000162800 001__ 162800
000162800 005__ 20230915092407.0
000162800 0247_ $$2doi$$a10.1093/neuonc/noab136
000162800 0247_ $$2pmid$$apmid:34077956
000162800 0247_ $$2pmc$$apmc:PMC8408859
000162800 0247_ $$2ISSN$$a1522-8517
000162800 0247_ $$2ISSN$$a1523-5866
000162800 0247_ $$2altmetric$$aaltmetric:106956292
000162800 037__ $$aDZNE-2021-01455
000162800 041__ $$aEnglish
000162800 082__ $$a610
000162800 1001_ $$00000-0002-5669-8546$$avon Hoff, Katja$$b0
000162800 245__ $$aTherapeutic implications of improved molecular diagnostics for rare CNS embryonal tumor entities: results of an international, retrospective study.
000162800 260__ $$aOxford$$bOxford Univ. Press$$c2021
000162800 3367_ $$2DRIVER$$aarticle
000162800 3367_ $$2DataCite$$aOutput Types/Journal article
000162800 3367_ $$0PUB:(DE-HGF)16$$2PUB:(DE-HGF)$$aJournal Article$$bjournal$$mjournal$$s1637585218_15706
000162800 3367_ $$2BibTeX$$aARTICLE
000162800 3367_ $$2ORCID$$aJOURNAL_ARTICLE
000162800 3367_ $$00$$2EndNote$$aJournal Article
000162800 520__ $$aOnly few data are available on treatment-associated behavior of distinct rare CNS embryonal tumor entities previously treated as 'CNS-primitive neuroectodermal tumors' (CNS-PNET). Respective data on specific entities, including CNS neuroblastoma, FOXR2 activated (CNS NB-FOXR2), and embryonal tumors with multilayered rosettes (ETMR) are needed for development of differentiated treatment strategies.Within this retrospective, international study, tumor samples of clinically well-annotated patients with the original diagnosis of CNS-PNET were analyzed using DNA methylation arrays (n = 307). Additional cases (n = 66) with DNA methylation pattern of CNS NB-FOXR2 were included irrespective of initial histological diagnosis. Pooled clinical data (n = 292) were descriptively analyzed.DNA methylation profiling of 'CNS-PNET' classified 58 (19%) cases as ETMR, 57 (19%) as high-grade glioma (HGG), 36 (12%) as CNS NB-FOXR2, and 89(29%) cases were classified into 18 other entities. Sixty-seven (22%) cases did not show DNA methylation patterns similar to established CNS tumor reference classes. Best treatment results were achieved for CNS NB-FOXR2 patients (5-year PFS: 63% ± 7%, OS: 85% ± 5%, n = 63), with 35/42 progression-free survivors after upfront craniospinal irradiation (CSI) and chemotherapy. The worst outcome was seen for ETMR and HGG patients with 5-year PFS of 18% ± 6% and 22% ± 7%, and 5-year OS of 24% ± 6% and 25% ± 7%, respectively.The historically reported poor outcome of CNS-PNET patients becomes highly variable when tumors are molecularly classified based on DNA methylation profiling. Patients with CNS NB-FOXR2 responded well to current treatments and a standard-risk CSI-based regimen may be prospectively evaluated. The poor outcome of ETMR across applied treatment strategies substantiates the necessity for evaluation of novel treatments.
