Acta Neurologica Belgica
https://doi.org/10.1007/s13760-017-0872-x

NEURO-IMAGES

Magnetic resonance imaging findings of two cases with multinodular
and vacuolating neuronal tumor
Erkan Gökçe1
Received: 3 October 2017 / Accepted: 7 December 2017
© Belgian Neurological Society 2017

Abstract
Multinodular and vacuolating neuronal tumors (MVNT) are low grade neuronal tumors classified in cerebral tumors based
on 2016 WHO central nerve system classification. Previously, MVNTs were probably tumors incidentally determined by
imaging methods, but they could not be diagnosed or they were misdiagnosed. Two cases are reported in the present study,
for which radiological imaging was carried out for epilepsy in one and for headache in the other. The first case was a twenty
seven years old female patient with headache complaint. Brain MRI examination revealed a clustering lesion of multinodular
character located in an about 4.0 × 3.5 × 1.5 cm area which had a left precuneal subcortical-juxtacortical location. The other
case was a male patient with epilepsy. A clustering lesion of multinodular nature was determined in an area of about 2.0 ×
2.0 × 1.5 cm in cortical-subcortical area in corona radiata-convexity plane of right middle frontal girus. No changes in size
or nature were determined in follow-up examinations. MVNTs are recently defined tumors, and the characteristic feature for
diagnosis of these rare tumor entities could be a subcortical white matter lesion with satellite nodules that manifest itself as
T2/FLAIRhyperintensity. Increasing number of case reports and studies in the literature could make the radiological and
pathological signs of the condition clearer.

Introduction

Case reports

Multinodular and vacuolating neuronal tumors (MVNT) are
superficially located cerebral neuronal tumors which were
first identified in 2013 [1–3]. World Health Organization
Classification of Tumors of the Central Nervous System
(2016) includes MVNT as a unique cytoarchitectural pattern
of gangliocytoma [4, 5]. The true epidemiology of MVNTs
is unknown, some of them either diagnosed incidentally or
detected on MR scanning in patients with epilepsy, but they
are often misdiagnosed on imaging [2–4, 6]. A small number
of case reports and several MVNT series have been reported
in the neuropathologic and radiologic literature [1–3, 5–9].
The present study reports two radiologically diagnosed cases
with MVNT, who underwent MRI because of epilepsy and
the other because of headache.

Case 1

* Erkan Gökçe
drerkangokce@gmail.com
1

Cranial computed tomography (CT) of a 27 years old
woman due to headache had been reported to be normal by
us (Fig. 1a). However, magnetic resonance imaging (MRI)
obtained with 1.5T MR unit showed a multinodular clustering lesion of about 4.0 × 3.5 × 1.5 cm dimensions with juxtacortical location in the precuneus which was hyperintense
on T2 weighted (Fig. 1b, c) and FLAIR series (Fig. 1d),
slightly hypointense on T1 weighted series (Fig. 1e), nonenhanced after contrast administration (Fig. 1f), without
peripheral edema and without mass effect on all the series
(Fig. 1b–e). Diffusion restriction was not observed on diffusion MR (Fig. 2a). A significant metabolite change was
not observed in lesion area with MR spectroscopy (MRS).
Dynamic susceptibility contrast (DSC) perfusion MR without preloading did not indicate perfusion increase in the
lesion area (Fig. 2b, c). No change in size or nature of the
lesion was observed in about 3 years of follow-up imaging.

Gaziosmanpaşa University Medicine School, Merkez, Tokat,
Turkey

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Fig. 1  CT and MR images of a 27-year-old female patient with headache complaints. a The mass cannot be clearly distinguished on the
CT examination. b Axial and c clustering multinodular lesion located
in subcortical–juxtacortical area of left precuneus is seen on sagittal

T2 weighted MRI (arrow). d Coronal FLAIR image shows hyperintense lesion (arrow). e Lesion cannot be distinguished on axial gradient T1 weighted image. f Non-enhancing is seen on axial contrastenhanced image

Fig. 2  Diffusion and DSC perfusion MR images of a 27-year-old
female patient with headache. a No diffusion restriction on diffusion weighted imaging. b A minimal rCBC increase was observed

in lesion area with perfusion MR. c Perfusion dynamics of the lesion
was similar to that of normal parenchyma in perfusion chart

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Acta Neurologica Belgica

Case 2

Discussion

A 2.0 × 2.0 × 1.5 cm lesion was detected in the brain
MRI (1.5T MR) of a 21 years old male patient who had
epilepsy. The lesion was located in the right middle frontal gyrus. The clustering multinodular lesion was hyperintense on T2 weighted (Fig. 3a, b) and FLAIR series
(Fig. 3c), slightly hypointense on T1 weighted series
(Fig. 3d), non-enhanced after contrast administration
(Fig. 1e), without peripheral edema, without mass effect
on all the series (Fig. 3a–e). The lesion did not have diffusion restriction on diffusion MR (Fig. 3f). MRS did not
indicate significant metabolite change in lesion area. DSC
perfusion MR series without preloading did not show perfusion increase in the lesion area (Fig. 4a, b). No change
was observed in size or nature of the lesion throughout the
2 years of follow-up imaging of the patient.

