• Nikolaos Vlachos, Marios Lampros, George A Alexiou, Efrosini Styliara, Vassileios Xydis, Spyridon Voulgaris, and Maria I Argyropoulou.
• Department of Neurosurgery, University of Ioannina, School of Medicine, Ioannina, Greece; Department of General Surgery, General Hospital of Ioannina "G. Hatzikosta", Ioannina, Greece.
• World Neurosurg. 2024 Dec 12.

AbstractMultinodular and Vacuolating Neuronal Tumors of the cerebrum (MVNTs) are rare, seizure-related, low-grade tumors of the Central Nervous System (CNS) which usually affect young adults. First described by Huse et al. in 2013, these neoplasms are usually located within the deep cortical ribbon and the superficial white matter and have a characteristic cytoarchitecture of cells with neuronal and glial differentiation that form multiple nodules with conspicuous vacuolation. Due to their benign nature and indolent clinical course, its radiological-based differentiation from other entities is of paramount importance to avoid unnecessary surgical intervention. To the best of our knowledge, our study represents the first systematic review in the literature aiming to delineate MVNTs' characteristics regarding epidemiology, clinical manifestation, histopathology, imaging, and management. PubMed/MEDLINE and SCOPUS databases were systematically investigated for MVNT cases until November 2023. The search yielded 29 case reports comprising 41 patients with a mean age of 32.6 years and 7 case series with 164 patients. MVNTs were most commonly located in the supratentorial compartment affecting the temporal, frontal or parietal lobes. Their most frequent initial clinical manifestation was either seizures or headaches. On conventional Magnetic Resonance Imaging (MRI) techniques, they usually appear hypointense in T1-weighted images and hyperintense in T2-weighted and FLAIR images, while lacking perilesional edema or post-contrast enhancement. MVNTs do not appear to change size or recur, even after partial resection of the tumor, indicating their indolent course and thus, surveillance with serial MRI scans immerses as the most appropriate management technique for these lesions.Copyright © 2024. Published by Elsevier Inc.

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