Multinodular and Vacuolating Neuronal Tumor of the Cerebrum (MVNT): A case series and review of the literature

Buffa, Geraldine Belén; Cháves, Hernán; Serra, Mercedes; Stefanoff, Nadia; Gagliardo, Allan Salvatore; Yañez, Paulina

doi:10.1016/j.neurad.2019.05.010

Cited by 27 publications

(17 citation statements)

References 13 publications

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“…MVNT is defined nowadays by the WHO as a benign tumor associated with seizures. These tumors have a typical radiological pattern described as FLAIR and T2-WI hyperintense lesions, clustered in multiple small nodules, affecting subcortial white matter surrounded by normal-appearing parenchyma [13,28]. The WHO has subsumed this variant, therefore, into the group of gangliocytoma and not yet assigned a grading [43].…”

Section: New Histomorphological Entitiesmentioning

confidence: 99%

Low-grade developmental and epilepsy associated brain tumors: a critical update 2020

Slegers

Blümcke

2020

acta neuropathol commun

133

185

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Brain tumors represent the second most frequent etiology in patients with focal seizure onset before 18 years of age and submitted to epilepsy surgery. Hence, this category of brain tumors, herein defined as low-grade, developmental, epilepsy-associated brain tumors (LEAT) is different from those frequently encountered in adults as (A): 77% of LEAT occur in the temporal lobe; (B): the vast majority of LEAT are of low malignancy and classified as WHO I°; (C): LEAT are often composed of mixed glial and neuronal cell components and present with variable growth patterns including small cysts or nodules; (D): LEAT do not share common gene driving mutations, such as IDH1 or 1p/19q co-deletions. Characteristic entities comprise the ganglioglioma (GG), the dysembryoplastic neuroepithelial tumor (DNT), the angiocentric glioma (AG), the isomorphic diffuse glioma (IDG) and the papillary glio-neuronal tumor (PGNT), representing 73.2% of 1680 tumors collected in a large German series of 6747 patients submitted to epilepsy surgery. In the realm of exciting discoveries of genetic drivers of brain tumors new genes have been also reported for LEAT. BRAF V600E mutations were linked to GG with CD34 expression, FGFR1 mutations to DNT, MYB alterations to AG and also IDG and PRKCA fusions to PGNT, suggesting the possibility to also develop a genetically driven tumor classification scheme for LEAT. Rare availability of LEAT in a single center is a challenging obstacle, however, to systematically unravel the neurobiological nature and clinical behavior of LEAT. Other challenges in need of clarification include malignant tumor progression of LEAT entities, seizure relapse in patients following bulk tumor resection and the controversial issue of associated focal cortical dysplasia as additional pathomechanism. In order to advance our understanding and promote reliable diagnostic work-up of LEAT, we recommend, therefore, international collaboration to achieve our goals.

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Section: New Histomorphological Entitiesmentioning

confidence: 99%

Low-grade developmental and epilepsy associated brain tumors: a critical update 2020

Slegers

Blümcke

2020

acta neuropathol commun

133

185

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“…First reported in 2013, MVNTs are sporadic benign brain tumors [ 1 , 3 , 6 ]. MVNTs have been associated with ganglion cell tumors found in the cortical white matter [ 3 ]. Very few cases of MVNT have been reported so far.…”

Section: Discussionmentioning

confidence: 99%

“…In 2016, MVNT was classified by the World Health Organization Classification of Tumors of the Central Nervous System [ 2 ]. Most of the MVNT cases reported yet have been in middle-aged patients with neurological symptoms including headaches and seizures [ 3 - 6 ]. Surgical intervention is often indicated in MVNTs related to epileptic symptoms [ 3 - 6 ].…”

Section: Introductionmentioning

confidence: 99%

“…Most of the MVNT cases reported yet have been in middle-aged patients with neurological symptoms including headaches and seizures [ 3 - 6 ]. Surgical intervention is often indicated in MVNTs related to epileptic symptoms [ 3 - 6 ]. However, asymptomatic cases of MVNT have rarely been reported, suggesting MVNT may go undiagnosed, and therefore surgical intervention is infrequently necessary [ 5 ].…”

