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Journal Articles Cell Year : 2016

New Brain Tumor Entities Emerge from Molecular Classification of CNS-PNETs

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David Capper
  • Function : Author
  • PersonId : 916728
Marc Remke
  • Function : Author

Abstract

Primitive neuroectodermal tumors of the central nervous system (CNS-PNETs) are highly aggressive, poorly differentiated embryonal tumors occurring predominantly in young children but also affecting adolescents and adults. Herein, we demonstrate that a significant proportion of institutionally diagnosed CNS-PNETs display molecular profiles indistinguishable from those of various other well-defined CNS tumor entities, facilitating diagnosis and appropriate therapy for patients with these tumors. From the remaining fraction of CNS-PNETs, we identify four new CNS tumor entities, each associated with a recurrent genetic alteration and distinct histopathological and clinical features. These new molecular entities, designated “CNS neuroblastoma with FOXR2 activation (CNS NB-FOXR2),” “CNS Ewing sarcoma family tumor with CIC alteration (CNS EFT-CIC),” “CNS high-grade neuroepithelial tumor with MN1 alteration (CNS HGNET-MN1),” and “CNS high-grade neuroepithelial tumor with BCOR alteration (CNS HGNET-BCOR),” will enable meaningful clinical trials and the development of therapeutic strategies for patients affected by poorly differentiated CNS tumors.
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Dates and versions

hal-01480124 , version 1 (20-03-2017)

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Dominik Sturm, Brent A.Orr, Umut H.Toprak, Volker Hovestadt, David T. W. Jones, et al.. New Brain Tumor Entities Emerge from Molecular Classification of CNS-PNETs. Cell, 2016, 164, pp.1060-1072. ⟨10.1016/j.cell.2016.01.015⟩. ⟨hal-01480124⟩
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