Multinodular and Vacuolating Neuronal Tumors of the Cerebrum: A Systematic Review of the Literature.

Multinodular and vacuolating neuronal tumors (MVNTs) of the cerebrum are rare, seizure-related, low-grade tumors of the central nervous system that 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. Because of their benign nature and indolent clinical course, radiologically 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 the characteristics of MVNTs 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 techniques, they usually appear hypointense in T1-weighted images and hyperintense in T2-weighted and fluid-attenuated inversion recovery images and lack perilesional edema or postcontrast enhancement. MVNTs do not seem to change size or recur, even after partial resection of the tumor, indicating their indolent course, and, thus, surveillance with serial magnetic resonance imaging is the most appropriate management technique for these lesions.