Natural history of lesions with the MR imaging appearance of multinodular and vacuolating neuronal tumor

Multinodular and vacuolating neuronal tumor (MVNT) is a new pattern of neuronal tumour included in the recently revised WHO 2016 classification of tumors of the CNS. There are 15 reports in the literature to date. They are typically associated with late onset epilepsy and a neoplastic vs. malformative biology has been questioned. We present a series of ten cases and compare their pathological and genetic features to better characterized epilepsy‐associated malformations including focal cortical dysplasia type II (FCDII) and low‐grade epilepsy‐associated tumors (LEAT). Clinical and neuroradiology data were reviewed and a broad immunohistochemistry panel was applied to explore neuronal and glial differentiation, interneuronal populations, mTOR pathway activation and neurodegenerative changes. Next generation sequencing was performed for targeted multi‐gene analysis to identify mutations common to epilepsy lesions including FCDII and LEAT. All of the surgical cases in this series presented with seizures, and were located in the temporal lobe. There was a lack of any progressive changes on serial pre‐operative MRI and a mean age at surgery of 45 years. The vacuolated cells of the lesion expressed mature neuronal markers (neurofilament/SMI32, MAP2, synaptophysin). Prominent labelling of the lesional cells for developmentally regulated proteins (OTX1, TBR1, SOX2, MAP1b, CD34, GFAPδ) and oligodendroglial lineage markers (OLIG2, SMI94) was observed. No mutations were detected in the mTOR pathway genes, BRAF, FGFR1 or MYB. Clinical, pathological and genetic data could indicate that MVNT aligns more with a malformative lesion than a true neoplasm with origin from a progenitor neuro‐glial cell type showing aberrant maturation.

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“…Since submission of this study, further cases and series have been reported, attesting to the indolent nature of MNVT .…”

Section: Discussionmentioning

confidence: 83%

Multinodular and vacuolating neuronal tumors in epilepsy: dysplasia or neoplasia?

et al. 2017

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“…MVNTs are of particular interest to the clinician/epileptologist, not only because of the frequent association with epileptic seizures, but also as an operatively treatable cause of epilepsy. A total of 96 cases (including the current case) have been described in the literature to date (Ratilal et al, 2007;Huse et al, 2013;Bodi et al, 2014;Fukushima et al, 2015;Nagaishi et al, 2015;Yamaguchi et al, 2016;Alsufayan et al, 2017;Badat et al, 2017;Cathcart et al, 2017;Gökç e, 2017;Monté et al, 2017;Nunes et al, 2017;Gonzalez-Quarante et al, 2018;Kapucu et al, 2018;Lobo and Srinivasan, 2018;Pekmezci et al, 2018;Thom et al, 2018) (supplementary table 1), of which 37 were histopathologically verified (HV) (38.54%). A slight female predominance was noticed in both non-HV and HV cases (M:F=2:3 and M:F=9:11, respectively).…”

Section: Discussionmentioning

confidence: 96%

Multinodular and vacuolating neuronal tumour of the cerebrum: a rare neuroimaging incidentaloma or a potentially treatable cause of focal epilepsy?

Baščarević¹,

Pejović²,

Ristić³

et al. 2019

Epileptic Disorders

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Multinodular and vacuolating neuronal tumour (MVNT) of the cerebrum is a relatively new, well defined histopathological and neuroradiological entity, in many cases associated with an early adult‐onset epilepsy. These lesions have an indolent course and resemble both malformative and neoplastic processes, combining a focal developmental anomaly and a low‐grade tumour. Herein, we report a case of a 48‐year‐old female patient with left temporal lobe epilepsy associated with MVNT. In addition, a comprehensive review of all the previously published cases is provided with a focus on seizure‐related cases, surgical treatment, and postoperative outcome.

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“…They have a characteristic “bubbly” cystic appearance with fairly extensive associated T2/FLAIR signal abnormality and minimal enhancement in 20‐30% of cases (Fig ) . MVNT is a more recently characterized benign, mixed glial neuronal lesion consisting of a subcortical cluster of tiny, cystic, nodular lesions with associated T2/FLAIR signal abnormality ( Fig ) . MVNTs are usually seen in the deep cortical ribbon and superficial subcortical white matter in the high convexities .…”

Section: Pathologic Entities Mimicking and Involving Perivascular Spacesmentioning

confidence: 99%

“…[34][35][36] MVNTs are usually seen in the deep cortical ribbon and superficial subcortical white matter in the high convexities. 35 They are associated with seizures in around 30% of patients and rarely show progression or enhancement. 36…”

Section: Neoplastic Cystic Entities Mimicking Perivascular Spacesmentioning

confidence: 99%

Neuroimaging of Dilated Perivascular Spaces: From Benign and Pathologic Causes to Mimics

Rudie

Rauschecker

Nabavizadeh

et al. 2017

Journal of Neuroimaging

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Perivascular spaces (PVSs), also known as Virchow-Robin spaces, are pial-lined, fluid-filled structures found in characteristic locations throughout the brain. They can become abnormally enlarged or dilated and in rare cases can cause hydrocephalus. Dilated PVSs can pose a diagnostic dilemma for radiologists because of their varied appearance, sometimes mimicking more serious entities such as cystic neoplasms, including dysembryoplastic neuroepithelial tumor and multinodular and vacuolating neuronal tumor, or cystic infections including toxoplasmosis and neurocysticercosis. In addition, various pathologic processes, including cryptococcosis and chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids, can spread into the brain via PVSs, resulting in characteristic magnetic resonance imaging appearances. This review aims to describe the key imaging characteristics of normal and dilated PVSs, as well as cystic mimics and pathologic processes that directly involve PVSs.

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Natural history of lesions with the MR imaging appearance of multinodular and vacuolating neuronal tumor

Abstract: The natural history of lesions with imaging features of MVNT is overall stable from a clinical and imaging appearance over time.

Cited by 40 publications

References 16 publications

Multinodular and vacuolating neuronal tumors in epilepsy: dysplasia or neoplasia?

Multinodular and vacuolating neuronal tumors in epilepsy: dysplasia or neoplasia?

Multinodular and vacuolating neuronal tumour of the cerebrum: a rare neuroimaging incidentaloma or a potentially treatable cause of focal epilepsy?

Neuroimaging of Dilated Perivascular Spaces: From Benign and Pathologic Causes to Mimics

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