Multinodular and vacuolating neuronal tumor (MVNT) of the central nervous system

Multinodular and Vacuolating Neuronal Tumor (MVNT) is a rare central nervous system tumor, first described by Huse et al. in 2013. MVNTs are often asymptomatic and discovered incidentally, but can manifest as seizures, especially in adults. They present as a well-defined cluster of nodular lesions located on the subcortical white matter, most commonly in the temporal lobe. Histopathologically, MVNTs exhibit a multinodular architecture, characterized by vacuolated tumor cells expressing a subset of neuronal antigens. Genetically, MVNTs show activating alterations in the MAP kinase signaling pathway, distinguished by the absence of canonical mutations. MVNTs are considered benign (WHO Grade 1), nonaggressive, and typically do not require biopsy.