TY  - JOUR
AU  - Federico, Aniello
AU  - Thomas, Christian
AU  - Miskiewicz, Katarzyna
AU  - Woltering, Niklas
AU  - Zin, Francesca
AU  - Nemes, Karolina
AU  - Bison, Brigitte
AU  - Johann, Pascal D
AU  - Hawes, Debra
AU  - Bens, Susanne
AU  - Kordes, Uwe
AU  - Albrecht, Steffen
AU  - Dohmen, Hildegard
AU  - Hauser, Peter
AU  - Keyvani, Kathy
AU  - van Landeghem, Frank K H
AU  - Lund, Eva Løbner
AU  - Scheie, David
AU  - Mawrin, Christian
AU  - Monoranu, Camelia-Maria
AU  - Parm Ulhøi, Benedicte
AU  - Pietsch, Torsten
AU  - Reinhard, Harald
AU  - Riemenschneider, Markus J
AU  - Sehested, Astrid
AU  - Sumerauer, David
AU  - Siebert, Reiner
AU  - Paulus, Werner
AU  - Frühwald, Michael C
AU  - Kool, Marcel
AU  - Hasselblatt, Martin
TI  - ATRT-SHH comprises three molecular subgroups with characteristic clinical and histopathological features and prognostic significance.
JO  - Acta neuropathologica
VL  - 143
IS  - 6
SN  - 0001-6322
CY  - Heidelberg
PB  - Springer
M1  - DKFZ-2022-00945
SP  - 697-711
PY  - 2022
N1  - #EA:B062#LA:B062# / 2022 Jun;143(6):697-711
AB  - 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
KW  - ASCL1 (Other)
KW  - Atypical teratoid/rhabdoid tumor (Other)
KW  - DNA methylation profiling (Other)
KW  - GFAP (Other)
KW  - Gene expression (Other)
KW  - Neuroradiology (Other)
KW  - OLIG2 (Other)
KW  - Overall survival (Other)
KW  - Prognosis (Other)
KW  - Sonic hedgehog (Other)
LB  - PUB:(DE-HGF)16
C6  - pmid:35501487
DO  - DOI:10.1007/s00401-022-02424-5
UR  - https://inrepo02.dkfz.de/record/179883
ER  -