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Cell ; 164(5): 1060-1072, 2016 Feb 25.
Article in English | MEDLINE | ID: mdl-26919435

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.


Subject(s)
Central Nervous System Neoplasms/genetics , Central Nervous System Neoplasms/pathology , DNA Methylation , Neuroectodermal Tumors/genetics , Neuroectodermal Tumors/pathology , Amino Acid Sequence , Central Nervous System Neoplasms/classification , Central Nervous System Neoplasms/diagnosis , Child , Forkhead Transcription Factors/genetics , Gene Expression Profiling , Gene Expression Regulation, Neoplastic , Humans , Molecular Sequence Data , Neuroectodermal Tumors/classification , Neuroectodermal Tumors/diagnosis , Proto-Oncogene Proteins/chemistry , Proto-Oncogene Proteins/genetics , Repressor Proteins/chemistry , Repressor Proteins/genetics , Signal Transduction , Trans-Activators , Tumor Suppressor Proteins/genetics
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