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Therapeutic implications of improved molecular diagnostics for rare CNS embryonal tumor entities: results of an international, retrospective study

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dc.contributor.author조재호-
dc.date.accessioned2021-12-28T16:42:36Z-
dc.date.available2021-12-28T16:42:36Z-
dc.date.issued2021-09-
dc.identifier.issn1522-8517-
dc.identifier.urihttps://ir.ymlib.yonsei.ac.kr/handle/22282913/186764-
dc.description.abstractBackground: Only 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. Methods: 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. Results: 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. Conclusion: 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.-
dc.description.statementOfResponsibilityrestriction-
dc.languageEnglish-
dc.publisherOxford University Press-
dc.relation.isPartOfNEURO-ONCOLOGY-
dc.rightsCC BY-NC-ND 2.0 KR-
dc.subject.MESHBrain Neoplasms* / diagnosis-
dc.subject.MESHBrain Neoplasms* / genetics-
dc.subject.MESHBrain Neoplasms* / therapy-
dc.subject.MESHCentral Nervous System Neoplasms* / diagnosis-
dc.subject.MESHCentral Nervous System Neoplasms* / genetics-
dc.subject.MESHCentral Nervous System Neoplasms* / therapy-
dc.subject.MESHForkhead Transcription Factors-
dc.subject.MESHHumans-
dc.subject.MESHNeoplasms, Germ Cell and Embryonal* / diagnosis-
dc.subject.MESHNeoplasms, Germ Cell and Embryonal* / genetics-
dc.subject.MESHNeoplasms, Germ Cell and Embryonal* / therapy-
dc.subject.MESHNeuroectodermal Tumors, Primitive* / diagnosis-
dc.subject.MESHNeuroectodermal Tumors, Primitive* / genetics-
dc.subject.MESHNeuroectodermal Tumors, Primitive* / therapy-
dc.subject.MESHPathology, Molecular-
dc.subject.MESHRetrospective Studies-
dc.titleTherapeutic implications of improved molecular diagnostics for rare CNS embryonal tumor entities: results of an international, retrospective study-
dc.typeArticle-
dc.contributor.collegeCollege of Medicine (의과대학)-
dc.contributor.departmentDept. of Radiation Oncology (방사선종양학교실)-
dc.contributor.googleauthorKatja von Hoff-
dc.contributor.googleauthorChristine Haberler-
dc.contributor.googleauthorFelix Schmitt-Hoffner-
dc.contributor.googleauthorElizabeth Schepke-
dc.contributor.googleauthorTeresa de Rojas-
dc.contributor.googleauthorSandra Jacobs-
dc.contributor.googleauthorMichal Zapotocky-
dc.contributor.googleauthorDavid Sumerauer-
dc.contributor.googleauthorMarta Perek-Polnik-
dc.contributor.googleauthorChristelle Dufour-
dc.contributor.googleauthorDannis van Vuurden-
dc.contributor.googleauthorIrene Slavc-
dc.contributor.googleauthorJohannes Gojo-
dc.contributor.googleauthorJessica C Pickles-
dc.contributor.googleauthorNicolas U Gerber-
dc.contributor.googleauthorMaura Massimino-
dc.contributor.googleauthorMaria Joao Gil-da-Costa-
dc.contributor.googleauthorMiklos Garami-
dc.contributor.googleauthorElla Kumirova-
dc.contributor.googleauthorAstrid Sehested-
dc.contributor.googleauthorDavid Scheie-
dc.contributor.googleauthorOfelia Cruz-
dc.contributor.googleauthorLucas Moreno-
dc.contributor.googleauthorJaeho Cho-
dc.contributor.googleauthorBernward Zeller-
dc.contributor.googleauthorNiels Bovenschen-
dc.contributor.googleauthorMichael Grotzer-
dc.contributor.googleauthorDaniel Alderete-
dc.contributor.googleauthorMatija Snuderl-
dc.contributor.googleauthorOlga Zheludkova-
dc.contributor.googleauthorAndrey Golanov-
dc.contributor.googleauthorKonstantin Okonechnikov-
dc.contributor.googleauthorMartin Mynarek-
dc.contributor.googleauthorBjörn Ole Juhnke-
dc.contributor.googleauthorStefan Rutkowski-
dc.contributor.googleauthorUlrich Schüller-
dc.contributor.googleauthorBarry Pizer-
dc.contributor.googleauthorBarbara von Zezschwitz-
dc.contributor.googleauthorRobert Kwiecien-
dc.contributor.googleauthorMaximilian Wechsung-
dc.contributor.googleauthorFrank Konietschke-
dc.contributor.googleauthorEugene I Hwang-
dc.contributor.googleauthorDominik Sturm-
dc.contributor.googleauthorStefan M Pfister-
dc.contributor.googleauthorAndreas von Deimling-
dc.contributor.googleauthorElisabeth J Rushing-
dc.contributor.googleauthorMarina Ryzhova-
dc.contributor.googleauthorPeter Hauser-
dc.contributor.googleauthorMaria Łastowska-
dc.contributor.googleauthorPieter Wesseling-
dc.contributor.googleauthorFelice Giangaspero-
dc.contributor.googleauthorCynthia Hawkins-
dc.contributor.googleauthorDominique Figarella-Branger-
dc.contributor.googleauthorCharles Eberhart-
dc.contributor.googleauthorPeter Burger-
dc.contributor.googleauthorMarco Gessi-
dc.contributor.googleauthorAndrey Korshunov-
dc.contributor.googleauthorTom S Jacques-
dc.contributor.googleauthorDavid Capper-
dc.contributor.googleauthorTorsten Pietsch-
dc.contributor.googleauthorMarcel Kool-
dc.identifier.doi10.1093/neuonc/noab136-
dc.contributor.localIdA03901-
dc.relation.journalcodeJ02346-
dc.identifier.eissn1523-5866-
dc.identifier.pmid34077956-
dc.identifier.urlhttps://academic.oup.com/neuro-oncology/article/23/9/1597/6291389-
dc.subject.keywordCNS NB-FOXR2-
dc.subject.keywordCNS embryonal tumor-
dc.subject.keywordCNS-PNET-
dc.subject.keywordDNA methylation profiling-
dc.subject.keywordETMR-
dc.contributor.alternativeNameCho, Jae Ho-
dc.contributor.affiliatedAuthor조재호-
dc.citation.volume23-
dc.citation.number9-
dc.citation.startPage1597-
dc.citation.endPage1611-
dc.identifier.bibliographicCitationNEURO-ONCOLOGY, Vol.23(9) : 1597-1611, 2021-09-
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Radiation Oncology (방사선종양학교실) > 1. Journal Papers

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