligible. Children with gross total resection (GTR) received radiotherapy, whilst those with STR received VEC before radiotherapy. DNA methylation, 1q, hTERT, ReLA, Tenascin-C, H3K27me3 and pAKT status were evaluated.Five- and ten-year EFS was 49.5% and 46.7%, OS was 69.3% and 60.5%. GTR was achieved in 33/74 (44.6%) and associated with improved EFS (p=0.003, HR=2.6, 95% confidence interval (CI) 1.4-5.1). Grade 3 tumours were associated with worse OS (p=0.005, HR=2.8, 95%CI 1.3-5.8). 1q gain and hTERT expression were associated with poorer EFS (p=0.003, HR=2.70, 95%CI 1.49-6.10 and p=0.014, HR=5.8, 95%CI 1.2-28) and H3K27me3 loss with worse OS (p=0.003, HR=4.6, 95%CI 1.5-13.2). Methylation profiles showed expected patterns. 12 participants with STR did not receive chemotherapy; a protocol violation. However, best chemotherapy RR was 65.5% (19/29, 95%CI 45.7-82.1), exceeding the prespecified 45%.Participants with totally resected ependymoma had the best outcomes. RR of STR to VEC exceeded the pre-specified efficacy criterion. However, cases of inaccurate stratification highlighted the need for rapid central review. 1q gain, H3K27me3 loss and hTERT expression were all associated with poorer survival outcomes.
536 _ _ |a 312 - Funktionelle und strukturelle Genomforschung (POF4-312)
|0 G:(DE-HGF)POF4-312
|c POF4-312
|f POF IV
|x 0
588 _ _ |a Dataset connected to CrossRef, PubMed, , Journals: inrepo01.inet.dkfz-heidelberg.de
650 _ 7 |a Chemotherapy
|2 Other
650 _ 7 |a Ependymoma
|2 Other
650 _ 7 |a Radiotherapy
|2 Other
650 _ 7 |a Recurrence
|2 Other
650 _ 7 |a Resection
|2 Other
700 1 _ |a Chapman, Rebecca J
|0 0000-0001-8050-8426
|b 1
700 1 _ |a Kilday, John-Paul
|0 0000-0002-0539-215X
|b 2
700 1 _ |a Thorp, Nicola
|b 3
700 1 _ |a Modena, Piergiorgio
|b 4
700 1 _ |a Dineen, Robert A
|0 0000-0002-9523-2546
|b 5
700 1 _ |a Macarthur, Donald
|b 6
700 1 _ |a Mallucci, Conor
|0 0000-0002-5509-0547
|b 7
700 1 _ |a Jaspan, Timothy
|b 8
700 1 _ |a Pajtler, Kristian
|0 P:(DE-He78)a7c1bbac024fa232d9c6b78443328d9d
|b 9
|u dkfz
700 1 _ |a Giagnacovo, Marzia
|b 10
700 1 _ |a Jacques, Thomas S
|b 11
700 1 _ |a Paine, Simon M L
|0 0000-0002-1434-3931
|b 12
700 1 _ |a Ellison, David W
|b 13
700 1 _ |a Bouffet, Eric
|0 0000-0002-6832-6539
|b 14
700 1 _ |a Grundy, Richard G
|0 0000-0002-2585-2539
|b 15
700 1 _ |a consortium, BIOMECA
|b 16
|e Collaboration Author
773 _ _ |a 10.1093/neuonc/noac012
|g p. noac012
|0 PERI:(DE-600)2094060-9
|n 6
|p 936-948
|t Neuro-Oncology
|v 24
|y 2022
|x 1522-8517
909 C O |p VDB
|o oai:inrepo02.dkfz.de:178484
910 1 _ |a Deutsches Krebsforschungszentrum
|0 I:(DE-588b)2036810-0
|k DKFZ
|b 9
|6 P:(DE-He78)a7c1bbac024fa232d9c6b78443328d9d
913 1 _ |a DE-HGF
|b Gesundheit
|l Krebsforschung
|1 G:(DE-HGF)POF4-310
|0 G:(DE-HGF)POF4-312
|3 G:(DE-HGF)POF4
|2 G:(DE-HGF)POF4-300
|4 G:(DE-HGF)POF
|v Funktionelle und strukturelle Genomforschung
|x 0
914 1 _ |y 2022
915 _ _ |a DBCoverage
|0 StatID:(DE-HGF)0160
|2 StatID
|b Essential Science Indicators
|d 2021-02-03
915 _ _ |a WoS
|0 StatID:(DE-HGF)0113
|2 StatID
|b Science Citation Index Expanded
|d 2021-02-03
915 _ _ |a Nationallizenz
|0 StatID:(DE-HGF)0420
|2 StatID
|d 2022-11-08
|w ger
915 _ _ |a DBCoverage
|0 StatID:(DE-HGF)0300
|2 StatID
|b Medline
|d 2022-11-08
915 _ _ |a DBCoverage
|0 StatID:(DE-HGF)0199
|2 StatID
|b Clarivate Analytics Master Journal List
|d 2022-11-08
915 _ _ |a DBCoverage
|0 StatID:(DE-HGF)0150
|2 StatID
|b Web of Science Core Collection
|d 2022-11-08
915 _ _ |a DBCoverage
|0 StatID:(DE-HGF)1110
|2 StatID
|b Current Contents - Clinical Medicine
|d 2022-11-08
915 _ _ |a JCR
|0 StatID:(DE-HGF)0100
|2 StatID
|b NEURO-ONCOLOGY : 2021
|d 2022-11-08
915 _ _ |a DBCoverage
|0 StatID:(DE-HGF)0200
|2 StatID
|b SCOPUS
|d 2022-11-08
915 _ _ |a IF >= 10
|0 StatID:(DE-HGF)9910
|2 StatID
|b NEURO-ONCOLOGY : 2021
|d 2022-11-08
920 1 _ |0 I:(DE-He78)B062-20160331
|k B062
|l B062 Pädiatrische Neuroonkologie
|x 0
920 1 _ |0 I:(DE-He78)HD01-20160331
|k HD01
|l DKTK HD zentral
|x 1
980 _ _ |a journal
980 _ _ |a VDB
980 _ _ |a I:(DE-He78)B062-20160331
980 _ _ |a I:(DE-He78)HD01-20160331
980 _ _ |a UNRESTRICTED

LibraryCollectionCLSMajorCLSMinorLanguageAuthor
Marc 21