Section: Introductionmentioning

confidence: 99%

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Multinodular and Vacuolating Neuronal Tumor: Incidental Diagnosis of a Rare Brain Lesion

Arbuiso¹,

Roster²,

Gill³

et al. 2021

Cureus

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Multinodular and vacuolating neuronal tumor (MVNT) is a rare benign brain lesion, commonly found in middle-aged adults. The patients experience a range of symptoms from being asymptomatic to epileptic seizures, with headache being the most common symptom. Here we report a case of an incidental diagnosis of MVNT in a young female. A 25-year-old female with a past medical history of occasional headaches without seizures or any focal neurological deficit presented after a motor vehicle rollover. The MRI brain revealed an incidental finding of a subcortical lesion in the right parietal lobe with T2-FLAIR (fluid-attenuated inversion recovery) hyperintensity between the cystic portions, indicative of a possible MVNT, with a less probable chance of dysembryoplastic neuroepithelial tumor based on the subcortical location of the lesion. No neurosurgical intervention was recommended. With one-year follow-up, no changes were noted on neuroimaging, and the patient remained stable without any neurological symptoms. The MVNT is a rare brain lesion that presents with benign features. In patients with epileptic symptoms, surgical resection of the lesion can be curative. However, in asymptomatic patients, careful monitoring may be sufficient, as described in this case.

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“…They are characterized by multiple tumor nodules, vacuolar alteration, and widespread immunolabeling for human neuronal protein HuC/HuD. These tumors appear as a cluster of well-circumscribed high T2 signal 'bubbles' located predominantly in the subcortical white matter but can involve the overlying cortex (56).…”

Section: Multinodular and Vacuolating Neuronal Tumor (Mvnt)mentioning

confidence: 99%

Narrative review of epilepsy: getting the most out of your neuroimaging

Vito¹,

Mankad²,

Pujar³

et al. 2021

Transl Pediatr

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Neuroimaging represents an important step in the evaluation of pediatric epilepsy. The crucial role of brain imaging in the diagnosis, follow-up and presurgical assessment of patients with epilepsy is noted and has to be familiar to all neuroradiologists and trainees approaching pediatric brain imaging.Morphological qualitative imaging shows the majority of cerebral lesions/alterations underlying focal epilepsy and can highlight some features which are useful in the differential diagnosis of the different types of epilepsy. Recent advances in MRI acquisitions including diffusion-weighted imaging (DWI), postacquisition image processing techniques, and quantification of imaging data are increasing the accuracy of lesion detection during the last decades. Functional MRI (fMRI) can be really useful and helps to identify cortical eloquent areas that are essential for language, motor function, and memory, and diffusion tensor imaging (DTI) can reveal white matter tracts that are vital for these functions, thus reducing the risk of epilepsy surgery causing new morbidities. Also positron emission tomography (PET), single photon emission computed tomography (SPECT), simultaneous electroencephalogram (EEG) and fMRI, and electrical and magnetic source imaging can be used to assess the exact localization of epileptic foci and help in the design of intracranial EEG recording strategies. The main role of these "hybrid" techniques is to obtain quantitative and qualitative informations, a necessary step to evaluate and demonstrate the complex relationship between abnormal structural and functional data and to manage a "patient-tailored" surgical approach in epileptic patients.

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Multinodular and Vacuolating Neuronal Tumor of the Cerebrum (MVNT): A case series and review of the literature

Cited by 27 publications

References 13 publications

Low-grade developmental and epilepsy associated brain tumors: a critical update 2020

Low-grade developmental and epilepsy associated brain tumors: a critical update 2020

Multinodular and Vacuolating Neuronal Tumor: Incidental Diagnosis of a Rare Brain Lesion

Narrative review of epilepsy: getting the most out of your neuroimaging

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