000300620 001__ 300620
000300620 005__ 20250427020240.0
000300620 0247_ $$2doi$$a10.1093/neuonc/noaf105
000300620 0247_ $$2pmid$$apmid:40249111
000300620 0247_ $$2ISSN$$a1522-8517
000300620 0247_ $$2ISSN$$a1523-5866
000300620 0247_ $$2altmetric$$aaltmetric:176296467
000300620 037__ $$aDKFZ-2025-00834
000300620 041__ $$aEnglish
000300620 082__ $$a610
000300620 1001_ $$0P:(DE-He78)8aad075b17d93a5636a34942bdbd7ee6$$aSievers, Philipp$$b0$$eFirst author$$udkfz
000300620 245__ $$aMolecular signatures define BAP1-altered meningioma as a distinct CNS tumor with deregulation of Polycomb repressive complex target genes.
000300620 260__ $$aOxford$$bOxford Univ. Press$$c2025
000300620 3367_ $$2DRIVER$$aarticle
000300620 3367_ $$2DataCite$$aOutput Types/Journal article
000300620 3367_ $$0PUB:(DE-HGF)16$$2PUB:(DE-HGF)$$aJournal Article$$bjournal$$mjournal$$s1745328739_26886
000300620 3367_ $$2BibTeX$$aARTICLE
000300620 3367_ $$2ORCID$$aJOURNAL_ARTICLE
000300620 3367_ $$00$$2EndNote$$aJournal Article
000300620 500__ $$a#EA:B300#LA:B300# / epub
000300620 520__ $$aMeningiomas are the most common primary intracranial neoplasms, with highly variable patient outcomes. While most meningiomas are benign, a significant subset recurs postoperatively, presenting substantial treatment challenges. BAP1 gene inactivation has been suggested as a marker for aggressive meningiomas, although its precise molecular and clinical roles remain poorly understood.To comprehensively investigate BAP1-altered meningiomas, we used six meningiomas with known BAP1 alterations as a discovery set. Genome-wide DNA methylation profiling of these samples, along 11,151 reference meningiomas, identified a distinct molecular cluster (n = 42) using unsupervised visualization approaches. These tumors were further characterized by DNA/RNA sequencing, histopathological examination, and a retrospective review of clinical data, compared to reference meningioma cohorts, providing a thorough characterization of this rare tumor subtype.Our integrative analysis revealed BAP1-altered meningiomas as a distinct CNS tumor subtype, characterized by recurrent loss of chromosome 3p21 and driven by various BAP1-inactivating alterations. Although rhabdoid morphology is present in some cases, it is not exclusive and should not be used as a grading criterion. Progression-free survival analysis showed a median of 21 months (95% CI: 12-NA), with a 2-year overall survival rate of 79% (95% CI: 60%-100%), highlighting the aggressive nature of these tumors. Gene expression profiling revealed upregulation of PRC target genes, dysregulated Polycomb signaling, and elevated expression in several cellular and growth factor pathways.BAP1-altered meningiomas represent a distinct and aggressive CNS tumor subtype associated with PRC dysregulation and recurrent 3p chromosome loss. These findings support the designation 'meningioma, BAP1-altered.'
000300620 536__ $$0G:(DE-HGF)POF4-312$$a312 - Funktionelle und strukturelle Genomforschung (POF4-312)$$cPOF4-312$$fPOF IV$$x0
000300620 588__ $$aDataset connected to CrossRef, PubMed, , Journals: inrepo02.dkfz.de
000300620 650_7 $$2Other$$aBAP1
000300620 650_7 $$2Other$$aChromosome 3p loss
000300620 650_7 $$2Other$$aMeningioma
000300620 650_7 $$2Other$$aPolycomb signaling
000300620 650_7 $$2Other$$arhabdoid
000300620 7001_ $$aArora, Sonali$$b1
000300620 7001_ $$0P:(DE-He78)743a4a82daab55306a2c88b9f6bf8c2f$$aHielscher, Thomas$$b2$$udkfz
000300620 7001_ $$0P:(DE-He78)335b04cc04673d0893cb2572e920fe17$$aSavran, Dilan$$b3$$udkfz
000300620 7001_ $$0P:(DE-He78)e54a1e0999c1d8c95869ef9188b794cc$$aSchrimpf, Daniel$$b4$$udkfz
