Journal article
Clinical significance of molecular subgroups of polymorphous low-grade neuroepithelial tumor of the young (PLNTY): a small single institutional case series and integrated analysis
Pathology, research and practice, Vol.252, 154922
12/2023
DOI: 10.1016/j.prp.2023.154922
PMID: 37984047
Abstract
Introduction
Polymorphous low-grade neuroepithelial tumor of the young (PLNTY) is a recently described entity. The clinicopathological features and prognosis of the molecular subgroups of these rare tumors is poorly understood. In this study, we presented a small case series of three new cases and integrated the data with published cases in the literature to characterize the similarities and differences of molecular subgroups of PLNTY.
Methods
We searched our institutional archive for PLNTY cases and searched PubMed and Web of Science for relevant data. Demographic, clinical, radiologic, histopathological, molecular, and follow-up data of our four cases with published cases were integrated for final analyses.
Results
We identified three institutional cases of PLNTY. The median age of our patients was 17 years (range: 13–42). All patients had a prior history of chronic seizures and all had tumors affecting the temporal lobes. Histopathologically, all cases showed oligodendroglial-like morphology with intratumoral calcifications and at least partially infiltrative growth patterns. Tumor cells were immunoreactive with CD34 and GFAP. Genetically, all cases harbored BRAF V600E mutations. Integrated analyses, including a total of 67 cases, demonstrated that PLNTYs with FGFR2 mutation were significantly younger (median age 11.0 years) than those with BRAF V600E or FGFR3 fusions (median age 41.0 and 16.0 years, respectively). All BRAF V600E-positive PLNTYs were free of tumor recurrence, while four of PLNTYs in other molecular subgroups developed tumor recurrence by imaging.
Conclusion
Our study suggests that PLNTYs have distinct clinicopathological features and are driven by genetic alterations in the MAPK pathway. The molecular subgroups of PLNTYs share similar findings, but also demonstrate distinct patient demographics.
Details
- Title: Subtitle
- Clinical significance of molecular subgroups of polymorphous low-grade neuroepithelial tumor of the young (PLNTY): a small single institutional case series and integrated analysis
- Creators
- Huy Gia Vuong - University of Iowa Hospitals and ClinicsEyas Alzayadneh - University of Iowa Hospitals and ClinicsThomas P. Reith - University of Iowa Hospitals and ClinicsKathryn L. Eschbacher - University of Iowa
- Resource Type
- Journal article
- Publication Details
- Pathology, research and practice, Vol.252, 154922
- DOI
- 10.1016/j.prp.2023.154922
- PMID
- 37984047
- ISSN
- 0344-0338
- eISSN
- 1618-0631
- Language
- English
- Electronic publication date
- 11/2023
- Date published
- 12/2023
- Academic Unit
- Pathology
- Record Identifier
- 9984511954602771
Metrics
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