%0 Journal Article
%A Federico, Aniello
%A Schmitt-Hoffner, Felix
%A Fonseca, Adriana
%A Geisemeyer, Neal
%A Bruckner, Katharina
%A Mauermann, Monika
%A Sill, Martin
%A Stichel, Damian
%A Sturm, Dominik
%A Schüller, Ulrich
%A Tauziede-Espariat, Arnault
%A Varlet, Pascale
%A Capper, David
%A Abdullaev, Zied
%A Schrimpf, Daniel
%A Selt, Florian
%A Williamson, Lane
%A Donson, Andrew M
%A Antonelli, Manila
%A Miele, Evelina
%A Snuderl, Matija
%A Brandner, Sebastian
%A Łastowska, Maria
%A Van Der Lugt, Jasper
%A Bunt, Jens
%A Kramm, Christof
%A Kolenova, Alexandra
%A Raghunathan, Aditya
%A Wilson, Yelena
%A Weintraub, Lauren
%A Hansford, Jordan R
%A Spiegl-Kreinecker, Sabine
%A Aistleitner, Barbara
%A Baroni, Lorena
%A Zapotocky, Michal
%A Ramaswamy, Vijay
%A Korshunov, Andrey
%A Jones, Barbara
%A Kjaersgaard, Mimi
%A Kranendonk, Mariëtte E
%A Haberler, Christine
%A Packer, Roger J
%A Jäger, Natalie
%A Deimling, Andreas Von
%A Sahm, Felix
%A Koster, Jan
%A Aldape, Kenneth
%A Pfister, Stefan M
%A Hoff, Katja Von
%A Gojo, Johannes
%A Kool, Marcel
%T Molecular and Clinical Stratification of Astroblastomas: Three distinct Fusion-Defined Groups Informing Risk-Adapted Treatment Strategies.
%J Neuro-Oncology
%V nn
%@ 1522-8517
%C Oxford
%I Oxford Univ. Press
%M DKFZ-2025-03033
%P nn
%D 2025
%Z #EA:B062#LA:B062# / epub
%X Astroblastomas are rare brain tumors predominantly affecting children and young adults, for which molecular subtypes and clinical management remain undefined.We analyzed tumor samples, molecular profiles, and clinical data from 200 patients, classified as 'Astroblastoma, MN1-altered' under WHO criteria, using DNA methylation profiling, DNA/RNA profiling/sequencing, and survival analyses.DNA methylation analyses identified three groups: Group A (n = 143, characterized by MN1::BEND2 fusions, predominantly supratentorial location, with striking female predominance and favorable survival); Group B (n = 37, epigenetically and transcriptionally closely related to Group A, but characterized by EWSR1::BEND2 fusions, with spinal and infratentorial locations and poor prognosis); and Group C (n = 20, epigenetically and transcriptionally distinct, characterized by MN1::CXXC5 fusions, exclusively supratentorially located, with favorable survival). Progression-free and overall survival were significantly shorter in Group B (5-year PFS 14
%K EWSR1::BEND2 (Other)
%K MN1-altered (Other)
%K MN1::BEND2 (Other)
%K MN1::CXXC5 (Other)
%K Astroblastoma (Other)
%K gene fusion (Other)
%F PUB:(DE-HGF)16
%9 Journal Article
%$ pmid:41429568
%R 10.1093/neuonc/noaf283
%U https://inrepo02.dkfz.de/record/307434