TY  - JOUR
AU  - Federico, Aniello
AU  - Schmitt-Hoffner, Felix
AU  - Fonseca, Adriana
AU  - Geisemeyer, Neal
AU  - Bruckner, Katharina
AU  - Mauermann, Monika
AU  - Sill, Martin
AU  - Stichel, Damian
AU  - Sturm, Dominik
AU  - Schüller, Ulrich
AU  - Tauziede-Espariat, Arnault
AU  - Varlet, Pascale
AU  - Capper, David
AU  - Abdullaev, Zied
AU  - Schrimpf, Daniel
AU  - Selt, Florian
AU  - Williamson, Lane
AU  - Donson, Andrew M
AU  - Antonelli, Manila
AU  - Miele, Evelina
AU  - Snuderl, Matija
AU  - Brandner, Sebastian
AU  - Łastowska, Maria
AU  - Van Der Lugt, Jasper
AU  - Bunt, Jens
AU  - Kramm, Christof
AU  - Kolenova, Alexandra
AU  - Raghunathan, Aditya
AU  - Wilson, Yelena
AU  - Weintraub, Lauren
AU  - Hansford, Jordan R
AU  - Spiegl-Kreinecker, Sabine
AU  - Aistleitner, Barbara
AU  - Baroni, Lorena
AU  - Zapotocky, Michal
AU  - Ramaswamy, Vijay
AU  - Korshunov, Andrey
AU  - Jones, Barbara
AU  - Kjaersgaard, Mimi
AU  - Kranendonk, Mariëtte E
AU  - Haberler, Christine
AU  - Packer, Roger J
AU  - Jäger, Natalie
AU  - Deimling, Andreas Von
AU  - Sahm, Felix
AU  - Koster, Jan
AU  - Aldape, Kenneth
AU  - Pfister, Stefan M
AU  - Hoff, Katja Von
AU  - Gojo, Johannes
AU  - Kool, Marcel
TI  - Molecular and Clinical Stratification of Astroblastomas: Three distinct Fusion-Defined Groups Informing Risk-Adapted Treatment Strategies.
JO  - Neuro-Oncology
VL  - nn
SN  - 1522-8517
CY  - Oxford
PB  - Oxford Univ. Press
M1  - DKFZ-2025-03033
SP  - nn
PY  - 2025
N1  - #EA:B062#LA:B062# / epub
AB  - 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
KW  - EWSR1::BEND2 (Other)
KW  - MN1-altered (Other)
KW  - MN1::BEND2 (Other)
KW  - MN1::CXXC5 (Other)
KW  - Astroblastoma (Other)
KW  - gene fusion (Other)
LB  - PUB:(DE-HGF)16
C6  - pmid:41429568
DO  - DOI:10.1093/neuonc/noaf283
UR  - https://inrepo02.dkfz.de/record/307434
ER  -