PDF herunterladen
CC BY-NC-ND 4.0 · Asian J Neurosurg 2024; 19(02): 126-136
DOI: 10.1055/s-0044-1786700
Review Article

Polymorphous Low-Grade Neuroepithelial Tumor of the Young (PLNTY): Scoping Review of Case Reports and Case Series

Daulat Singh
1   Department of Radiotherapy and Clinical Oncology, Government Doon Medical College, Dehradun, Uttarakhand, India
,
Vijay P. Joshi
2   Sparsh Neuro and Superspeciality Hospital, Solapur, Maharashtra, India
,
Sanjeev Pattankar
3   Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
,
Ved Prakash Maurya 
3   Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
,
Rakesh Mishra
4   Department of Neurosurgery, Institute of Medical Sciences, Trauma Centre and Mahamana Centenary Superspeciality Hospital, Banaras Hindu University, Varanasi, Uttar Pradesh, India
,
Rafael Cincu
5   Department of Neurosurgery, General University Hospital, Valencia, Spain
,
Luis Rafael Moscote-Salazar 
6   Neurocritical Care, Colombian Clinical Research Group in Neurocritical Care, Bogota, Colombia
,
Amit Agrawal 
7   Department of Neurosurgery, All India Institute of Medical Sciences, Saket Nagar, Bhopal, Madhya Pradesh, India
› Institutsangaben Funding None.
› Weitere Informationen
Auch verfügbar auf
   Lizenzen und Reprints

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 - pseudorosetting

Note

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.




Publikationsverlauf

Artikel online veröffentlicht:
27. Mai 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/)

Thieme Medical and Scientific Publishers Pvt. Ltd.
A-12, 2nd Floor, Sector 2, Noida-201301 UP, India

 

