Oligosarcomas, IDH-mutant are distinct and aggressive

  1. Suwala, Abigail K.
  2. Felix, Marius
  3. Friedel, Dennis
  4. Stichel, Damian
  5. Schrimpf, Daniel
  6. Hinz, Felix
  7. Hewer, Ekkehard
  8. Schweizer, Leonille
  9. Dohmen, Hildegard
  10. Pohl, Ute
  11. Staszewski, Ori
  12. Korshunov, Andrey
  13. Stein, Marco
  14. Wongsurawat, Thidathip
  15. Cheunsuacchon, Pornsuk
  16. Sathornsumetee, Sith
  17. Koelsche, Christian
  18. Turner, Clinton
  19. Le Rhun, Emilie
  20. Mühlebner, Angelika
  21. Schucht, Philippe
  22. Özduman, Koray
  23. Ono, Takahiro
  24. Shimizu, Hiroaki
  25. Prinz, Marco
  26. Acker, Till
  27. Herold-Mende, Christel
  28. Kessler, Tobias
  29. Wick, Wolfgang
  30. Capper, David
  31. Wesseling, Pieter
  32. Sahm, Felix
  33. von Deimling, Andreas
  34. Hartmann, Christian
  35. Reuß, David ORCID logo

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WeightNameValue
1000 Titel
  • Oligosarcomas, IDH-mutant are distinct and aggressive
1000 Autor/in
  1. Suwala, Abigail K. |
  2. Felix, Marius |
  3. Friedel, Dennis |
  4. Stichel, Damian |
  5. Schrimpf, Daniel |
  6. Hinz, Felix |
  7. Hewer, Ekkehard |
  8. Schweizer, Leonille |
  9. Dohmen, Hildegard |
  10. Pohl, Ute |
  11. Staszewski, Ori |
  12. Korshunov, Andrey |
  13. Stein, Marco |
  14. Wongsurawat, Thidathip |
  15. Cheunsuacchon, Pornsuk |
  16. Sathornsumetee, Sith |
  17. Koelsche, Christian |
  18. Turner, Clinton |
  19. Le Rhun, Emilie |
  20. Mühlebner, Angelika |
  21. Schucht, Philippe |
  22. Özduman, Koray |
  23. Ono, Takahiro |
  24. Shimizu, Hiroaki |
  25. Prinz, Marco |
  26. Acker, Till |
  27. Herold-Mende, Christel |
  28. Kessler, Tobias |
  29. Wick, Wolfgang |
  30. Capper, David |
  31. Wesseling, Pieter |
  32. Sahm, Felix |
  33. von Deimling, Andreas |
  34. Hartmann, Christian |
  35. Reuß, David |
1000 Erscheinungsjahr 2021
1000 Publikationstyp
  1. Artikel |
1000 Online veröffentlicht
  • 2021-12-30
1000 Erschienen in
1000 Quellenangabe
  • 143(2):263-281
1000 Copyrightjahr
  • 2021
1000 Lizenz
1000 Verlagsversion
  • https://doi.org/10.1007/s00401-021-02395-z |
  • https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8742817/ |
1000 Publikationsstatus
1000 Sprache der Publikation
1000 Abstract/Summary
  • Oligodendrogliomas are defined at the molecular level by the presence of an IDH mutation and codeletion of chromosomal arms 1p and 19q. In the past, case reports and small studies described gliomas with sarcomatous features arising from oligodendrogliomas, so called oligosarcomas. Here, we report a series of 24 IDH-mutant oligosarcomas from 23 patients forming a distinct methylation class. The tumors were recurrences from prior oligodendrogliomas or developed de novo. Precursor tumors of 12 oligosarcomas were histologically and molecularly indistinguishable from conventional oligodendrogliomas. Oligosarcoma tumor cells were embedded in a dense network of reticulin fibers, frequently showing p53 accumulation, positivity for SMA and CALD1, loss of OLIG2 and gain of H3K27 trimethylation (H3K27me3) as compared to primary lesions. In 5 oligosarcomas no 1p/19q codeletion was detectable, although it was present in the primary lesions. Copy number neutral LOH was determined as underlying mechanism. Oligosarcomas harbored an increased chromosomal copy number variation load with frequent CDKN2A/B deletions. Proteomic profiling demonstrated oligosarcomas to be highly distinct from conventional CNS WHO grade 3 oligodendrogliomas with consistent evidence for a smooth muscle differentiation. Expression of several tumor suppressors was reduced with NF1 being lost frequently. In contrast, oncogenic YAP1 was aberrantly overexpressed in oligosarcomas. Panel sequencing revealed mutations in NF1 and TP53 along with IDH1/2 and TERT promoter mutations. Survival of patients was significantly poorer for oligosarcomas as first recurrence than for grade 3 oligodendrogliomas as first recurrence. These results establish oligosarcomas as a distinct group of IDH-mutant gliomas differing from conventional oligodendrogliomas on the histologic, epigenetic, proteomic, molecular and clinical level. The diagnosis can be based on the combined presence of (a) sarcomatous histology, (b) IDH-mutation and (c) TERT promoter mutation and/or 1p/19q codeletion, or, in unresolved cases, on its characteristic DNA methylation profile.
1000 Sacherschließung
lokal NF1
lokal SMA
lokal Aged [MeSH]
lokal Gliosarcoma
lokal Prognosis
lokal 1p/19q
lokal TP53
lokal Male [MeSH]
lokal DNA methylation
lokal Variant
lokal Type
lokal Oligodendroglioma
lokal Female [MeSH]
lokal Mutation [MeSH]
lokal Sarcoma/genetics [MeSH]
lokal Oligosarcoma
lokal Brain Neoplasms/genetics [MeSH]
lokal Adult [MeSH]
lokal Humans [MeSH]
lokal Oligodendroglioma/pathology [MeSH]
lokal Middle Aged [MeSH]
lokal YAP1
lokal Codeletion
lokal Oligodendroglioma/genetics [MeSH]
lokal Subtype
lokal TERT
lokal Sarcoma/pathology [MeSH]
lokal Original Paper
lokal Isocitrate Dehydrogenase/genetics [MeSH]
lokal Brain Neoplasms/pathology [MeSH]
1000 Liste der Beteiligten
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  1. Oligosarcomas, IDH-mutant are distinct and aggressive
    Oligosarcomas, IDH-mutant are distinct and aggressive
1000 Objektart article
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1000 @id frl:6447458.rdf
1000 Erstellt am 2023-04-28T16:06:53.844+0200
1000 Erstellt von 322
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1000 Zuletzt bearbeitet 2023-10-20T20:42:21.674+0200
1000 Objekt bearb. Fri Oct 20 20:42:21 CEST 2023
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