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Multinodular and Vacuolating Neuronal Tumour (MVNT)

Summary

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  • Rare, benign neuronal tumour characterised by clusters of vacuolated neurons
  • Typically located in the cerebral hemispheres, often subcortical
  • Usually asymptomatic and incidentally discovered on neuroimaging1

Pathophysiology

  • Classified as a WHO grade 1 tumour
  • Composed of nodules of vacuolated dysplastic neurons
  • Immunohistochemistry:
    • Positive for HuC/HuD, MAP2, and synaptophysin
    • Negative for NeuN, GFAP, and IDH1 R132H mutation
  • Molecular profile:
    • No specific genetic alterations identified
    • MAPK pathway activation suggested

Demographics

  • Typically affects adults (mean age 40-50 years)
  • No significant gender predilection
  • Rare in children

Diagnosis

  • Often asymptomatic and incidentally discovered
  • When symptomatic:
    • Seizures (most common)
    • Headaches
    • Cognitive impairment (rare)
  • Differential diagnosis:
    • Focal cortical dysplasia
    • Low-grade glioma
    • Dysembryoplastic neuroepithelial tumour (DNET)

Imaging

  • MRI characteristics:
    • T1: Hypointense to isointense
    • T2/FLAIR: Hyperintense, 'bubbly' appearance
    • T2*: No susceptibility artefacts
    • DWI: No restricted diffusion
    • Contrast: Typically non-enhancing
  • Location:
    • Subcortical white matter
    • Commonly in frontal and temporal lobes
  • Morphology:
    • Multiple small (< 5 mm) nodules
    • 'Soap bubble' or 'Swiss cheese' appearance

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  • Incidental T2-hyperintense lesion without mass effect in the right occipital lobe. There was neither diffusion restriction nor pathological enhancement.

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  • On an MRI performed for headache, an incidental frontal lobe lesion was identified.
  • The bubbly T2-hyperintensity and lack of enhancement are typical of MVNT.

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  • The "soap bubble" T2-hyperintense lesion in the left cerebral hemisphere without mass effect, diffusion restriction or enhancement was typical of MVNT.

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  • A 30-year-old patient who present with a headache had an incidental lesion in the right occipital lobe that was consistent with an MVNT.

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  • Clustered subcortical hyperintensities in the right occipital pole were identified incidentally.
  • The appearance, lack of enhancement and stability over four years, were consistent with MVNT.

Treatment

  • None required; a benign incidental "leave-me-alone" lesion. The bubbly, non-enhancing, FLAIR-hyperintense clusters at the deep cortex/subcortical junction are characteristic

Differential diagnosis

Differential Diagnosis Distinguishing Feature
Dysembryoplastic neuroepithelial tumour (DNET) MVNT lacks the specific glioneuronal element seen in DNET
Focal cortical dysplasia (FCD) MVNT has a more nodular appearance and lacks cortical dyslamination typical of FCD
Ganglioglioma MVNT lacks the biphasic pattern of neoplastic glial and neuronal cells seen in ganglioglioma
Low-grade glioma MVNT has a characteristic vacuolated appearance and lacks infiltrative growth
Multiple sclerosis plaques MVNT is typically confined to gray matter, while MS plaques often involve white matter
Tuberous sclerosis complex lesions MVNT lacks calcifications and subependymal nodules typical of TSC
Metastatic disease MVNT is typically non-enhancing and lacks the surrounding oedema often seen with metastases
Hamartoma MVNT has a more organised, nodular structure compared to the disorganised appearance of hamartomas
Polymicrogyria MVNT does not show the characteristic cortical folding abnormalities of polymicrogyria
Leukoencephalopathy MVNT primarily affects gray matter, while leukoencephalopathy involves white matter

  1. Rima et al. Multinodular and vacuolating neuronal tumour: emphasis on expression of early and late neuronal immunomarkers. 2026. Journal of clinical pathology - Open in new tab. ↩