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Background: Multinodular Vacuolating Neuronal Tumors (MVNTs) are mixed glial–neuronal brain lesions classified as World Health Organization (WHO) CNS grade 1 tumors, often associated with long-term epilepsy. First described by Huse et al. in 2013 and included in the WHO CNS classification in 2016, MVNTs present a range of clinical manifestations, from symptomatic to asymptomatic. They typically affect young to middle-aged adults and exhibit diverse presentations. Radiologically, MVNTs are usually supratentorial, frequently located in the temporal lobe but also observed in the frontal and parietal lobes. MRI is essential for diagnosis, revealing multiple coalescing subcortical or cortical nodules with hyperintense signals on T2-weighted/FLAIR sequences, often without peripheral edema or mass effects. Case Reports: This paper presents two cases: one symptomatic MVNT with significant clinical manifestations, and the other documenting an incidental finding of MVNT in an asymptomatic patient. One case shows typical temporal lobe localization, while the other highlights a rare frontal lobe localization, with clear radiological findings on T2/FLAIR sequences. Conclusions: These cases illustrate the varied clinical presentations of MVNTs and emphasize MRI’s critical role in diagnosis and management. Asymptomatic cases often require conservative management, stressing the avoidance of unnecessary invasive procedures and the importance of regular monitoring.
Background: Multinodular Vacuolating Neuronal Tumors (MVNTs) are mixed glial–neuronal brain lesions classified as World Health Organization (WHO) CNS grade 1 tumors, often associated with long-term epilepsy. First described by Huse et al. in 2013 and included in the WHO CNS classification in 2016, MVNTs present a range of clinical manifestations, from symptomatic to asymptomatic. They typically affect young to middle-aged adults and exhibit diverse presentations. Radiologically, MVNTs are usually supratentorial, frequently located in the temporal lobe but also observed in the frontal and parietal lobes. MRI is essential for diagnosis, revealing multiple coalescing subcortical or cortical nodules with hyperintense signals on T2-weighted/FLAIR sequences, often without peripheral edema or mass effects. Case Reports: This paper presents two cases: one symptomatic MVNT with significant clinical manifestations, and the other documenting an incidental finding of MVNT in an asymptomatic patient. One case shows typical temporal lobe localization, while the other highlights a rare frontal lobe localization, with clear radiological findings on T2/FLAIR sequences. Conclusions: These cases illustrate the varied clinical presentations of MVNTs and emphasize MRI’s critical role in diagnosis and management. Asymptomatic cases often require conservative management, stressing the avoidance of unnecessary invasive procedures and the importance of regular monitoring.
Objective: Multinodular vacuolating neuronal tumor (MVNT) is a rare entity that radiologists should recognize based on its unique imaging characteristics. We aimed to present the imaging findings of 26 patients diagnosed with MVNT. Materials and Methods: The brain MRI findings of 26 patients with a pre-diagnosis of multinodular vacuolating neuronal tumor (MVNT) were retrospectively evaluated across five centers. Conventional MRI sequences were used for all patients. Additionally, diffusion MRI images were obtained for 25 patients, and contrast-enhanced sequences were performed on 19 patients. Results: The mean age of the patients was 39.7 years. The lesion was located in the cerebrum in 25 patients (96.15%) and in the cerebellum in 1 patient (3.85%). When classified by location, the most common site was the left frontal lobe, observed in 6 patients (23.07%). In 1 patient (3.85%), the lesion was located in the left cerebellum, classified as MVNT/MV PLUS. The long axis of the lesion was measured in the axial plane, with an average size of 18.6 mm. On T1-weighted images (T1W), the lesion was isointense in 23 patients (88.46%) and hypointense in 3 patients (11.54%). On T2-weighted (T2W) and FLAIR sequences, the lesion was hyperintense in all patients. Diffusion-weighted images were obtained in 25 patients; on b1000 sequences, the lesion was hyperintense in 20 patients (80%), isointense in 4 patients (16%), and hypointense in 1 patient (4%). Contrast-enhanced sequences were acquired in 19 patients, with no enhancement observed in any case. Conclusion: Conventional MRI is the primary imaging modality for diagnosing multinodular vacuolating neuronal tumor (MVNT), as its imaging features are characteristic. In addition to these features, diffusion imaging may aid in the diagnosis of MVNT.
A multinodular and vacuolating neuronal tumor (MVNT) is a rare non-malign central nervous system tumor. The cerebral cortex of young to middle-aged adults is affected and seizures and focal neurological deficits could reveal it. Despite being considered a low-grade tumor, MVNT can be challenging to diagnose and manage due to its histopathological similarities to other neuronal tumors. The limited number of reported cases makes it difficult to establish definitive guidelines for treatment and follow-up. Given the relatively recent discovery of MVNT, there is a need for further research to better understand its pathogenesis, optimal treatment and long-term strategies. Furthermore, studying MVNT can contribute to the broader understanding of brain tumor biology and the intricacies of tumor heterogeneity. This paper explores the challenges and perspectives surrounding MVNT to improve clinical decision-making and patient outcomes. Based on the present narrative review, advancements in diagnostic technologies have shown promise in addressing the challenges associated with diagnosing MVNTs. Liquid biopsy is an innovative and non-invasive diagnostic tool that analyzes biomarkers to provide information about tumors, and radiomics is a method that extracts quantitative data from medical images to provide detailed insights into tumor characteristics. Such innovative diagnostic technologies are important because they have the potential to significantly improve the current diagnostic landscape for MVNTs, allowing for earlier detection, accurate classification, and personalized treatment strategies.
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