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000127673 1001_ $$0P:(DE-He78)8d9c904a6cea14d4c99c78ba46e41f93$$aKorshunov, Andrey$$b0$$eFirst author$$udkfz
000127673 245__ $$aEmbryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma, and medulloepithelioma share molecular similarity and comprise a single clinicopathological entity.
000127673 260__ $$aBerlin$$bSpringer$$c2014
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000127673 520__ $$aThree histological variants are known within the family of embryonal rosette-forming neuroepithelial brain tumors. These include embryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma (EBL), and medulloepithelioma (MEPL). In this study, we performed a comprehensive clinical, pathological, and molecular analysis of 97 cases of these rare brain neoplasms, including genome-wide DNA methylation and copy number profiling of 41 tumors. We identified uniform molecular signatures in all tumors irrespective of histological patterns, indicating that ETANTR, EBL, and MEPL comprise a single biological entity. As such, future WHO classification schemes should consider lumping these variants into a single diagnostic category, such as embryonal tumor with multilayered rosettes (ETMR). We recommend combined LIN28A immunohistochemistry and FISH analysis of the 19q13.42 locus for molecular diagnosis of this tumor category. Recognition of this distinct pediatric brain tumor entity based on the fact that the three histological variants are molecularly and clinically uniform will help to distinguish ETMR from other embryonal CNS tumors and to better understand the biology of these highly aggressive and therapy-resistant pediatric CNS malignancies, possibly leading to alternate treatment strategies.
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000127673 7001_ $$0P:(DE-He78)a46a5b2a871859c8e2d63d2f8c666807$$aSturm, Dominik$$b1$$udkfz
000127673 7001_ $$aRyzhova, Marina$$b2
000127673 7001_ $$0P:(DE-He78)744146d3b5a3df1e0ac555e5bf1ee5cc$$aHovestadt, Volker$$b3$$udkfz
000127673 7001_ $$aGessi, Marco$$b4
000127673 7001_ $$0P:(DE-He78)551bb92841f634070997aa168d818492$$aJones, David$$b5$$udkfz
000127673 7001_ $$0P:(DE-HGF)0$$aRemke, Marc$$b6
000127673 7001_ $$0P:(DE-HGF)0$$aNorthcott, Paul$$b7
000127673 7001_ $$aPerry, Arie$$b8
000127673 7001_ $$aPicard, Daniel$$b9
000127673 7001_ $$aRosenblum, Marc$$b10
000127673 7001_ $$aAntonelli, Manila$$b11
000127673 7001_ $$aAronica, Eleonora$$b12
000127673 7001_ $$aSchüller, Ulrich$$b13
000127673 7001_ $$aHasselblatt, Martin$$b14
000127673 7001_ $$aWoehrer, Adelheid$$b15
000127673 7001_ $$aZheludkova, Olga$$b16
000127673 7001_ $$aKumirova, Ella$$b17
000127673 7001_ $$aPuget, Stephanie$$b18
000127673 7001_ $$aTaylor, Michael D$$b19
000127673 7001_ $$aGiangaspero, Felice$$b20
000127673 7001_ $$aPeter Collins, V.$$b21
000127673 7001_ $$0P:(DE-He78)a8a10626a848d31e70cfd96a133cc144$$avon Deimling, Andreas$$b22$$udkfz
000127673 7001_ $$0P:(DE-He78)e13b4363c5fe858044ef8a39c02c870c$$aLichter, Peter$$b23$$udkfz
000127673 7001_ $$aHuang, Annie$$b24
000127673 7001_ $$aPietsch, Torsten$$b25
000127673 7001_ $$0P:(DE-He78)f746aa965c4e1af518b016de3aaff5d9$$aPfister, Stefan$$b26$$udkfz
000127673 7001_ $$0P:(DE-He78)4c28e2aade5f44d8eca9dd8e97638ec8$$aKool, Marcel$$b27$$eLast author$$udkfz
000127673 773__ $$0PERI:(DE-600)1458410-4$$a10.1007/s00401-013-1228-0$$gVol. 128, no. 2, p. 279 - 289$$n2$$p279 - 289$$tActa neuropathologica$$v128$$x1432-0533$$y2014
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