Abstract
Background/Objectives: Multinodular and vacuolating neuronal tumors (MNVTs) are a type of recently identified benign neuroepithelial tumor with debated malformative or neoplastic origins. This review summarizes their neuroanatomical localization, imaging, histopathology, immunohistochemistry, and diagnostic challenges. Methods: A systematic review of PUBMED/MEDLINE was performed in December 2024. Results: Of 118 screened articles, 39 were eligible, covering 299 patients. MNVTs are often asymptomatic "leave me alone" lesions, discovered incidentally, though nonspecific symptoms (59.9%) and seizures (19.7%) are reported. Immunohistochemistry reveals variable profiles, reflecting complex cellular differentiation. The characteristic "bubble-like" MRI pattern along the subcortical ribbon and superficial white matter is a reliable diagnostic feature. Rare cortical involvement and atypical band-like lesions occur. MRI signal intensity varies. Over a mean follow-up of 36 months, lesions were stable or non-recurrent, with only one case of progression. Conclusion: MVNT imaging mimics other glioneuronal lesions, but reliable diagnostic MRI features include a "bubble-clustered" appearance, lack of cortical involvement, absence of enhancement, and temporal lobe predominance. Hemodynamic and metabolic properties support the diagnosis. Most lesions remain stable, requiring no treatment. Surgical resection is reserved for cases with uncontrolled seizures or atypical locations where the diagnosis is unclear.