Polymorphous low-grade neuroepithelial tumor of the young: Rare tumor and review of the literature

Ali H Palejwala; Christen M O'Neal; Michael R Quinton; James D Battiste; Jo Elle G Peterson; Ian F Dunn

doi:10.1177/20363613221083360

Polymorphous low-grade neuroepithelial tumor of the young: Rare tumor and review of the literature

Rare Tumors. 2022 Mar 28:14:20363613221083360. doi: 10.1177/20363613221083360. eCollection 2022.

Authors

Ali H Palejwala¹, Christen M O'Neal¹, Michael R Quinton², James D Battiste¹, Jo Elle G Peterson², Ian F Dunn¹

Affiliations

¹ Department of Neurosurgery, University of Oklahoma Health Sciences Center, Oklahoma City, Ok, USA.
² Department of Pathology, University of Oklahoma Health Sciences Center, Oklahoma City, Ok, USA.

Abstract

Polymorphous low-grade neuroepithelial tumor of the young (PLNTY) is a recently described low-grade neuroepithelial tumor with an infiltrative growth pattern and oligodendrocyte-like cells that are CD34 immunopositive. Correlating histology and results from molecular testing is critical to correctly diagnosing PLNTY, as its histologic appearance is similar to oligodendrogliomas and shares genetic abnormalities common to other low-grade epilepsy associated tumors (LEATs). In this case report, we describe a 31-year-old female with intractable epilepsy found to have a temporal mass and diagnosed with PLNTY after histopathologic and molecular testing. We describe the radiographic, histologic, and genetic features in relation to the epileptic and oncologic outcomes for this patient. Then, we compare these features and outcomes to other cases of PLNTY described in the literature.

Keywords: BRAF; Polymorphous low-grade neuroepithelial tumor of the young; fibroblast growth receptor2; fibroblast growth receptor3; low-grade epilepsy associated tumors; molecular analysis.

Publication types

Case Reports