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A 36-year-old female with right-sided focal motor seizures and absence.
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MRI scan showed multiple nodules in white matter of left superior temporal gyrus.
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The lesion was a multinodular and vacuolating neuronal tumor (MVNT) of the CNS.
Abstract
A 36-year-old female patient was referred to our hospital for a six-months clinical history of episodes of right-sided focal motor seizures and absence. MRI scan showed a multi-nodular lesion in the subcortical white matter of left superior temporal gyrus. After a complete resection, the neuropathological examination revealed a lesion with marked cell vacuolation which was composed of neuroepithelial cells ranging from small to medium sized elements to larger, neuron-like cells, occasionally bi-nucleated. The neuropathological features of the lesion were compatible with the diagnosis of multinodular and vacuolating neuronal tumor (MVNT) of the CNS. MVNT is very rare, recently described entity among the large spectrum of glioneuronal tumors. It is characterized by benign clinical behavior and neurosurgical resection usually provide control of disease.
A 36-year-old female patient was referred to our hospital for a six-months clinical history of episodes of right-sided focal motor seizures and absence. The remainder of her clinical history was unremarkable. On examination the patient did not show neurological deficits.
MR images (Fig. 1) revealed a left temporal lesion involving the deep cortical ribbon and the adjacent superficial subcortical white matter along the gyral contour. The lesion was hyper-intense on T2 and T2 FLAIR images, mildly hypo-intense on T1, and showed no diffusion restriction, no susceptibility or blooming on susceptibility-weighted imaging (SWI). The lesion contained cluster of small cyst-like signal alterations that were iso-intense to CSF on T2 and T1 weighted-images. The biggest nodule content suppressed on T2 FLAIR, and showed a very tiny focus of contrast enhancement along its posterior margin. Dynamic-susceptibility contrast perfusion showed no abnormality. There was no mass effect and edema, and the margins to the adjacent gray and white matter were well-defined. There was no evidence of alterations of gyration and sulcation.
Fig. 1Neuroradiological features of the lesion. MRI scan showed multiple nodules in the subcortical white matter of left superior temporal gyrus: sagittal T2 (A) better demonstrated the subcortical location, without mass effect; nodules appeared hyper-intense on T2 and FLAIR (B), but the biggest nodule suppressed on FLAIR (B arrow) and appeared iso-intense on T1 (C). There was no evidence of restricted diffusion (D) or blooming on susceptibility-weighted imaging (E). T1 after gadolinium (F, arrow) showed a tiny focus of enhancement along the posterior margin of the biggest nodule. CBV map (G) showed no abnormalities.
Multinodular and vacuolating neuronal tumor (MVNT).
e.
Cryptococcosis.
3. Answer
d. Multinodular and vacuolating neuronal tumor (MVNT) of the CNS.
4. Discussion
The patient underwent complete excision of the lesion via temporal craniotomy. The resected specimen consisted of brain tissue characterized by the presence of discrete, multiple nodules mostly localized in the cortex-white matter junction or in the deep cortical layers. The nodules showed marked vacuolation and were composed of neuroepithelial cells ranging from small to medium sized elements to larger, neuron-like cells, occasionally bi-nucleated (Fig. 2). Such cells stained positively for synaptophysin and Olig2, and were negative for NeuN, neurofilament, chromogranin, GFAP, CD34 and IDH1(R132H). The proliferation index (MIB1) was <1%. The lesion displayed no mitotic activity. The neuroradiological and neuropathological findings were consistent with the diagnosis of multinodular and vacuolating neuronal tumor (MVNT) of the CNS.
Fig. 2Neuropathological features of the lesion. The neuropathological examination revealed multiple nodules (A, arrows) mostly localized in the cortex-white matter junction. The nodules showed marked vacuolation (B). The neuropathological features of the lesion were compatible with MVNT diagnosis (A, B: H&E staining; magnification A: 40×; B: 200×).
MVNT is recently described entity among the large spectrum of glioneuronal tumors of the CNS, However, its nosological position remains unclear and it still debated whether this entity represents a tumor or a hamartomatous lesion [
in: Louis D.N. Ohgaki H. Wiestler O.D. Cavenee W.K. Ellison S.W. Figarella-Branger D. Perry A. Reifenberger G. von Deimling A. WHO classification of tumours of the central nervous system. IARC Press,
Lyon2016: 137
in: Louis D.N. Ohgaki H. Wiestler O.D. Cavenee W.K. Ellison S.W. Figarella-Branger D. Perry A. Reifenberger G. von Deimling A. WHO classification of tumours of the central nervous system. IARC Press,
Lyon2016: 137
] and are mainly, but not exclusively, associated with adult-onset seizures. Other neurological symptoms or neurological deficits have been occasionally reported [
]. According to the follow-up data available, MVNTs usually show benign clinical behavior and neurosurgical resection usually provides good control of disease [
in: Louis D.N. Ohgaki H. Wiestler O.D. Cavenee W.K. Ellison S.W. Figarella-Branger D. Perry A. Reifenberger G. von Deimling A. WHO classification of tumours of the central nervous system. IARC Press,
Lyon2016: 137
The authors declare that they have no financial or other conflicts of interest in relation to this report and its publication.
References
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Rosemblum M.K.
et al.
Gangliocytoma.
in: Louis D.N. Ohgaki H. Wiestler O.D. Cavenee W.K. Ellison S.W. Figarella-Branger D. Perry A. Reifenberger G. von Deimling A. WHO classification of tumours of the central nervous system. IARC Press,
Lyon2016: 137
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