A newly diagnosed case of polymorphous low-grade neuroepithelial tumor of the young

Polymorphous low-grade neuroepithelial tumor of the young (PLNTY) is a recently described variant of low-grade neuroepithelial tumors that exhibits infiltrative growth, histopathological variability with frequently prominent oligodendroglioma-like components, intense labeling for CD34, absence of 1P/19Q codeletion, a distinct DNA methylation signature and genetic alterations involving MAP kinase pathway constituents of either the B-Raf proto-oncogene BRAF or fibroblast growth factor receptors 2 or 3 (FGFR2 and FGFR3). We here report a newly diagnosed case of PLNTY involving the temporal lobe in a 31-year-old man with chronic focal epilepsy. This tumor had histologic and immunophenotypic features similar to the recently described PLNTY and proved BRAF V600E mutant. Biomolecular profiling is becoming increasingly important in characterizing neuroepithelial tumors. Furthermore, biomolecular features such as CD34 expression and BRAF mutation have been reported to be significantly associated with the clinical behavior of these tumors. Like other low-grade neuroepithelial tumors, PLNTYs appear to be generally indolent with excellent seizure relief after total surgical resection. It is important to recognize cases of PLNTY in order to guide clinical management including the indication for surgery.