000162800 536__ $$0G:(DE-HGF)POF4-899$$a899 - ohne Topic (POF4-899)$$cPOF4-899$$fPOF IV$$x0
000162800 588__ $$aDataset connected to CrossRef, PubMed, , Journals: pub.dzne.de
000162800 650_7 $$2Other$$aCNS NB-FOXR2
000162800 650_7 $$2Other$$aCNS embryonal tumor
000162800 650_7 $$2Other$$aCNS-PNET
000162800 650_7 $$2Other$$aDNA methylation profiling
000162800 650_7 $$2Other$$aETMR
000162800 650_7 $$2NLM Chemicals$$aFOXR2 protein, human
000162800 650_7 $$2NLM Chemicals$$aForkhead Transcription Factors
000162800 650_2 $$2MeSH$$aBrain Neoplasms: diagnosis
000162800 650_2 $$2MeSH$$aBrain Neoplasms: genetics
000162800 650_2 $$2MeSH$$aBrain Neoplasms: therapy
000162800 650_2 $$2MeSH$$aCentral Nervous System Neoplasms: diagnosis
000162800 650_2 $$2MeSH$$aCentral Nervous System Neoplasms: genetics
000162800 650_2 $$2MeSH$$aCentral Nervous System Neoplasms: therapy
000162800 650_2 $$2MeSH$$aForkhead Transcription Factors
000162800 650_2 $$2MeSH$$aHumans
000162800 650_2 $$2MeSH$$aNeoplasms, Germ Cell and Embryonal: diagnosis
000162800 650_2 $$2MeSH$$aNeoplasms, Germ Cell and Embryonal: genetics
000162800 650_2 $$2MeSH$$aNeoplasms, Germ Cell and Embryonal: therapy
000162800 650_2 $$2MeSH$$aNeuroectodermal Tumors, Primitive: diagnosis
000162800 650_2 $$2MeSH$$aNeuroectodermal Tumors, Primitive: genetics
000162800 650_2 $$2MeSH$$aNeuroectodermal Tumors, Primitive: therapy
000162800 650_2 $$2MeSH$$aPathology, Molecular
000162800 650_2 $$2MeSH$$aRetrospective Studies
000162800 7001_ $$aHaberler, Christine$$b1
000162800 7001_ $$aSchmitt-Hoffner, Felix$$b2
000162800 7001_ $$aSchepke, Elizabeth$$b3
000162800 7001_ $$ade Rojas, Teresa$$b4
000162800 7001_ $$aJacobs, Sandra$$b5
000162800 7001_ $$aZapotocky, Michal$$b6
000162800 7001_ $$aSumerauer, David$$b7
000162800 7001_ $$aPerek-Polnik, Marta$$b8
000162800 7001_ $$00000-0001-5993-8077$$aDufour, Christelle$$b9
000162800 7001_ $$avan Vuurden, Dannis$$b10
000162800 7001_ $$aSlavc, Irene$$b11
000162800 7001_ $$aGojo, Johannes$$b12
000162800 7001_ $$aPickles, Jessica C$$b13
000162800 7001_ $$aGerber, Nicolas U$$b14
000162800 7001_ $$aMassimino, Maura$$b15
000162800 7001_ $$aGil-da-Costa, Maria Joao$$b16
000162800 7001_ $$aGarami, Miklos$$b17
000162800 7001_ $$aKumirova, Ella$$b18
000162800 7001_ $$aSehested, Astrid$$b19
000162800 7001_ $$00000-0002-9772-8828$$aScheie, David$$b20
000162800 7001_ $$aCruz, Ofelia$$b21
000162800 7001_ $$aMoreno, Lucas$$b22
000162800 7001_ $$aCho, Jaeho$$b23
000162800 7001_ $$aZeller, Bernward$$b24
000162800 7001_ $$aBovenschen, Niels$$b25
000162800 7001_ $$aGrotzer, Michael$$b26
000162800 7001_ $$aAlderete, Daniel$$b27
000162800 7001_ $$aSnuderl, Matija$$b28
000162800 7001_ $$aZheludkova, Olga$$b29
000162800 7001_ $$aGolanov, Andrey$$b30
000162800 7001_ $$aOkonechnikov, Konstantin$$b31
000162800 7001_ $$aMynarek, Martin$$b32
000162800 7001_ $$aJuhnke, Björn Ole$$b33
