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Neuroradiological features of the polymorphous low-grade neuroepithelial tumor of the young: five new cases with a systematic review of the literature

  • Paediatric Neuroradiology
  • Published:
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Abstract

Purpose

Polymorphous low-grade neuroepithelial tumors of the young (PLNTY) is a newly recognized brain tumor with genetic abnormalities frequently involving either BRAF or FGFR2/FGFR3. There are few publications available about the neuroradiological features of PLNTY. In this systematic review, we assessed the demographic, clinical, and neuroradiological features of PLNTY.

Methods

Literature data were extracted from database searches in MEDLINE and SCOPUS databases up to June 10, 2021. Studies reporting on pathologically proven PLNTY with neuroradiological findings were included. After reviewing 103 abstracts, 9 articles encompassing 19 cases met the inclusion criteria. We also added five patients from our hospital. The correlations between the presence of “transmantle-like sign” and the following three factors: duration of seizures; tumor size; and pathologically proven cortical dysplasia, were examined.

Results

The median patient age was 15.5 years (range, 5–57 years), and 15/24 (62.5%) were female. All tumors were localized supratentorialy. The main radiological features included cortical or subcortical masses (95.8%) in the temporal lobe (66.7%), calcification (83.3%), well-defined margins (72.7%), solid and cystic components (66.6%), and T2-weighted imaging (T2WI) hyperintensity (50.0%). The duration of seizure was significantly longer (positive vs. negative (median [range]), 24 months [6 − 96 months] vs. 5 months [1 − 12 months], p = 0.042), and the presence of the cortical dysplasia was significantly more frequent (3/8 vs 0/16, p = 0.042) in the patients with transmantle-like sign.

Conclusion

PLNTY typically represents a calcified, well-defined mass in the supratentorial cortical or subcortical regions. The radiological findings defined here could facilitate the diagnosis of PLNTY.

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Availability of data and material

The underlying data can be accessed at reasonable request to the corresponding author.

Code availability

Not applicable.

Abbreviations

ADC:

Apparent diffusion coefficient

BRAF:

B-Raf proto-oncogene

CD34:

Cluster of differentiation 34

FGFR:

Fibroblast growth factor receptor

FLAIR:

Fluid-attenuated inversion recovery

MAPK:

Mitogen-activated protein kinase

MRI:

Magnetic resonance imaging

PLNTY:

Polymorphous low-grade neuroepithelial tumor of the young

SWI:

Susceptibility-weighted imaging

T2WI:

T2-weighted imaging

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Acknowledgements

We would like to thank Ms. Dobbs Danielle for her illustration representing the characteristic imaging findings of PLNTY.

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Authors and Affiliations

  1. Division of Neuroradiology, Department of Radiology, University of Michigan, 1500 E Medical Center Dr, UH B2, Ann Arbor, MI, 48109, USA

    Mariko Kurokawa, Ryo Kurokawa, Aristides A. Capizzano, Akira Baba, Yoshiaki Ota, Ashok Srinivasan & Toshio Moritani

  2. Department of Pathology, University of Michigan, Ann Arbor, MI, USA

    Emile Pinarbasi

  3. Department of Biostatistics, School of Public Health, University of Michigan, 1415 Washington Heights, Ann Arbor, MI, 48109, USA

    Timothy Johnson

Authors
  1. Mariko Kurokawa
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Contributions

Conceptualization: Mariko Kurokawa, Ryo Kurokawa.

Data curation: Mariko Kurokawa, Ryo Kurokawa, Akira Baba, Pinarbasi Emile.

Methodology: Mariko Kurokawa, Ryo Kurokawa, Akira Baba, Timothy Johnson, Ashok Srinivasan.

Formal analysis and investigation: Mariko Kurokawa, Ryo Kurokawa, Akira Baba, Timothy Johnson.

Writing—original draft preparation: Mariko Kurokawa.

Writing—review and editing: Ryo Kurokawa, Aristides A. Capizzano, Akira Baba, Pinarbasi Emile, Yoshiaki Ota, Timothy Johnson, Ashok Srinivasan, Toshio Moritani.

Validation: Toshio Moritani.

Supervision: Toshio Moritani.

Corresponding author

Correspondence to Ryo Kurokawa.

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All authors declare that they have no competing interests.

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The study was approved by the ethics committee of the University of Michigan, in accordance with a waiver of informed consent. Data were de-identified prior to any analysis.

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Written informed consent was waived for this HIPAA-compliant retrospective study.

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All authors gave consent for publication.

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Kurokawa, M., Kurokawa, R., Capizzano, A.A. 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 64, 1255–1264 (2022). https://doi.org/10.1007/s00234-021-02879-5

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  • DOI: https://doi.org/10.1007/s00234-021-02879-5

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