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

Download
Author
Huse, JT; Snuderl, M; Jones, DTW; Brathwaite, CD; Altman, N; Lavi, E; Saffery, R; Sexton-Oates, A; Blumcke, I; Capper, D; ...Date
2017-03-01Source Title
Acta NeuropathologicaPublisher
SPRINGERAffiliation
Paediatrics (RCH)Medicine and Radiology
Metadata
Show full item recordDocument Type
Journal ArticleCitations
Huse, J. T., Snuderl, M., Jones, D. T. W., Brathwaite, C. D., Altman, N., Lavi, E., Saffery, R., Sexton-Oates, A., Blumcke, I., Capper, D., Karajannis, M. A., Benayed, R., Chavez, L., Thomas, C., Serrano, J., Borsu, L., Ladanyi, M. & Rosenblum, M. K. (2017). 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. ACTA NEUROPATHOLOGICA, 133 (3), pp.417-429. https://doi.org/10.1007/s00401-016-1639-9.Access Status
Open AccessAbstract
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.
Export Reference in RIS Format
Endnote
- Click on "Export Reference in RIS Format" and choose "open with... Endnote".
Refworks
- Click on "Export Reference in RIS Format". Login to Refworks, go to References => Import References