000300620 7001_ $$0P:(DE-He78)0e2d2ed45aacbf5e0b8a61172854671f$$aBanan, Rouzbeh$$b5$$udkfz
000300620 7001_ $$0P:(DE-He78)c0872917a9023422a66a7e1c8080446b$$aVonhören, David$$b6$$udkfz
000300620 7001_ $$0P:(DE-He78)f2efee17b6ca2f790176a2c036912536$$aPusch, Stefan$$b7$$udkfz
000300620 7001_ $$0P:(DE-He78)45440b44791309bd4b7dbb4f73333f9b$$aSill, Martin$$b8$$udkfz
000300620 7001_ $$aAppay, Romain$$b9
000300620 7001_ $$aWirsching, Hans-Georg$$b10
000300620 7001_ $$00000-0001-5732-7942$$aHortobagyi, Tibor$$b11
000300620 7001_ $$aDohmen, Hildegard$$b12
000300620 7001_ $$aAcker, Till$$b13
000300620 7001_ $$aKohlhof-Meinecke, Patricia$$b14
000300620 7001_ $$0P:(DE-He78)db2e8cf0dd8fa826896839ffee4b1411$$aSchweizer, Leonille$$b15$$udkfz
000300620 7001_ $$aWefers, Annika K$$b16
000300620 7001_ $$0P:(DE-He78)b15b56a6ed37417d476470c60c0140ff$$aHarter, Patrick$$b17
000300620 7001_ $$aHartmann, Christian$$b18
000300620 7001_ $$aBeschorner, Rudi$$b19
000300620 7001_ $$00000-0002-9168-6209$$aSchittenhelm, Jens$$b20
000300620 7001_ $$aBehling, Felix$$b21
000300620 7001_ $$aTabatabai, Ghazaleh$$b22
000300620 7001_ $$aMawrin, Christian$$b23
000300620 7001_ $$00000-0003-0752-0917$$aSnuderl, Matija$$b24
000300620 7001_ $$aMaas, Sybren L N$$b25
000300620 7001_ $$aWesseling, Pieter$$b26
000300620 7001_ $$00000-0002-8635-7195$$aBrandner, Sebastian$$b27
000300620 7001_ $$0P:(DE-He78)8d9c904a6cea14d4c99c78ba46e41f93$$aKorshunov, Andrey$$b28$$udkfz
000300620 7001_ $$0P:(DE-He78)ce9cbc0fcc94b04884d88105f812ed01$$aRatliff, Miriam$$b29$$udkfz
000300620 7001_ $$aKrieg, Sandro M$$b30
000300620 7001_ $$0P:(DE-He78)92e9783ca7025f36ce14e12cd348d2ee$$aWick, Wolfgang$$b31$$udkfz
000300620 7001_ $$0P:(DE-He78)551bb92841f634070997aa168d818492$$aJones, David T W$$b32$$udkfz
000300620 7001_ $$0P:(DE-He78)f746aa965c4e1af518b016de3aaff5d9$$aPfister, Stefan M$$b33$$udkfz
000300620 7001_ $$aHolland, Eric C$$b34
000300620 7001_ $$0P:(DE-He78)a8a10626a848d31e70cfd96a133cc144$$avon Deimling, Andreas$$b35$$udkfz
000300620 7001_ $$aSzulzewsky, Frank$$b36
000300620 7001_ $$0P:(DE-He78)a1f4b408b9155beb2a8f7cba4d04fe88$$aSahm, Felix$$b37$$eLast author$$udkfz
000300620 773__ $$0PERI:(DE-600)2094060-9$$a10.1093/neuonc/noaf105$$gp. noaf105$$pnn$$tNeuro-Oncology$$vnn$$x1522-8517$$y2025
000300620 909CO $$ooai:inrepo02.dkfz.de:300620$$pVDB
000300620 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)8aad075b17d93a5636a34942bdbd7ee6$$aDeutsches Krebsforschungszentrum$$b0$$kDKFZ
000300620 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)743a4a82daab55306a2c88b9f6bf8c2f$$aDeutsches Krebsforschungszentrum$$b2$$kDKFZ
000300620 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)335b04cc04673d0893cb2572e920fe17$$aDeutsches Krebsforschungszentrum$$b3$$kDKFZ
000300620 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)e54a1e0999c1d8c95869ef9188b794cc$$aDeutsches Krebsforschungszentrum$$b4$$kDKFZ
000300620 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)0e2d2ed45aacbf5e0b8a61172854671f$$aDeutsches Krebsforschungszentrum$$b5$$kDKFZ
000300620 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)c0872917a9023422a66a7e1c8080446b$$aDeutsches Krebsforschungszentrum$$b6$$kDKFZ
000300620 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)f2efee17b6ca2f790176a2c036912536$$aDeutsches Krebsforschungszentrum$$b7$$kDKFZ
000300620 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)45440b44791309bd4b7dbb4f73333f9b$$aDeutsches Krebsforschungszentrum$$b8$$kDKFZ
000300620 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)db2e8cf0dd8fa826896839ffee4b1411$$aDeutsches Krebsforschungszentrum$$b15$$kDKFZ
