TY  - JOUR
AU  - Deng, Maximilian Yuanzhe
AU  - Sill, Martin
AU  - Sturm, Dominik
AU  - Stichel, Damian
AU  - Witt, Hendrik
AU  - Ecker, Jonas
AU  - Wittmann, Andreas
AU  - Schittenhelm, Jens
AU  - Ebinger, Martin
AU  - Schuhmann, Martin U
AU  - Figarella-Branger, Dominique
AU  - Aronica, Eleonora
AU  - Staszewski, Ori
AU  - Preusser, Matthias
AU  - Haberler, Christine
AU  - Lauten, Melchior
AU  - Schüller, Ulrich
AU  - Hartmann, Christian
AU  - Snuderl, Matija
AU  - Dunham, Christopher
AU  - Jabado, Nada
AU  - Wesseling, Pieter
AU  - Deckert, Martina
AU  - Keyvani, Kathy
AU  - Gottardo, Nick
AU  - Giangaspero, Felice
AU  - von Hoff, Katja
AU  - Ellison, David W
AU  - Pietsch, Torsten
AU  - Herold Mende, Christel
AU  - Milde, Till
AU  - Witt, Olaf
AU  - Kool, Marcel
AU  - Korshunov, Andrey
AU  - Wick, Wolfgang
AU  - von Deimling, Andreas
AU  - Pfister, Stefan M
AU  - Jones, David T W
AU  - Sahm, Felix
TI  - Diffuse Glioneuronal tumour with Oligodendroglioma-like features and Nuclear Clusters (DGONC) - a molecularly-defined glioneuronal CNS tumour class displaying recurrent monosomy 14.
JO  - Neuropathology & applied neurobiology
VL  - 46
IS  - 5
SN  - 1365-2990
CY  - Oxford [u.a.]
PB  - Wiley-Blackwell
M1  - DKFZ-2020-00010
SP  - 422-430
PY  - 2020
N1  - 2020 Aug;46(5):422-430#EA:B360#LA:B300#
AB  - DNA methylation-based central nervous system (CNS) tumour classification has identified numerous molecularly-distinct tumour types, and clinically relevant subgroups among known CNS tumour entities that were previously thought to represent homogeneous diseases. Our study aimed at characterising a novel, molecularly-defined variant of glioneuronal CNS tumour.DNA methylation profiling was performed using the Infinium MethylationEPIC or 450k BeadChip arrays (Illumina) and analysed using the 'conumee' package in R computing environment. Additional gene panel sequencing was also performed. Tumour samples were collected at the German Cancer Research Centre (DKFZ) and provided by multi-national collaborators. Histological sections were also collected and independently reviewed.Genome-wide DNA methylation data from >25,000 CNS tumours were screened for clusters separated from established DNA methylation classes, revealing a novel group comprising 31 tumours, mainly found in paediatric patients. This DNA methylation-defined variant of low-grade CNS tumours with glioneuronal differentiation displays recurrent monosomy 14, nuclear clusters within a morphology that is otherwise reminiscent of oligodendroglioma and other established entities with clear-cell histology, and a lack of genetic alterations commonly observed in other (paediatric) glioneuronal entities.DNA methylation-based tumour classification is an objective method of assessing tumour origins, which may aid in diagnosis, especially for atypical cases. With increasing sample size, methylation analysis allows for the identification of rare, putative new tumour entities, which are currently not recognized by the WHO classification. Our study revealed the existence of a DNA methylation-defined class of low-grade glioneuronal tumours with recurrent monosomy 14, oligodendroglioma-like features and nuclear clusters.
LB  - PUB:(DE-HGF)16
C6  - pmid:31867747
DO  - DOI:10.1111/nan.12590
UR  - https://inrepo02.dkfz.de/record/148818
ER  -