TY - JOUR
T1 - Polymorphous low-grade neuroepithelial tumor of the young (PLNTY)
T2 - an epileptogenic neoplasm with oligodendroglioma-like components, aberrant CD34 expression, and genetic alterations involving the MAP kinase pathway
AU - Huse, Jason T.
AU - Snuderl, Matija
AU - Jones, David T.W.
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.
N1 - Publisher Copyright:
© 2016, The Author(s).
PY - 2017/3/1
Y1 - 2017/3/1
N2 - 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.
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.
KW - BRAF
KW - Epilepsy
KW - FGFR2
KW - FGFR3
KW - Low-grade neuroepithelial tumor
KW - Oligodendroglioma
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U2 - 10.1007/s00401-016-1639-9
DO - 10.1007/s00401-016-1639-9
M3 - Article
C2 - 27812792
AN - SCOPUS:84994300077
SN - 0001-6322
VL - 133
SP - 417
EP - 429
JO - Acta neuropathologica
JF - Acta neuropathologica
IS - 3
ER -