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000241157 1001_ $$aTräger, Malte$$b0
000241157 245__ $$aAdult intracranial ependymoma - relevance of DNA methylation profiling for diagnosis, prognosis and treatment.
000241157 260__ $$aOxford$$bOxford Univ. Press$$c2023
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000241157 520__ $$aA methylation-based classification of ependymoma has recently found broad application. However, the diagnostic advantage and implications for treatment decisions remain unclear. Here, we retrospectively evaluate the impact of surgery and radiotherapy on outcome after molecular reclassification of adult intracranial ependymomas.Tumors diagnosed as intracranial ependymomas from 170 adult patients collected from eight diagnostic institutions were subjected to DNA methylation profiling. Molecular classes, patient characteristics, and treatment were correlated with progression-free survival (PFS).The classifier indicated an ependymal tumor in 73.5%, a different tumor entity in 10.6% and non-classifiable tumors in 15.9% of cases, respectively. The most prevalent molecular classes were posterior fossa ependymoma group B (EPN-PFB, 32.9%), posterior fossa subependymoma (PF-SE, 25.9%), and supratentorial ZFTA fusion-positive ependymoma (EPN-ZFTA, 11.2%). With a median follow-up of 60.0 months, the 5- and 10-year-PFS rates were 64.5% and 41.8% for EPN-PFB, 67.4% and 45.2% for PF-SE and 60.3% and 60.3% for EPN-ZFTA. In EPN-PFB, but not in other molecular classes, gross total resection (p=0.009) and postoperative radiotherapy (p=0.007) were significantly associated with improved PFS in multivariable analysis. Histological tumor grading (WHO 2 vs. 3) was not a predictor of prognosis within molecularly defined ependymoma classes.DNA methylation profiling improves diagnostic accuracy and risk stratification in adult intracranial ependymoma. The molecular class of PF-SE is unexpectedly prevalent among adult tumors with ependymoma histology and relapsed as frequently as EPN-PFB, despite the supposed benign nature. Gross total resection and radiotherapy may represent key factors in determining the outcome of EPN-PFB patients.
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000241157 650_7 $$2Other$$aDNA methylation
000241157 650_7 $$2Other$$aadult
000241157 650_7 $$2Other$$aependymoma
000241157 650_7 $$2Other$$aintracranial
000241157 650_7 $$2Other$$aradiotherapy
000241157 7001_ $$0P:(DE-He78)db2e8cf0dd8fa826896839ffee4b1411$$aSchweizer, Leonille$$b1$$udkfz
000241157 7001_ $$aEilís, Pérez$$b2
000241157 7001_ $$aSchmid, Simone$$b3
000241157 7001_ $$aHain, Elisabeth G$$b4
000241157 7001_ $$aDittmayer, Carsten$$b5
000241157 7001_ $$aOnken, Julia$$b6
000241157 7001_ $$aFukuoka, Kohei$$b7
000241157 7001_ $$00000-0002-3851-2349$$aIchimura, Koichi$$b8
000241157 7001_ $$00000-0002-8731-1121$$aSchüller, Ulrich$$b9
000241157 7001_ $$aDührsen, Lasse$$b10
000241157 7001_ $$00000-0001-9427-5555$$aMüther, Michael$$b11
000241157 7001_ $$aPaulus, Werner$$b12
000241157 7001_ $$00000-0002-6642-7774$$aThomas, Christian$$b13
000241157 7001_ $$aGutt-Will, Marielena$$b14
000241157 7001_ $$aSchucht, Philippe$$b15
000241157 7001_ $$aMaragkou, Theoni$$b16
000241157 7001_ $$00000-0002-9168-6209$$aSchittenhelm, Jens$$b17
000241157 7001_ $$aEckert, Franziska$$b18
000241157 7001_ $$0P:(DE-HGF)0$$aNiyazi, Maximilian$$b19
000241157 7001_ $$aFleischmann, Daniel$$b20
000241157 7001_ $$aDorostkar, Mario$$b21
000241157 7001_ $$aFeyer, Petra$$b22
000241157 7001_ $$aMay, Sven-Axel$$b23
000241157 7001_ $$aMoskopp, Dag$$b24
000241157 7001_ $$aBadakhshi, Harun$$b25
000241157 7001_ $$aRadke, Cornelia$$b26
000241157 7001_ $$aWalter, Jan$$b27
000241157 7001_ $$aEhret, Felix$$b28
000241157 7001_ $$0P:(DE-He78)51bf9ae9cb5771b30c483e5597ef606c$$aCapper, David$$b29$$udkfz
000241157 7001_ $$0P:(DE-HGF)0$$aKaul, David$$b30
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