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000124362 0247_ $$2ISSN$$a1432-0533
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000124362 037__ $$aDKFZ-2017-01241
000124362 041__ $$aeng
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000124362 1001_ $$00000-0003-4440-3250$$aHuse, Jason T$$b0
000124362 245__ $$aPolymorphous low-grade neuroepithelial tumor of the young (PLNTY): an epileptogenic neoplasm with oligodendroglioma-like components, aberrant CD34 expression, and genetic alterations involving the MAP kinase pathway.
000124362 260__ $$aBerlin$$bSpringer$$c2017
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000124362 520__ $$aEpileptogenic tumors affecting children and young adults are a morphologically diverse collection of neuroepithelial neoplasms that, as a group, exhibit varying levels of glial and/or neuronal differentiation. Recent advances in molecular profiling technology, including comprehensive DNA sequencing and methylation analysis, have enabled the application of more precise and biologically relevant classification schemes to these tumors. In this report, we describe a morphologically and molecularly distinct epileptogenic neoplasm, the polymorphous low-grade neuroepithelial tumor of the young (PLNTY), which likely accounts for a sizable portion of oligodendroglioma-like tumors affecting the pediatric population. Characteristic microscopic findings most notably include infiltrative growth, the invariable presence of oligodendroglioma-like cellular components, and intense immunolabeling for cluster of differentiation 34 (CD34). Moreover, integrative molecular profiling reveals a distinct DNA methylation signature for PLNTYs, along with frequent genetic abnormalities involving either B-Raf proto-oncogene (BRAF) or fibroblast growth factor receptors 2 and 3 (FGFR2, FGFR3). These findings suggest that PLNTY represents a distinct biological entity within the larger spectrum of pediatric, low-grade neuroepithelial tumors.
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000124362 7001_ $$aSnuderl, Matija$$b1
000124362 7001_ $$0P:(DE-He78)551bb92841f634070997aa168d818492$$aJones, David$$b2
000124362 7001_ $$aBrathwaite, Carole D$$b3
000124362 7001_ $$aAltman, Nolan$$b4
000124362 7001_ $$aLavi, Ehud$$b5
000124362 7001_ $$aSaffery, Richard$$b6
000124362 7001_ $$aSexton-Oates, Alexandra$$b7
000124362 7001_ $$aBlumcke, Ingmar$$b8
000124362 7001_ $$0P:(DE-He78)51bf9ae9cb5771b30c483e5597ef606c$$aCapper, David$$b9$$udkfz
000124362 7001_ $$aKarajannis, Matthias A$$b10
000124362 7001_ $$aBenayed, Ryma$$b11
000124362 7001_ $$0P:(DE-HGF)0$$aChavez, Lukas$$b12
000124362 7001_ $$aThomas, Cheddhi$$b13
000124362 7001_ $$aSerrano, Jonathan$$b14
000124362 7001_ $$aBorsu, Laetitia$$b15
000124362 7001_ $$aLadanyi, Marc$$b16
000124362 7001_ $$aRosenblum, Marc K$$b17
000124362 773__ $$0PERI:(DE-600)1458410-4$$a10.1007/s00401-016-1639-9$$gVol. 133, no. 3, p. 417 - 429$$n3$$p417 - 429$$tActa neuropathologica$$v133$$x1432-0533$$y2017
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