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000168126 1001_ $$00000-0002-6642-7774$$aThomas, Christian$$b0
000168126 245__ $$aTERT promoter mutation and chromosome 6 loss define a high-risk subtype of ependymoma evolving from posterior fossa subependymoma.
000168126 260__ $$aHeidelberg$$bSpringer$$c2021
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000168126 520__ $$aSubependymomas are benign tumors characteristically encountered in the posterior fossa of adults that show distinct epigenetic profiles assigned to the molecular group 'subependymoma, posterior fossa' (PFSE) of the recently established DNA methylation-based classification of central nervous system tumors. In contrast, most posterior fossa ependymomas exhibit a more aggressive biological behavior and are allocated to the molecular subgroups PFA or PFB. A subset of ependymomas shows epigenetic similarities with subependymomas, but the precise biology of these tumors and their potential relationships remain unknown. We therefore set out to characterize epigenetic traits, mutational profiles, and clinical outcomes of 50 posterior fossa ependymal tumors of the PFSE group. On histo-morphology, these tumors comprised 12 ependymomas, 14 subependymomas and 24 tumors with mixed ependymoma-subependymoma morphology. Mixed ependymoma-subependymoma tumors varied in their extent of ependymoma differentiation (2-95%) but consistently exhibited global epigenetic profiles of the PFSE group. Selective methylome analysis of microdissected tumor components revealed CpG signatures in mixed tumors that coalesce with their pure counterparts. Loss of chr6 (20/50 cases), as well as TERT mutations (21/50 cases), were frequent events enriched in tumors with pure ependymoma morphology (p < 0.001) and confined to areas with ependymoma differentiation in mixed tumors. Clinically, pure ependymoma phenotype, chr6 loss, and TERT mutations were associated with shorter progression-free survival (each p < 0.001). In conclusion, our results suggest that subependymomas may acquire genetic and epigenetic changes throughout tumor evolution giving rise to subclones with ependymoma morphology (resulting in mixed tumors) that eventually overpopulate the subependymoma component (pure PFSE ependymomas).
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000168126 650_7 $$2Other$$aChromosome 6
000168126 650_7 $$2Other$$aDNA methylation
000168126 650_7 $$2Other$$aMixed ependymoma–subependymoma
000168126 650_7 $$2Other$$aSubependymoma
000168126 650_7 $$2Other$$aTERT
000168126 7001_ $$0P:(DE-He78)560b340ec11109efc8f88c383f51ba8e$$aThierfelder, Felix$$b1$$udkfz
000168126 7001_ $$aTräger, Malte$$b2
000168126 7001_ $$aSoschinski, Patrick$$b3
000168126 7001_ $$aMüther, Michael$$b4
000168126 7001_ $$0P:(DE-He78)92820b4867c955a04f642707ecf35b40$$aEdelmann, Dominic$$b5$$udkfz
000168126 7001_ $$aFörster, Alexandra$$b6
000168126 7001_ $$aGeiler, Carola$$b7
000168126 7001_ $$0P:(DE-He78)96b5c6d770f3aa58db900d1434f5a3f5$$aKim, Hee-Yeong$$b8$$udkfz
000168126 7001_ $$0P:(DE-He78)832f5277c0186f22e7704f1930239636$$aFilipski, Katharina$$b9$$udkfz
000168126 7001_ $$0P:(DE-He78)b15b56a6ed37417d476470c60c0140ff$$aHarter, Patrick$$b10
000168126 7001_ $$aSchittenhelm, Jens$$b11
000168126 7001_ $$aEckert, Franziska$$b12
000168126 7001_ $$aNtoulias, Georgios$$b13
000168126 7001_ $$aMay, Sven-Axel$$b14
000168126 7001_ $$aStummer, Walter$$b15
000168126 7001_ $$aOnken, Julia$$b16
000168126 7001_ $$aVajkoczy, Peter$$b17
000168126 7001_ $$aSchüller, Ulrich$$b18
000168126 7001_ $$0P:(DE-HGF)0$$aHeppner, Frank L$$b19
000168126 7001_ $$0P:(DE-He78)51bf9ae9cb5771b30c483e5597ef606c$$aCapper, David$$b20$$udkfz
000168126 7001_ $$0P:(DE-HGF)0$$aKoch, Arend$$b21
000168126 7001_ $$aKaul, David$$b22
000168126 7001_ $$aPaulus, Werner$$b23
000168126 7001_ $$aHasselblatt, Martin$$b24
000168126 7001_ $$0P:(DE-He78)db2e8cf0dd8fa826896839ffee4b1411$$aSchweizer, Leonille$$b25$$udkfz
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