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024 7 _ |a 10.1016/j.cell.2016.01.015
|2 doi
024 7 _ |a pmid:26919435
|2 pmid
024 7 _ |a pmc:PMC5139621
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024 7 _ |a 0092-8674
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024 7 _ |a 1097-4172
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024 7 _ |a altmetric:6004472
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037 _ _ |a DKFZ-2017-05705
041 _ _ |a eng
082 _ _ |a 570
100 1 _ |a Sturm, Dominik
|0 P:(DE-He78)a46a5b2a871859c8e2d63d2f8c666807
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245 _ _ |a New Brain Tumor Entities Emerge from Molecular Classification of CNS-PNETs.
260 _ _ |a [Cambridge, Mass.]
|c 2016
|b Cell Press
336 7 _ |a article
|2 DRIVER
336 7 _ |a Output Types/Journal article
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336 7 _ |a Journal Article
|b journal
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|s 1522144110_18537
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336 7 _ |a ARTICLE
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336 7 _ |a JOURNAL_ARTICLE
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336 7 _ |a Journal Article
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520 _ _ |a 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.
536 _ _ |a 312 - Functional and structural genomics (POF3-312)
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650 _ 7 |a BCOR protein, human
|2 NLM Chemicals
650 _ 7 |a CIC protein, human
|2 NLM Chemicals
650 _ 7 |a FOXR2 protein, human
|2 NLM Chemicals
650 _ 7 |a Forkhead Transcription Factors
|2 NLM Chemicals
650 _ 7 |a MN1 protein, human
|2 NLM Chemicals
650 _ 7 |a Proto-Oncogene Proteins
|2 NLM Chemicals
650 _ 7 |a Repressor Proteins
|2 NLM Chemicals
650 _ 7 |a Tumor Suppressor Proteins
|2 NLM Chemicals
700 1 _ |a Orr, Brent A
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700 1 _ |a Toprak, Umut
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700 1 _ |a Hovestadt, Volker
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700 1 _ |a Buchhalter, Ivo
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700 1 _ |a Northcott, Paul A
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700 1 _ |a Leis, Irina
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700 1 _ |a Ryzhova, Marina
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700 1 _ |a Pfaff, Elke
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700 1 _ |a Allen, Sariah J
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700 1 _ |a Balasubramanian, Gnanaprakash
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773 _ _ |a 10.1016/j.cell.2016.01.015
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