Home > Publications Database > Therapeutic implications of improved molecular diagnostics for rare CNS embryonal tumor entities: results of an international, retrospective study. > print

001     162800
005     20230915092407.0
024 7 _ |a 10.1093/neuonc/noab136
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024 7 _ |a pmid:34077956
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024 7 _ |a pmc:PMC8408859
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024 7 _ |a 1522-8517
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024 7 _ |a 1523-5866
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037 _ _ |a DZNE-2021-01455
041 _ _ |a English
082 _ _ |a 610
100 1 _ |a von Hoff, Katja
|0 0000-0002-5669-8546
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245 _ _ |a Therapeutic implications of improved molecular diagnostics for rare CNS embryonal tumor entities: results of an international, retrospective study.
260 _ _ |a Oxford
|c 2021
|b Oxford Univ. Press
336 7 _ |a article
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520 _ _ |a 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.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.
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650 _ 7 |a CNS NB-FOXR2
|2 Other
650 _ 7 |a CNS embryonal tumor
|2 Other
650 _ 7 |a CNS-PNET
|2 Other
650 _ 7 |a DNA methylation profiling
|2 Other
650 _ 7 |a ETMR
|2 Other
650 _ 7 |a FOXR2 protein, human
|2 NLM Chemicals
650 _ 7 |a Forkhead Transcription Factors
|2 NLM Chemicals
650 _ 2 |a Brain Neoplasms: diagnosis
|2 MeSH
650 _ 2 |a Brain Neoplasms: genetics
|2 MeSH
650 _ 2 |a Brain Neoplasms: therapy
|2 MeSH
650 _ 2 |a Central Nervous System Neoplasms: diagnosis
|2 MeSH
650 _ 2 |a Central Nervous System Neoplasms: genetics
|2 MeSH
650 _ 2 |a Central Nervous System Neoplasms: therapy
|2 MeSH
650 _ 2 |a Forkhead Transcription Factors
|2 MeSH
650 _ 2 |a Humans
|2 MeSH
650 _ 2 |a Neoplasms, Germ Cell and Embryonal: diagnosis
|2 MeSH
650 _ 2 |a Neoplasms, Germ Cell and Embryonal: genetics
|2 MeSH
650 _ 2 |a Neoplasms, Germ Cell and Embryonal: therapy
|2 MeSH
650 _ 2 |a Neuroectodermal Tumors, Primitive: diagnosis
|2 MeSH
650 _ 2 |a Neuroectodermal Tumors, Primitive: genetics
|2 MeSH
650 _ 2 |a Neuroectodermal Tumors, Primitive: therapy
|2 MeSH
650 _ 2 |a Pathology, Molecular
|2 MeSH
650 _ 2 |a Retrospective Studies
|2 MeSH
700 1 _ |a Haberler, Christine
|b 1
700 1 _ |a Schmitt-Hoffner, Felix
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700 1 _ |a Schepke, Elizabeth
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700 1 _ |a de Rojas, Teresa
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700 1 _ |a Jacobs, Sandra
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700 1 _ |a Zapotocky, Michal
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700 1 _ |a Sumerauer, David
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700 1 _ |a Perek-Polnik, Marta
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700 1 _ |a Dufour, Christelle
|0 0000-0001-5993-8077
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700 1 _ |a van Vuurden, Dannis
|b 10
700 1 _ |a Slavc, Irene
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700 1 _ |a Gojo, Johannes
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700 1 _ |a Pickles, Jessica C
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700 1 _ |a Gerber, Nicolas U
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700 1 _ |a Massimino, Maura
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700 1 _ |a Gil-da-Costa, Maria Joao
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700 1 _ |a Garami, Miklos
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700 1 _ |a Kumirova, Ella
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700 1 _ |a Sehested, Astrid
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700 1 _ |a Scheie, David
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700 1 _ |a Cruz, Ofelia
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700 1 _ |a Moreno, Lucas
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700 1 _ |a Cho, Jaeho
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700 1 _ |a Zeller, Bernward
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700 1 _ |a Bovenschen, Niels
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700 1 _ |a Grotzer, Michael
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700 1 _ |a Alderete, Daniel
|b 27
700 1 _ |a Snuderl, Matija
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700 1 _ |a Zheludkova, Olga
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700 1 _ |a Golanov, Andrey
|b 30
700 1 _ |a Okonechnikov, Konstantin
|b 31
700 1 _ |a Mynarek, Martin
|b 32
700 1 _ |a Juhnke, Björn Ole
|b 33
700 1 _ |a Rutkowski, Stefan
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700 1 _ |a Schüller, Ulrich
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700 1 _ |a Pizer, Barry
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700 1 _ |a von Zezschwitz, Barbara
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700 1 _ |a Kwiecien, Robert
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700 1 _ |a Wechsung, Maximilian
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700 1 _ |a Konietschke, Frank
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700 1 _ |a Hwang, Eugene I
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700 1 _ |a Sturm, Dominik
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700 1 _ |a Pfister, Stefan M
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700 1 _ |a von Deimling, Andreas
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700 1 _ |a Rushing, Elisabeth J
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700 1 _ |a Ryzhova, Marina
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700 1 _ |a Hauser, Peter
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700 1 _ |a Łastowska, Maria
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700 1 _ |a Wesseling, Pieter
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700 1 _ |a Giangaspero, Felice
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700 1 _ |a Hawkins, Cynthia
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700 1 _ |a Eberhart, Charles
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700 1 _ |a Burger, Peter
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700 1 _ |a Gessi, Marco
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700 1 _ |a Korshunov, Andrey
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700 1 _ |a Jacques, Tom S
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700 1 _ |a Capper, David
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700 1 _ |a Pietsch, Torsten
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700 1 _ |a Kool, Marcel
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773 _ _ |a 10.1093/neuonc/noab136
|g Vol. 23, no. 9, p. 1597 - 1611
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