TY  - JOUR
AU  - Karremann, Michael
AU  - Gielen, Gerrit H
AU  - Hoffmann, Marion
AU  - Wiese, Maria
AU  - Colditz, Niclas
AU  - Warmuth-Metz, Monika
AU  - Bison, Brigitte
AU  - Claviez, Alexander
AU  - van Vuurden, Dannis G
AU  - von Bueren, André O
AU  - Gessi, Marco
AU  - Kühnle, Ingrid
AU  - Hans, Volkmar H
AU  - Benesch, Martin
AU  - Sturm, Dominik
AU  - Kortmann, Rolf-Dieter
AU  - Waha, Andreas
AU  - Pietsch, Torsten
AU  - Kramm, Christof M
TI  - Diffuse high-grade gliomas with H3 K27M mutations carry a dismal prognosis independent of tumor location.
JO  - Neuro-Oncology
VL  - 20
IS  - 1
SN  - 1523-5866
CY  - Oxford
PB  - Oxford Univ. Press
M1  - DKFZ-2018-00080
SP  - 123 - 131
PY  - 2018
AB  - The novel entity of 'diffuse midline glioma, H3 K27M-mutant' has been defined in the 2016 revision of the World Health Organization (WHO) classification of tumors of the central nervous system (CNS). Tumors of this entity arise in CNS midline structures of predominantly pediatric patients and are associated with an overall dismal prognosis. They are defined by K27M mutations in H3F3A or HIST1H3B/C, encoding for histone 3 variants H3.3 and H3.1, respectively, which are considered hallmark events driving gliomagenesis.Here, we characterized 85 centrally reviewed diffuse gliomas on midline locations enrolled in the nationwide pediatric German HIT-HGG registry regarding tumor site, histone 3 mutational status, WHO grade, age, sex, and extent of tumor resection.We found 56 H3.3 K27M-mutant tumors (66
LB  - PUB:(DE-HGF)16
C6  - pmid:29016894
C2  - pmc:PMC5761525
DO  - DOI:10.1093/neuonc/nox149
UR  - https://inrepo02.dkfz.de/record/131783
ER  -