TY  - JOUR
AU  - Huse, Jason T
AU  - Snuderl, Matija
AU  - Jones, David
AU  - Brathwaite, Carole D
AU  - Altman, Nolan
AU  - Lavi, Ehud
AU  - Saffery, Richard
AU  - Sexton-Oates, Alexandra
AU  - Blumcke, Ingmar
AU  - Capper, David
AU  - Karajannis, Matthias A
AU  - Benayed, Ryma
AU  - Chavez, Lukas
AU  - Thomas, Cheddhi
AU  - Serrano, Jonathan
AU  - Borsu, Laetitia
AU  - Ladanyi, Marc
AU  - Rosenblum, Marc K
TI  - Polymorphous 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.
JO  - Acta neuropathologica
VL  - 133
IS  - 3
SN  - 1432-0533
CY  - Berlin
PB  - Springer
M1  - DKFZ-2017-01241
SP  - 417 - 429
PY  - 2017
AB  - Epileptogenic 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.
LB  - PUB:(DE-HGF)16
C6  - pmid:27812792
C2  - pmc:PMC5325850
DO  - DOI:10.1007/s00401-016-1639-9
UR  - https://inrepo02.dkfz.de/record/124362
ER  -