%0 Journal Article
%A Federico, Aniello
%A Thomas, Christian
%A Miskiewicz, Katarzyna
%A Woltering, Niklas
%A Zin, Francesca
%A Nemes, Karolina
%A Bison, Brigitte
%A Johann, Pascal D
%A Hawes, Debra
%A Bens, Susanne
%A Kordes, Uwe
%A Albrecht, Steffen
%A Dohmen, Hildegard
%A Hauser, Peter
%A Keyvani, Kathy
%A van Landeghem, Frank K H
%A Lund, Eva Løbner
%A Scheie, David
%A Mawrin, Christian
%A Monoranu, Camelia-Maria
%A Parm Ulhøi, Benedicte
%A Pietsch, Torsten
%A Reinhard, Harald
%A Riemenschneider, Markus J
%A Sehested, Astrid
%A Sumerauer, David
%A Siebert, Reiner
%A Paulus, Werner
%A Frühwald, Michael C
%A Kool, Marcel
%A Hasselblatt, Martin
%T ATRT-SHH comprises three molecular subgroups with characteristic clinical and histopathological features and prognostic significance.
%J Acta neuropathologica
%V 143
%N 6
%@ 0001-6322
%C Heidelberg
%I Springer
%M DKFZ-2022-00945
%P 697-711
%D 2022
%Z #EA:B062#LA:B062# / 2022 Jun;143(6):697-711
%X Atypical teratoid/rhabdoid tumor (ATRT) is an aggressive central nervous system tumor characterized by loss of SMARCB1/INI1 protein expression and comprises three distinct molecular groups, ATRT-TYR, ATRT-MYC and ATRT-SHH. ATRT-SHH represents the largest molecular group and is heterogeneous with regard to age, tumor location and epigenetic profile. We, therefore, aimed to investigate if heterogeneity within ATRT-SHH might also have biological and clinical importance. Consensus clustering of DNA methylation profiles and confirmatory t-SNE analysis of 65 ATRT-SHH yielded three robust molecular subgroups, i.e., SHH-1A, SHH-1B and SHH-2. These subgroups differed by median age of onset (SHH-1A: 18 months, SHH-1B: 107 months, SHH-2: 13 months) and tumor location (SHH-1A: 88
%K ASCL1 (Other)
%K Atypical teratoid/rhabdoid tumor (Other)
%K DNA methylation profiling (Other)
%K GFAP (Other)
%K Gene expression (Other)
%K Neuroradiology (Other)
%K OLIG2 (Other)
%K Overall survival (Other)
%K Prognosis (Other)
%K Sonic hedgehog (Other)
%F PUB:(DE-HGF)16
%9 Journal Article
%$ pmid:35501487
%R 10.1007/s00401-022-02424-5
%U https://inrepo02.dkfz.de/record/179883