  • References

  • 1 Huse JT, Snuderl M, Jones DT. et al. Polymorphous low-grade neuroepithelial tumor of the young (PLNTY): an epileptogenic neoplasm with oligodendroglioma-like components, aberrant CD34 expression, and genetic alterations involving the MAP kinase pathway. Acta Neuropathol 2017; 133 (03) 417-429
    CrossrefPubMedGoogle Scholar
  • 2 Fei X, Zhao J, Wei W. et al. Clinical, radiological, pathological features and seizure outcome with surgical management of polymorphous low-grade neuroepithelial tumor of the young associated with epilepsy. Front Oncol 2022; 12: 863373
    CrossrefPubMedGoogle Scholar
  • 3 Arksey H, O'Malley L. Scoping studies: towards a methodological framework. Int J Soc Res Methodol 2005; 8: 19-32
    CrossrefGoogle Scholar
  • 4 Mays N, Roberts E, Popay J. Synthesising research evidence. In: Fulop N, Allen P, Clarke A, Black N. eds. Studying the Organisation and Delivery of Health Services: Research Methods. London: Routledge; 2001
    Google Scholar
  • 5 Khalil H, Peters M, Godfrey CM, McInerney P, Soares CB, Parker D. An evidence-based approach to scoping reviews. Worldviews Evid Based Nurs 2016; 13 (02) 118-123
    CrossrefPubMedGoogle Scholar
  • 6 Tricco AC, Lillie E, Zarin W. et al. PRISMA extension for scoping reviews (PRISMA-ScR): checklist and explanation. Ann Intern Med 2018; 169 (07) 467-473
    CrossrefPubMedGoogle Scholar
  • 7 Bale TA, Sait SF, Benhamida J. et al. Malignant transformation of a polymorphous low grade neuroepithelial tumor of the young (PLNTY). Acta Neuropathol 2021; 141 (01) 123-125
    CrossrefPubMedGoogle Scholar
  • 8 Benson JC, Summerfield D, Carr C. et al. Polymorphous low-grade neuroepithelial tumor of the young as a partially calcified intra-axial mass in an adult. AJNR Am J Neuroradiol 2020; 41 (04) 573-578
    CrossrefPubMedGoogle Scholar
  • 9 Bitar M, Danish SF, Rosenblum MK. A newly diagnosed case of polymorphous low-grade neuroepithelial tumor of the young. Clin Neuropathol 2018; 37 (04) 178-181
    CrossrefPubMedGoogle Scholar
  • 10 Broggi G, Certo F, Altieri R, Caltabiano R, Gessi M, Barbagallo GMV. A “polymorphous low-grade neuroepithelial tumor of the young (PLNTY)” diagnosed in an adult. Report of a case and review of the literature. Surg Neurol Int 2021; 12: 470
    CrossrefPubMedGoogle Scholar
  • 11 Chen Y, Tian T, Guo X. et al. Polymorphous low-grade neuroepithelial tumor of the young: case report and review focus on the radiological features and genetic alterations. BMC Neurol 2020; 20 (01) 123
    CrossrefPubMedGoogle Scholar
  • 12 Danyeli AE, Altinoz MA, Sari R, Elmaci I. Polymorphous low-grade neuroepithelial tumor of the young: a detailed pathomolecular analysis and discussion of a case. Clin Neuropathol 2021; 40 (05) 271-278
    CrossrefPubMedGoogle Scholar
  • 13 Ge R, Fang H, Yin X, Liu C, Chang Y. Case report of low-grade neuroepithelial tumour with oligodendroglioma-like components and CD34 expression. Pathology 2021; 53 (04) 547-549
    CrossrefPubMedGoogle Scholar
  • 14 Gupta R, Lucas CG, Wu J. et al. Low-grade glioneuronal tumors with FGFR2 fusion resolve into a single epigenetic group corresponding to 'Polymorphous low-grade neuroepithelial tumor of the young'. Acta Neuropathol 2021; 142 (03) 595-599
    CrossrefPubMedGoogle Scholar
  • 15 Gupta VR, Giller C, Kolhe R, Forseen SE, Sharma S. Polymorphous low-grade neuroepithelial tumor of the young: a case report with genomic findings. World Neurosurg 2019; 132: 347-355
    CrossrefPubMedGoogle Scholar
  • 16 Hendrych M, Hemza J, Kočvarová J. et al. Polymorphous low-grade neuroepithelial tumor of the young. Ces Slov Neurol Neurochir 2021; 84/117: 282-285
    Google Scholar
  • 17 Ida CM, Johnson DR, Nair AA. et al. Polymorphous low-grade neuroepithelial tumor of the young (PLNTY): molecular profiling confirms frequent MAPK pathway activation. J Neuropathol Exp Neurol 2021; 80 (09) 821-829
    CrossrefPubMedGoogle Scholar
  • 18 Johnson DR, Giannini C, Jenkins RB, Kim DK, Kaufmann TJ. Plenty of calcification: imaging characterization of polymorphous low-grade neuroepithelial tumor of the young. Neuroradiology 2019; 61 (11) 1327-1332
    CrossrefPubMedGoogle Scholar
  • 19 Kamalboor H, Dababo A, Alkhateeb M. Polymorphous low-grade neuroepithelial tumor of the young (PLNTY): case report and literature review. J Neurol Sci 2019; 405: 84
    CrossrefGoogle Scholar
  • 20 Kikuchi K, Togao O, Yamashita K. et al. Diagnostic accuracy for the epileptogenic zone detection in focal epilepsy could be higher in FDG-PET/MRI than in FDG-PET/CT. Eur Radiol 2021; 31 (05) 2915-2922
    CrossrefPubMedGoogle Scholar
  • 21 Kurokawa M, Kurokawa R, Capizzano AA. et al. Neuroradiological features of the polymorphous low-grade neuroepithelial tumor of the young: five new cases with a systematic review of the literature. Neuroradiology 2022; 64 (06) 1255-1264
    CrossrefPubMedGoogle Scholar
  • 22 Lelotte J, Duprez T, Raftopoulos C, Michotte A. Polymorphous low-grade neuroepithelial tumor of the young: case report of a newly described histopathological entity. Acta Neurol Belg 2020; 120 (03) 729-732
    CrossrefPubMedGoogle Scholar
  • 23 Palejwala AH, O'Neal CM, Quinton MR, Battiste JD, Peterson JEG, Dunn IF. Polymorphous low-grade neuroepithelial tumor of the young: rare tumor and review of the literature. Rare Tumors 2022 ;14:20363613221083360
    PubMedGoogle Scholar
  • 24 Riva G, Cima L, Villanova M. et al. Low-grade neuroepithelial tumor: unusual presentation in an adult without history of seizures. Neuropathology 2018; 38 (05) 557-560
