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

• Published in: Neuroradiology Vol. 64; no. 6; pp. 1255 - 1264
• Main Authors: Kurokawa, Mariko, Kurokawa, Ryo, Capizzano, Aristides A., Baba, Akira, Ota, Yoshiaki, Pinarbasi, Emile, Johnson, Timothy, Srinivasan, Ashok, Moritani, Toshio
• Format: Journal Article
• Language: English
• Published: Berlin/Heidelberg Springer Berlin Heidelberg 01-06-2022; Springer Nature B.V
• Subjects: Abnormalities; Adolescent; Adult; Brain cancer; Brain Neoplasms - diagnostic imaging; Brain Neoplasms - genetics; Brain tumors; Calcification; Child; Child, Preschool; Dysplasia; Feature extraction; Female; Fibroblast growth factor receptor 2; Fibroblast growth factor receptors; Genetic abnormalities; Humans; Imaging; Literature reviews; Magnetic Resonance Imaging; Male; Malformations of Cortical Development; Medicine; Medicine & Public Health; Middle Aged; Neoplasms, Neuroepithelial - diagnostic imaging; Neuroimaging; Neurology; Neuroradiology; Neurosciences; Neurosurgery; Paediatric Neuroradiology; Patients; Radiology; Seizures; Systematic review; Temporal lobe; Tumors; Young Adult; Systematic review; Computed tomography; Magnetic resonance imaging; Polymorphous low-grade neuroepithelial tumor of the young (PLNTY)
• ISSN: 0028-3940; 1432-1920
• DOI: 10.1007/s00234-021-02879-5

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.