MVNTs are low-grade tumors which have been placed in
the cerebral tumors list in 2016 WHO’s classification of
central nerve system tumors [4]. Macroscopically, MVNTs
are round, nodular-looking, small, well-bordered, clustering tumors located subcortically and in next to deep cortical layers [2–4]. Microscopically, they have mature-looking
neurons embedded in a fibrillary background and have large
cytoplasmic vacuoles [2–4]. Tumor cells have immunopositivity for human early stage neuronal development associated protein HuC/HuD and Olig2. On the other hand, they
exhibit negativity or weak positivity for mature neurons
related neuronal lineage markers such as neuronal nuclear
antigen, synaptophysin and neurofilament proteins [1–3, 7,
8]. They also have immunopositivity for alpha-internexin
[8]. Alpha-internexin positivity of MVNT supports the

Fig. 3  Brain MRI of a 21-year-old male patient with epilepsy. a
Axial, b Sagittal T2 weighted images of multinodular hyperintense
lesion in cortical–subcortical area in right middle frontal gyrus. c
Heterogeneous hyperintense appearance of the lesion on FLAIR

sequence. d Hypointense appearance of the lesion on T1 weighted
image, e Non-enhancing lesion on contrast-enhanced image. f No diffusion restriction on diffusion weighted imaging

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Acta Neurologica Belgica

Fig. 4  DSC perfusion MR images of a 21-year-old male patient who had epilepsy. a A clear perfusion increase was not observed on colored
rCBV map of lesion area. b Perfusion dynamic of the lesion was similar to that of normal parenchyma on perfusion graph

hypothesis that the lesion could represent a dysplastically
early neuronal phenotype and could be categorized somewhere between cortical malformation and hamartomatous
lesions of benign nature [5]. Characteristic modular changes
such as IDH, 1p19q status and BRAF1,2,4 were not detected
in genetic analyses except for one case in which a MEK1
mutation was found [1, 3, 8].
Radiological features of MVNTs are similar. It is difficult to identify small lesions on CT, but they appear as
non-enhancing and non-calcifying low attenuation lesions
in subcortical or juxtacortical white matter [8]. On MRI,
on the other hand, they appear on T2 weighted and FLAIR
series as clustering multinodular lesions in subcortical white
matter without mass effect or peripheral edema [1–3, 5].
They appear hyperintense on T2 weighted, do not suppress
on FLAIR series [5]. On contrast-enhanced series, they generally do not have enhancement, but there are some reports
indicating weak and focal enhancement [1–3, 5]. Alsufayan
et al. detected contrast-enhancement in a dotted and linear
pattern in a total of three lesions in two out of 24 MVNT
cases [6]. In MVNTs studied histopathologically and radiologically in literature, distinct and coalesced gray nodules
were adjacent to deep cortex, but most lesions were subcortical. It was mentioned that this type of localization can
also be seen in DNT, but it is more characteristic in MVNT
[2]. In their radiology-based studies, Alsufayan et al. [6]
reported that 43% of the lesions were in subcortical/juxtacortical white matter, while 57% were in cortical and subcortical white matter. In accordance with the literature, lesions

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found in our cases did not have diffusion restriction [3, 5, 6].
Fukushima [1] and Huse et al. [2] reported relative increases
in choline/NAA in one case each where they used MRS.
Nagaishi et al. [7], on the other hand, reported increase in
choline peak of their cases. In our cases, no clear changes
were observed in metabolite levels on MRS.
MVNTs could be differentially diagnosed using entities
with a neuronal component. Tumors of ganglion cells are
solid masses with well circumscription or cystic lesions having a mural nodule. They contain disarrayed dysmorphic
ganglion cells which may or may not have neoplastic glial
components. Unlike ganglion cell tumors which often have
EGBs, Rosenthal fibers, perivascular lymphatic infiltrate
and microcalcifications in addition to neoplastic elements,
MVNTs carries no such secondary characteristics [2, 7].
Dysembryoplastic neuroepithelial tumors (DNT) and similar tumors, focal cortical dysplasia (FCD) and perivascular
spaces (PVS) should be considered radiologically in differential diagnosis of MVNTs. Although DNT is the tumor to
be most probably confused with MVNTs, DNT has predominantly cortical, rather than subcortical, location. DNTs often
have thickening in cerebral cortex and bright FLAIR rim
without mass effect and edema. In previous cases studied by
Huse et al. [1] and Bodi et al. [3], at least 3 out of 12 MVNT
cases had radiological misdiagnoses of DNT [2]. In their
study involving a case with Klinefelter syndrome, Monté
et al. considered a lesion they detected on cerebral MRI as
DNET first, but after a literature investigation, they concluded that the lesion was MVNT [9]. Similarly, we also had

Acta Neurologica Belgica

specified in radiological reporting of our cases that lesions
could primarily be DNT or similar tumors. FCD (Type II),
on the other hand, has high T2 signal deep to cortex in the
same location, but it is generally accompanied by a radial
glial band (transmantle sign) and has a thickened abnormal
overlying cortex [6]. PVSs could have similar localizations
as MVNTs, but they are generally more elongated along the
long axis of vessel. PVSs are observed isointense relative to
cerebrospinal fluid on FLAIR [5, 6].
In terms of treatment and prognosis, MVNTs appear to be
benign tumors. They are biologically very indolent and can
only be followed through imaging when they are asymptomatic [1–3, 5]. In symptomatic patients with epilepsy, surgical resection often eliminates seizures without evident tumor
regrowth [1–3]. In almost all cases reported in literature,
lesions had stable prognoses albeit short follow-up periods
[1, 3, 5, 6]. Slight progressions were reported only in two
cases [1, 6].
Although the present study had the limitation that it is
only radiology-based, characteristic MRI features of lesions
and lack of nature or size changes in follow-up examinations
suggested that they were MVNTs.
In conclusion, MVNTs are recently described neuronal
tumors. As case reports and studies in the literature accumulate, MVNTs will be more frequently and more accurately
diagnosed in daily clinical practice.
Funding This study was not funded by any organization.

Compliance with ethical standards
Conflict of interest I declare that I have no conflict of interest.
Ethical approval For this type of study, formal consent is not required.
This article does not contain any studies with animals performed by
any of the authors.

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Informed consent No personally identifiable information is available
in this study.

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