Atypical presentation of polymorphous low-grade neuroepithelial tumor of young (PLNTY): a case report

Abstract

Abstract The polymorphous low-grade neuroepithelial tumor of young (PLNTY) is considered as one among the low-grade neuroepithelial tumor; as per WHO-2021 classification of Brain Tumors in the fifth edition. The term PLNTY was first coined by Huse in 2016. These morphologically variable tumors are characterized by their oligodendroglioma-like cellular components, infiltrative growth pattern, and Cluster of Differentiation 34 (CD34) immunopositivity. Frequent genetic abnormalities involving mitogen-activated protein kinase pathway constituents like the BRAF proto-oncogene or fibroblast growth receptor 2/3 are harbored by PLNTYs. Radiologically, these are found to be well-circumscribed lesions with calcified and cystic components, affecting primarily temporal lobes. Clinically, they present with seizures/epilepsy in young adults (< 30 years). In the present manuscript we are reporting a case of 37-year-old male, presenting with a gradually progressive headache for 6 months, found to have a left frontal multiloculated cystic lesion with dystrophic calcifications. Based on the distinctive histopathological feature of the oligodendroglioma-like infiltrative lesion showing CD34 immunopositivity, a diagnosis of a polymorphous low-grade neuroepithelial tumor of young was made.

Keywords

• Polymorphous low-grade neuroepithelial tumor of young
• PLNTY
• CD34
• MAPK pathway
• Low-grade epilepsy-associated tumors