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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.author | 조재호 | - |
dc.date.accessioned | 2021-12-28T16:42:36Z | - |
dc.date.available | 2021-12-28T16:42:36Z | - |
dc.date.issued | 2021-09 | - |
dc.identifier.issn | 1522-8517 | - |
dc.identifier.uri | https://ir.ymlib.yonsei.ac.kr/handle/22282913/186764 | - |
dc.description.abstract | Background: 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.statementOfResponsibility | restriction | - |
dc.language | English | - |
dc.publisher | Oxford University Press | - |
dc.relation.isPartOf | NEURO-ONCOLOGY | - |
dc.rights | CC BY-NC-ND 2.0 KR | - |
dc.subject.MESH | Brain Neoplasms* / diagnosis | - |
dc.subject.MESH | Brain Neoplasms* / genetics | - |
dc.subject.MESH | Brain Neoplasms* / therapy | - |
dc.subject.MESH | Central Nervous System Neoplasms* / diagnosis | - |
dc.subject.MESH | Central Nervous System Neoplasms* / genetics | - |
dc.subject.MESH | Central Nervous System Neoplasms* / therapy | - |
dc.subject.MESH | Forkhead Transcription Factors | - |
dc.subject.MESH | Humans | - |
dc.subject.MESH | Neoplasms, Germ Cell and Embryonal* / diagnosis | - |
dc.subject.MESH | Neoplasms, Germ Cell and Embryonal* / genetics | - |
dc.subject.MESH | Neoplasms, Germ Cell and Embryonal* / therapy | - |
dc.subject.MESH | Neuroectodermal Tumors, Primitive* / diagnosis | - |
dc.subject.MESH | Neuroectodermal Tumors, Primitive* / genetics | - |
dc.subject.MESH | Neuroectodermal Tumors, Primitive* / therapy | - |
dc.subject.MESH | Pathology, Molecular | - |
dc.subject.MESH | Retrospective Studies | - |
dc.title | Therapeutic implications of improved molecular diagnostics for rare CNS embryonal tumor entities: results of an international, retrospective study | - |
dc.type | Article | - |
dc.contributor.college | College of Medicine (의과대학) | - |
dc.contributor.department | Dept. of Radiation Oncology (방사선종양학교실) | - |
dc.contributor.googleauthor | Katja von Hoff | - |
dc.contributor.googleauthor | Christine Haberler | - |
dc.contributor.googleauthor | Felix Schmitt-Hoffner | - |
dc.contributor.googleauthor | Elizabeth Schepke | - |
dc.contributor.googleauthor | Teresa de Rojas | - |
dc.contributor.googleauthor | Sandra Jacobs | - |
dc.contributor.googleauthor | Michal Zapotocky | - |
dc.contributor.googleauthor | David Sumerauer | - |
dc.contributor.googleauthor | Marta Perek-Polnik | - |
dc.contributor.googleauthor | Christelle Dufour | - |
dc.contributor.googleauthor | Dannis van Vuurden | - |
dc.contributor.googleauthor | Irene Slavc | - |
dc.contributor.googleauthor | Johannes Gojo | - |
dc.contributor.googleauthor | Jessica C Pickles | - |
dc.contributor.googleauthor | Nicolas U Gerber | - |
dc.contributor.googleauthor | Maura Massimino | - |
dc.contributor.googleauthor | Maria Joao Gil-da-Costa | - |
dc.contributor.googleauthor | Miklos Garami | - |
dc.contributor.googleauthor | Ella Kumirova | - |
dc.contributor.googleauthor | Astrid Sehested | - |
dc.contributor.googleauthor | David Scheie | - |
dc.contributor.googleauthor | Ofelia Cruz | - |
dc.contributor.googleauthor | Lucas Moreno | - |
dc.contributor.googleauthor | Jaeho Cho | - |
dc.contributor.googleauthor | Bernward Zeller | - |
dc.contributor.googleauthor | Niels Bovenschen | - |
dc.contributor.googleauthor | Michael Grotzer | - |
dc.contributor.googleauthor | Daniel Alderete | - |
dc.contributor.googleauthor | Matija Snuderl | - |
dc.contributor.googleauthor | Olga Zheludkova | - |
dc.contributor.googleauthor | Andrey Golanov | - |
dc.contributor.googleauthor | Konstantin Okonechnikov | - |
dc.contributor.googleauthor | Martin Mynarek | - |
dc.contributor.googleauthor | Björn Ole Juhnke | - |
dc.contributor.googleauthor | Stefan Rutkowski | - |
dc.contributor.googleauthor | Ulrich Schüller | - |
dc.contributor.googleauthor | Barry Pizer | - |
dc.contributor.googleauthor | Barbara von Zezschwitz | - |
dc.contributor.googleauthor | Robert Kwiecien | - |
dc.contributor.googleauthor | Maximilian Wechsung | - |
dc.contributor.googleauthor | Frank Konietschke | - |
dc.contributor.googleauthor | Eugene I Hwang | - |
dc.contributor.googleauthor | Dominik Sturm | - |
dc.contributor.googleauthor | Stefan M Pfister | - |
dc.contributor.googleauthor | Andreas von Deimling | - |
dc.contributor.googleauthor | Elisabeth J Rushing | - |
dc.contributor.googleauthor | Marina Ryzhova | - |
dc.contributor.googleauthor | Peter Hauser | - |
dc.contributor.googleauthor | Maria Łastowska | - |
dc.contributor.googleauthor | Pieter Wesseling | - |
dc.contributor.googleauthor | Felice Giangaspero | - |
dc.contributor.googleauthor | Cynthia Hawkins | - |
dc.contributor.googleauthor | Dominique Figarella-Branger | - |
dc.contributor.googleauthor | Charles Eberhart | - |
dc.contributor.googleauthor | Peter Burger | - |
dc.contributor.googleauthor | Marco Gessi | - |
dc.contributor.googleauthor | Andrey Korshunov | - |
dc.contributor.googleauthor | Tom S Jacques | - |
dc.contributor.googleauthor | David Capper | - |
dc.contributor.googleauthor | Torsten Pietsch | - |
dc.contributor.googleauthor | Marcel Kool | - |
dc.identifier.doi | 10.1093/neuonc/noab136 | - |
dc.contributor.localId | A03901 | - |
dc.relation.journalcode | J02346 | - |
dc.identifier.eissn | 1523-5866 | - |
dc.identifier.pmid | 34077956 | - |
dc.identifier.url | https://academic.oup.com/neuro-oncology/article/23/9/1597/6291389 | - |
dc.subject.keyword | CNS NB-FOXR2 | - |
dc.subject.keyword | CNS embryonal tumor | - |
dc.subject.keyword | CNS-PNET | - |
dc.subject.keyword | DNA methylation profiling | - |
dc.subject.keyword | ETMR | - |
dc.contributor.alternativeName | Cho, Jae Ho | - |
dc.contributor.affiliatedAuthor | 조재호 | - |
dc.citation.volume | 23 | - |
dc.citation.number | 9 | - |
dc.citation.startPage | 1597 | - |
dc.citation.endPage | 1611 | - |
dc.identifier.bibliographicCitation | NEURO-ONCOLOGY, Vol.23(9) : 1597-1611, 2021-09 | - |
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