000300620 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)b15b56a6ed37417d476470c60c0140ff$$aDeutsches Krebsforschungszentrum$$b17$$kDKFZ
000300620 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)8d9c904a6cea14d4c99c78ba46e41f93$$aDeutsches Krebsforschungszentrum$$b28$$kDKFZ
000300620 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)ce9cbc0fcc94b04884d88105f812ed01$$aDeutsches Krebsforschungszentrum$$b29$$kDKFZ
000300620 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)92e9783ca7025f36ce14e12cd348d2ee$$aDeutsches Krebsforschungszentrum$$b31$$kDKFZ
000300620 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)551bb92841f634070997aa168d818492$$aDeutsches Krebsforschungszentrum$$b32$$kDKFZ
000300620 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)f746aa965c4e1af518b016de3aaff5d9$$aDeutsches Krebsforschungszentrum$$b33$$kDKFZ
000300620 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)a8a10626a848d31e70cfd96a133cc144$$aDeutsches Krebsforschungszentrum$$b35$$kDKFZ
000300620 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)a1f4b408b9155beb2a8f7cba4d04fe88$$aDeutsches Krebsforschungszentrum$$b37$$kDKFZ
000300620 9131_ $$0G:(DE-HGF)POF4-312$$1G:(DE-HGF)POF4-310$$2G:(DE-HGF)POF4-300$$3G:(DE-HGF)POF4$$4G:(DE-HGF)POF$$aDE-HGF$$bGesundheit$$lKrebsforschung$$vFunktionelle und strukturelle Genomforschung$$x0
000300620 9141_ $$y2025
000300620 915__ $$0StatID:(DE-HGF)0420$$2StatID$$aNationallizenz$$d2024-12-11$$wger
000300620 915__ $$0StatID:(DE-HGF)0100$$2StatID$$aJCR$$bNEURO-ONCOLOGY : 2022$$d2024-12-11
000300620 915__ $$0StatID:(DE-HGF)0200$$2StatID$$aDBCoverage$$bSCOPUS$$d2024-12-11
000300620 915__ $$0StatID:(DE-HGF)0300$$2StatID$$aDBCoverage$$bMedline$$d2024-12-11
000300620 915__ $$0StatID:(DE-HGF)0199$$2StatID$$aDBCoverage$$bClarivate Analytics Master Journal List$$d2024-12-11
000300620 915__ $$0StatID:(DE-HGF)0160$$2StatID$$aDBCoverage$$bEssential Science Indicators$$d2024-12-11
000300620 915__ $$0StatID:(DE-HGF)1110$$2StatID$$aDBCoverage$$bCurrent Contents - Clinical Medicine$$d2024-12-11
000300620 915__ $$0StatID:(DE-HGF)0113$$2StatID$$aWoS$$bScience Citation Index Expanded$$d2024-12-11
000300620 915__ $$0StatID:(DE-HGF)0150$$2StatID$$aDBCoverage$$bWeb of Science Core Collection$$d2024-12-11
000300620 915__ $$0StatID:(DE-HGF)9915$$2StatID$$aIF >= 15$$bNEURO-ONCOLOGY : 2022$$d2024-12-11
000300620 9202_ $$0I:(DE-He78)B300-20160331$$kB300$$lKKE Neuropathologie$$x0
000300620 9201_ $$0I:(DE-He78)B300-20160331$$kB300$$lKKE Neuropathologie$$x0
000300620 9201_ $$0I:(DE-He78)C060-20160331$$kC060$$lC060 Biostatistik$$x1
000300620 9201_ $$0I:(DE-He78)B062-20160331$$kB062$$lB062 Pädiatrische Neuroonkologie$$x2
000300620 9201_ $$0I:(DE-He78)HD01-20160331$$kHD01$$lDKTK HD zentral$$x3
000300620 9201_ $$0I:(DE-He78)FM01-20160331$$kFM01$$lDKTK Koordinierungsstelle Frankfurt$$x4
000300620 9201_ $$0I:(DE-He78)MU01-20160331$$kMU01$$lDKTK Koordinierungsstelle München$$x5
000300620 9201_ $$0I:(DE-He78)B320-20160331$$kB320$$lKKE Neuroonkologie$$x6
000300620 9201_ $$0I:(DE-He78)B360-20160331$$kB360$$lPädiatrische Gliomforschung$$x7
000300620 9200_ $$0I:(DE-He78)B300-20160331$$kB300$$lKKE Neuropathologie$$x0
000300620 980__ $$ajournal
000300620 980__ $$aVDB
000300620 980__ $$aI:(DE-He78)B300-20160331
000300620 980__ $$aI:(DE-He78)C060-20160331
000300620 980__ $$aI:(DE-He78)B062-20160331
000300620 980__ $$aI:(DE-He78)HD01-20160331
000300620 980__ $$aI:(DE-He78)FM01-20160331
000300620 980__ $$aI:(DE-He78)MU01-20160331
000300620 980__ $$aI:(DE-He78)B320-20160331
000300620 980__ $$aI:(DE-He78)B360-20160331
000300620 980__ $$aUNRESTRICTED