000162800 7001_ $$aRutkowski, Stefan$$b34
000162800 7001_ $$aSchüller, Ulrich$$b35
000162800 7001_ $$aPizer, Barry$$b36
000162800 7001_ $$avon Zezschwitz, Barbara$$b37
000162800 7001_ $$aKwiecien, Robert$$b38
000162800 7001_ $$aWechsung, Maximilian$$b39
000162800 7001_ $$aKonietschke, Frank$$b40
000162800 7001_ $$aHwang, Eugene I$$b41
000162800 7001_ $$aSturm, Dominik$$b42
000162800 7001_ $$aPfister, Stefan M$$b43
000162800 7001_ $$avon Deimling, Andreas$$b44
000162800 7001_ $$aRushing, Elisabeth J$$b45
000162800 7001_ $$aRyzhova, Marina$$b46
000162800 7001_ $$aHauser, Peter$$b47
000162800 7001_ $$aŁastowska, Maria$$b48
000162800 7001_ $$00000-0001-5453-5201$$aWesseling, Pieter$$b49
000162800 7001_ $$aGiangaspero, Felice$$b50
000162800 7001_ $$aHawkins, Cynthia$$b51
000162800 7001_ $$aFigarella-Branger, Dominique$$b52
000162800 7001_ $$aEberhart, Charles$$b53
000162800 7001_ $$aBurger, Peter$$b54
000162800 7001_ $$aGessi, Marco$$b55
000162800 7001_ $$aKorshunov, Andrey$$b56
000162800 7001_ $$aJacques, Tom S$$b57
000162800 7001_ $$aCapper, David$$b58
000162800 7001_ $$0P:(DE-2719)2812617$$aPietsch, Torsten$$b59$$udzne
000162800 7001_ $$aKool, Marcel$$b60
000162800 773__ $$0PERI:(DE-600)2094060-9$$a10.1093/neuonc/noab136$$gVol. 23, no. 9, p. 1597 - 1611$$n9$$p1597 - 1611$$tNeuro-Oncology$$v23$$x1523-5866$$y2021
000162800 909CO $$ooai:pub.dzne.de:162800$$pVDB
000162800 9101_ $$0I:(DE-588)1065079516$$6P:(DE-2719)2812617$$aDeutsches Zentrum für Neurodegenerative Erkrankungen$$b59$$kDZNE
000162800 9131_ $$0G:(DE-HGF)POF4-899$$1G:(DE-HGF)POF4-890$$2G:(DE-HGF)POF4-800$$3G:(DE-HGF)POF4$$4G:(DE-HGF)POF$$aDE-HGF$$bProgrammungebundene Forschung$$lohne Programm$$vohne Topic$$x0
000162800 9141_ $$y2021
000162800 915__ $$0StatID:(DE-HGF)0160$$2StatID$$aDBCoverage$$bEssential Science Indicators$$d2021-02-03
000162800 915__ $$0StatID:(DE-HGF)0113$$2StatID$$aWoS$$bScience Citation Index Expanded$$d2021-02-03
000162800 915__ $$0StatID:(DE-HGF)0420$$2StatID$$aNationallizenz$$d2022-11-08$$wger
000162800 915__ $$0StatID:(DE-HGF)0300$$2StatID$$aDBCoverage$$bMedline$$d2022-11-08
000162800 915__ $$0StatID:(DE-HGF)0199$$2StatID$$aDBCoverage$$bClarivate Analytics Master Journal List$$d2022-11-08
000162800 915__ $$0StatID:(DE-HGF)0150$$2StatID$$aDBCoverage$$bWeb of Science Core Collection$$d2022-11-08
000162800 915__ $$0StatID:(DE-HGF)1110$$2StatID$$aDBCoverage$$bCurrent Contents - Clinical Medicine$$d2022-11-08
000162800 915__ $$0StatID:(DE-HGF)0100$$2StatID$$aJCR$$bNEURO-ONCOLOGY : 2021$$d2022-11-08
000162800 915__ $$0StatID:(DE-HGF)0200$$2StatID$$aDBCoverage$$bSCOPUS$$d2022-11-08
000162800 915__ $$0StatID:(DE-HGF)9910$$2StatID$$aIF >= 10$$bNEURO-ONCOLOGY : 2021$$d2022-11-08
000162800 9201_ $$0I:(DE-2719)1011009$$kBrainbank Unit Bonn$$lBrainbank Unit Bonn$$x0
000162800 980__ $$ajournal
000162800 980__ $$aVDB
000162800 980__ $$aI:(DE-2719)1011009
000162800 980__ $$aUNRESTRICTED