    CrossrefPubMedGoogle Scholar
  • 25 Sumdani H, Shahbuddin Z, Harper G, Hamilton L. Case report of rarely described polymorphous low-grade neuroepithelial tumor of the young and comparison with oligodendroglioma. World Neurosurg 2019; 127: 47-51
    CrossrefPubMedGoogle Scholar
  • 26 Surrey LF, Jain P, Zhang B. et al. Genomic analysis of dysembryoplastic neuroepithelial tumor spectrum reveals a diversity of molecular alterations dysregulating the MAPK and PI3K/mTOR pathways. J Neuropathol Exp Neurol 2019; 78 (12) 1100-1111
    CrossrefPubMedGoogle Scholar
  • 27 Tateishi K, Ikegaya N, Udaka N. et al. BRAF V600E mutation mediates FDG-methionine uptake mismatch in polymorphous low-grade neuroepithelial tumor of the young. Acta Neuropathol Commun 2020; 8 (01) 139
    CrossrefPubMedGoogle Scholar
  • 28 Zhang K, Sassoon A. Polymorphous low-grade neuroepithelial tumor of the young (PLNTY): two cases of the recently described epileptogenic neoplasm with oligodendroglioma-like components and heavy calcification. Am J Clin Pathol 2018; 150 (Suppl. 01) S16-S17
    CrossrefGoogle Scholar
  • 29 Gilani A, Davies K, Kleinschmidt-DeMasters B. Histological features in pediatric central nervous system tumors with FGFR alterations. Folia Neuropathol 2020; 58 (04) 347-356
    CrossrefPubMedGoogle Scholar
  • 30 Zhao S, Wang J, Li MN, Ding Y, Pan MH, Song K. [Clinicopathological features of polymorphous low-grade neuroepithelial tumor of the young]. Zhonghua Bing Li Xue Za Zhi 2022; 51 (07) 640-646
    PubMedGoogle Scholar
  • 31 Ge R, Fang HF, Chang YQ, Li Z, Liu CF. [Clinicopathological features of polymorphous low-grade neuroepithelial tumor of the young]. Zhonghua Bing Li Xue Za Zhi 2020; 49 (11) 1131-1135
    PubMedGoogle Scholar
  • 32 Bale TA, Rosenblum MK. The 2021 WHO classification of tumors of the central nervous system: an update on pediatric low-grade gliomas and glioneuronal tumors. Brain Pathol 2022; 32 (04) e13060
    CrossrefPubMedGoogle Scholar
  • 33 Joshi V, Chougule MV, Mudkanna A. et al. Atypical presentation of polymorphous low-grade neuroepithelial tumor of young (PLNTY): a case report. Egypt J Neurosurg 2023; 38: 7
    CrossrefGoogle Scholar
  • 34 Métais A, Appay R, Pagès M. et al. Contributors of the Biopathology RENOCLIP-LOC network. Low-grade epilepsy-associated neuroepithelial tumours with a prominent oligodendroglioma-like component: The diagnostic challenges. Neuropathol Appl Neurobiol 2022; 48 (02) e12769
    CrossrefPubMedGoogle Scholar
  • 35 Slocum C, Hoffman C, Solomon J, Pisapia D. Abstract 197 – Cerebellar Tumor with FGFR2-KIAA1598 Fusion and Features of Polymorphous Low-grade Neuroepithelial Tumor of the Young. American Association of Neuropathologists, Inc. Abstracts of the 98th Annual Meeting June 9–12, 2022 Bonita Springs, Florida. Journal of Neuropathology & Experimental Neurology 2022; 81 (06) 494-495
    Google Scholar
  • 36 Cases-Cunillera S, van Loo KMJ, Pitsch J. et al. Heterogeneity and excitability of BRAFV600E-induced tumors is determined by Akt/mTOR-signaling state and Trp53-loss. Neuro Oncol 2022; 24 (05) 741-754
    CrossrefPubMedGoogle Scholar
  • 37 Golub D, Lynch DG, Pan PC. et al. Polymorphous low-grade neuroepithelial tumor of the young with FGFR3-TACC3 fusion mimicking high-grade glioma: case report and series of high-grade correlates. Front Oncol 2023; 21 (13) 1307591
    CrossrefPubMedGoogle Scholar
  • 38 Vandenheuvel J, Zinn D. A case of polymorphous low-grade neuroepithelial tumor of the young with polysomy of chromosome 1 and 19 lacking BRAF mutation. Poster presented at Lehigh Valley Health Network, Allentown, PA..
    Google Scholar
  • 39 Majeed M, McMillan C, Jo J, Lee K, Sippey C, Trembath D, Boyer P. Abstract 165 – Polymorphous Low-Grade Neuroepithelial Tumor of the Young in a 47-Year-Old Man Presenting with Headache and Visual Deficits. American Association of Neuropathologists, Inc. Abstracts of the 97th Annual Meeting June 10–13, 2021 St. Louis, Missouri. Journal of Neuropathology & Experimental Neurology 2021; 80 (06) 600-601
    Google Scholar
  • 40 Sharma S, Kolhe R, Giller C. Abstract 169 – Polymorphous low-grade neuroepithelial tumor of the young: a case report with genomic findings. American Association of Neuropathologists, Inc. Abstracts of the 94th Annual Meeting June 7–10, 2018 Louisville, Kentucky. Journal of Neuropathology & Experimental Neurology 2018; 77 (06) 522
    Google Scholar
  • 41 Vornetti G, Marucci G, Zenesini C. et al. Relationship among clinical, pathological and bio-molecular features in low-grade epilepsy-associated neuroepithelial tumors. J Clin Neurosci 2017; 44: 158-163
    CrossrefPubMedGoogle Scholar
  • 42 Bale TA. FGFR- gene family alterations in low-grade neuroepithelial tumors. Acta Neuropathol Commun 2020; 8 (01) 21
    CrossrefPubMedGoogle Scholar
  • 43 Blumcke I, Aronica E, Urbach H, Alexopoulos A, Gonzalez-Martinez JA. A neuropathology-based approach to epilepsy surgery in brain tumors and proposal for a new terminology use for long-term epilepsy-associated brain tumors. Acta Neuropathol 2014; 128 (01) 39-54
    CrossrefPubMedGoogle Scholar
  • 44 Blümcke I, Thom M, Aronica E. et al. The clinicopathologic spectrum of focal cortical dysplasias: a consensus classification proposed by an ad hoc Task Force of the ILAE Diagnostic Methods Commission. Epilepsia 2011; 52 (01) 158-174
    CrossrefPubMedGoogle Scholar