ArticleJournal of neuropathology and experimental neurology2021
Polymorphous Low-Grade Neuroepithelial Tumor of the Young (PLNTY): Molecular Profiling Confirms Frequent MAPK Pathway Activation.
Article in Journal of neuropathology and experimental neurology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers, 2 of them syntheses that pooled it.
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Who cites it
23 citing papers in PubMed, 2 syntheses or guidelines pooled it, 32 citations in OpenAlex.
- Systematic literature review of the epidemiology of neurotrophic tyrosine receptor kinase positive solid tumor sites.Future oncology (London, England) · 2025Pooled it
- Systematic review and cumulative analysis of clinical properties of BRAF V600E mutations in PLNTY histological samples.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2024Pooled it
- Article
- Identification of novel FGFR2::TXLNB Fusion in a polymorphous low-grade neuroepithelial tumor of the young.Brain pathology (Zurich, Switzerland) · 2026Article
- From a Polymorphous Low-Grade Neuroepithelial Tumor to a Glioblastoma in an Adult Patient with FGFR3-TACC3 Fusion: A Case Report and Literature Review of the Molecular Profile.Current oncology (Toronto, Ont.) · 2026Review
- Genomic analysis of PLNTY-like tumor progression into epithelioid glioblastoma: a case report.Acta neuropathologica communications · 2026Article
- Polymorphic low-grade neuroepithelial tumor of the young and treatment of epilepsy: a case report.Frontiers in oncology · 2026Article
- A rare tumor with unique features: polymorphous low-grade neuroepithelial tumor of the young with third ventricular location and TP53 mutation. Illustrative case.Journal of neurosurgery. Case lessons · 2025Article
- Molecular profile of adult primary leptomeningeal gliomatosis aligns with glioblastoma, IDH-wildtype.Brain pathology (Zurich, Switzerland) · 2025Article
- Review
- Targeted therapy for pediatric glioma: RAF(t)ing in the molecular era.World journal of pediatrics : WJP · 2025Review
- Cortically Based Brain Tumors in Children: A Decision-Tree Approach in the Radiology Reading Room.AJNR. American journal of neuroradiology · 2025Review
- Phenotypic and epigenetic heterogeneity in FGFR2-fused glial and glioneuronal tumours.Neuropathology and applied neurobiology · 2024Article
- MAPK pathway alterations in polymorphous low-grade neuroepithelial tumor of the young: diagnostic considerations.Brain tumor pathology · 2024Article
- Paediatric low-grade glioma: the role of classical pathology in integrated diagnostic practice.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2024Review
- Polymorphous Low-Grade Neuroepithelial Tumor of the Young (PLNTY): Scoping Review of Case Reports and Case Series.Asian journal of neurosurgery · 2024Article
- Glioma.Nature reviews. Disease primers · 2024Review
- Expanding the Imaging Spectrum of Polymorphous Low-Grade Neuroepithelial Tumor of the Young in Children.AJNR. American journal of neuroradiology · 2024Article
- Polymorphous low-grade neuroepithelial tumour of young (PLNTY): the new kid on the block.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2024Article
- Adult-type and Pediatric-type Diffuse Gliomas : What the Neuroradiologist Should Know.Clinical neuroradiology · 2023Review
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Authors and funding
15 authors at 4 institutions in 1 country.
Funding
Abstract
Polymorphous low-grade neuroepithelial tumor of the young (PLNTY) is a recently described epileptogenic tumor characterized by oligodendroglioma-like components, aberrant CD34 expression, and frequent mitogen-activated protein kinase (MAPK) pathway activation. We molecularly profiled 13 cases with diagnostic histopathological features of PLNTY (10 female; median age, 16 years; range, 5-52). Patients frequently presented with seizures (9 of 12 with available history) and temporal lobe tumors (9 of 13). MAPK pathway activating alterations were identified in all 13 cases. Fusions were present in the 7 youngest patients: FGFR2-CTNNA3 (n = 2), FGFR2-KIAA1598 (FGFR2-SHTN1) (n = 1), FGFR2-INA (n = 1), FGFR2-MPRIP (n = 1), QKI-NTRK2 (n = 1), and KIAA1549-BRAF (n = 1). BRAF V600E mutation was present in 6 patients (17 years or older). Two fusion-positive cases additionally harbored TP53/RB1 abnormalities suggesting biallelic inactivation. Copy number changes predominantly involving whole chromosomes were observed in all 10 evaluated cases, with losses of chromosome 10q occurring with FGFR2-KIAA1598 (SHTN1)/CTNNA3 fusions. The KIAA1549-BRAF and QKI-NTRK2 fusions were associated respectively with a 7q34 deletion and 9q21 duplication. This study shows that despite its name, PLNTY also occurs in older adults, who frequently show BRAF V600E mutation. It also expands the spectrum of the MAPK pathway activating alterations associated with PLNTY and demonstrates recurrent chromosomal copy number changes consistent with chromosomal instability.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.