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

Huse JT, Snuderl M, Jones DTW, Brathwaite CD, Altman N, Lavi E, Saffery R, Sexton-Oates A, Blümcke I, Capper D, Karajannis MA, Benayed R, Chavez L, Thomas C, Serrano J, Borsu L, Ladanyi M, Rosenblum MK (2017)

Publication Type: Journal article

Publication year: 2017

Journal

Acta Neuropathologica Springer Verlag (Germany)

Book Volume: 133

Pages Range: 417-429

Journal Issue: 3

DOI: 10.1007/s00401-016-1639-9

Abstract

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.

Authors with CRIS profile

Ingmar Blümcke Lehrstuhl für Neuropathologie

Involved external institutions

Memorial Sloan Kettering Cancer Center US United States (USA) (US) New York University (NYU) US United States (USA) (US) Deutsches Krebsforschungszentrum (DKFZ) DE Germany (DE) Ruprecht-Karls-Universität Heidelberg DE Germany (DE) Royal Children's Hospital Parkville AU Australia (AU) Cornell University US United States (USA) (US) Miami Children's Hospital US United States (USA) (US) University of Texas MD Anderson Cancer Center US United States (USA) (US)

How to cite

APA:

Huse, J.T., Snuderl, M., Jones, D.T.W., Brathwaite, C.D., Altman, N., Lavi, E.,... 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), 417-429. https://doi.org/10.1007/s00401-016-1639-9

MLA:

Huse, Jason T., et al. "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 (2017): 417-429.

BibTeX: Download