000131722 001__ 131722 000131722 005__ 20240229105009.0 000131722 0247_ $$2doi$$a10.1016/j.jocn.2017.09.013 000131722 0247_ $$2pmid$$apmid:28993028 000131722 0247_ $$2ISSN$$a0967-5868 000131722 0247_ $$2ISSN$$a1532-2653 000131722 0247_ $$2altmetric$$aaltmetric:27295857 000131722 037__ $$aDKFZ-2018-00028 000131722 041__ $$aeng 000131722 082__ $$a610 000131722 1001_ $$aHalliday, Gail C$$b0 000131722 245__ $$aThe case for DNA methylation based molecular profiling to improve diagnostic accuracy for central nervous system embryonal tumors (not otherwise specified) in adults. 000131722 260__ $$aBurlington, Mass.$$bHarcourt$$c2018 000131722 3367_ $$2DRIVER$$aarticle 000131722 3367_ $$2DataCite$$aOutput Types/Journal article 000131722 3367_ $$0PUB:(DE-HGF)16$$2PUB:(DE-HGF)$$aJournal Article$$bjournal$$mjournal$$s1565606817_561 000131722 3367_ $$2BibTeX$$aARTICLE 000131722 3367_ $$2ORCID$$aJOURNAL_ARTICLE 000131722 3367_ $$00$$2EndNote$$aJournal Article 000131722 520__ $$aCentral nervous system primitive neuro-ectodermal tumors (CNS-PNETs), have recently been re-classified in the most recent 2016 WHO Classification into a standby catch all category, 'CNS Embryonal Tumor, not otherwise specified' (CNS embryonal tumor, NOS) based on epigenetic, biologic and histopathologic criteria. CNS embryonal tumors (NOS) are a rare, histologically and molecularly heterogeneous group of tumors that predominantly affect children, and occasionally adults. Diagnosis of this entity continues to be challenging and the ramifications of misdiagnosis of this aggressive class of brain tumors are significant. We report the case of a 45-year-old woman who was diagnosed with a central nervous system embryonal tumor (NOS) based on immunohistochemical analysis of the patients tumor at diagnosis. However, later genome-wide methylation profiling of the diagnostic tumor undertaken to guide treatment, revealed characteristics most consistent with IDH-mutant astrocytoma. DNA sequencing and immunohistochemistry confirmed the presence of IDH1 and ATRX mutations resulting in a revised diagnosis of high-grade small cell astrocytoma, and the implementation of a less aggressive treatment regime tailored more appropriately to the patients tumor type. This case highlights the inadequacy of histology alone for the diagnosis of brain tumours and the utility of methylation profiling and integrated genomic analysis for the diagnostic verification of adults with suspected CNS embryonal tumor (NOS), and is consistent with the increasing realization in the field that a combined diagnostic approach based on clinical, histopathological and molecular data is required to more accurately distinguish brain tumor subtypes and inform more effective therapy. 000131722 536__ $$0G:(DE-HGF)POF3-319H$$a319H - Addenda (POF3-319H)$$cPOF3-319H$$fPOF III$$x0 000131722 588__ $$aDataset connected to CrossRef, PubMed, 000131722 7001_ $$aJunckerstorff, Reimar C$$b1 000131722 7001_ $$aBentel, Jacqueline M$$b2 000131722 7001_ $$aMiles, Andrew$$b3 000131722 7001_ $$0P:(DE-He78)551bb92841f634070997aa168d818492$$aJones, David$$b4$$udkfz 000131722 7001_ $$0P:(DE-He78)744146d3b5a3df1e0ac555e5bf1ee5cc$$aHovestadt, Volker$$b5$$udkfz 000131722 7001_ $$0P:(DE-He78)51bf9ae9cb5771b30c483e5597ef606c$$aCapper, David$$b6$$udkfz 000131722 7001_ $$aEndersby, Raelene$$b7 000131722 7001_ $$aCole, Catherine H$$b8 000131722 7001_ $$avan Hagen, Tom$$b9 000131722 7001_ $$aGottardo, Nicholas G$$b10 000131722 773__ $$0PERI:(DE-600)2009190-4$$a10.1016/j.jocn.2017.09.013$$gVol. 47, p. 163 - 167$$p163 - 167$$tJournal of clinical neuroscience$$v47$$x0967-5868$$y2018 000131722 909CO $$ooai:inrepo02.dkfz.de:131722$$pVDB 000131722 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)551bb92841f634070997aa168d818492$$aDeutsches Krebsforschungszentrum$$b4$$kDKFZ 000131722 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)744146d3b5a3df1e0ac555e5bf1ee5cc$$aDeutsches Krebsforschungszentrum$$b5$$kDKFZ 000131722 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)51bf9ae9cb5771b30c483e5597ef606c$$aDeutsches Krebsforschungszentrum$$b6$$kDKFZ 000131722 9131_ $$0G:(DE-HGF)POF3-319H$$1G:(DE-HGF)POF3-310$$2G:(DE-HGF)POF3-300$$3G:(DE-HGF)POF3$$4G:(DE-HGF)POF$$aDE-HGF$$bGesundheit$$lKrebsforschung$$vAddenda$$x0 000131722 9141_ $$y2018 000131722 915__ $$0StatID:(DE-HGF)0420$$2StatID$$aNationallizenz 000131722 915__ $$0StatID:(DE-HGF)0300$$2StatID$$aDBCoverage$$bMedline 000131722 915__ $$0StatID:(DE-HGF)0310$$2StatID$$aDBCoverage$$bNCBI Molecular Biology Database 000131722 915__ $$0StatID:(DE-HGF)0100$$2StatID$$aJCR$$bJ CLIN NEUROSCI : 2015 000131722 915__ $$0StatID:(DE-HGF)0200$$2StatID$$aDBCoverage$$bSCOPUS 000131722 915__ $$0StatID:(DE-HGF)0199$$2StatID$$aDBCoverage$$bThomson Reuters Master Journal List 000131722 915__ $$0StatID:(DE-HGF)0111$$2StatID$$aWoS$$bScience Citation Index Expanded 000131722 915__ $$0StatID:(DE-HGF)0150$$2StatID$$aDBCoverage$$bWeb of Science Core Collection 000131722 915__ $$0StatID:(DE-HGF)9900$$2StatID$$aIF < 5 000131722 9201_ $$0I:(DE-He78)B062-20160331$$kB062$$lPädiatrische Neuroonkologie$$x0 000131722 9201_ $$0I:(DE-He78)B060-20160331$$kB060$$lMolekulare Genetik$$x1 000131722 9201_ $$0I:(DE-He78)G380-20160331$$kG380$$lKKE Neuropathologie$$x2 000131722 9201_ $$0I:(DE-He78)L101-20160331$$kL101$$lDKTK Heidelberg$$x3 000131722 980__ $$ajournal 000131722 980__ $$aVDB 000131722 980__ $$aI:(DE-He78)B062-20160331 000131722 980__ $$aI:(DE-He78)B060-20160331 000131722 980__ $$aI:(DE-He78)G380-20160331 000131722 980__ $$aI:(DE-He78)L101-20160331 000131722 980__ $$aUNRESTRICTED