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DOI: 10.1055/s-0044-1786700
Polymorphous Low-Grade Neuroepithelial Tumor of the Young (PLNTY): Scoping Review of Case Reports and Case Series
Authors
Funding None.
Abstract
Polymorphous low-grade neuroepithelial tumor of the young (PLNTY) is considered one of the low-grade neuroepithelial tumors, as per the World Health Organization 2021 classification of brain tumors. First described in 2016, these morphologically variable tumors are characterized by oligodendroglioma-like cellular components, infiltrative growth patterns, and cluster of differentiation 34 immunopositivity. A literature search of the PubMed/MEDLINE, SCOPUS, ScienceDirect, and COCHRANE databases (from inception to 20th June 2022) was carried out to identify relevant studies. To identify additional studies, we performed a recursive search of the bibliographies of the selected articles and published systematic reviews on this topic. The search yielded a total of 64 results. After removing duplicates, 26 articles were eligible for the review. The diagnostic criteria for these glioneuronal variants, representing a broad neuropathological spectrum, are not distinct and hence impede proper diagnosis and prognosis. Frequent genetic abnormalities involving mitogen-activated protein kinase pathway constituents, such as B-Raf proto-oncogene or fibroblast growth receptor 2/3, are harbored by PLNTYs. Recent advances in molecular diagnostics have resulted in more accurate tumor classification systems, based on gene expression profiles and DNA methylation patterns. Gross total resection seems curative, with a low recurrence rate. Malignant transformation is rare; however, adjuvant radiation therapy and chemotherapy may be beneficial in selected cases.
Keywords
polymorphous low-grade neuroepithelial tumor of the young (PLNTY) - scoping review - low-grade neuroepithelial tumor - calcification - pseudorosettingNote
PLNTY has emerged as one of the differential diagnoses for low-grade glioma. The diagnostic criteria and optimum management are not well defined in view of rarity of the condition. Well-circumscribed tumors situated in temporal lobe, with variegated appearance, central calcifications, and peripherally located cysts with minimal mass effects are characteristic radiological features. Gross total resection seems curative, with a low recurrence rate.
Publication History
Article published online:
27 May 2024
© 2024. Asian Congress of Neurological Surgeons. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)
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