Dysembryoplastic neuroepithelial tumour: insight into the pathology and pathogenesis

Sontowska, Iwona; Matyja, Ewa; Malejczyk, Jacek; Grajkowska, Wiesława

doi:10.5114/fn.2017.66708

Cited by 21 publications

(11 citation statements)

References 60 publications

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“…DNTs frequently arise in the temporal lobe and less frequently in the frontal or parietal lobe. 1,8 The common sites of supratentorial RGNTs remain unclear. Although any generalization based on our observations of a small number of cases may be premature, we suggest that RGNT should be included as a differential diagnosis for patients with a suspected DNT in regions other than the temporal lobe.…”

Section: Discussionmentioning

confidence: 77%

Intractable epilepsy due to a rosette‐forming glioneuronal tumor with a dysembryoplastic neuroepithelial background

et al. 2017

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A rosette-forming glioneuronal tumor (RGNT) was initially reported as an infratentorial tumor that comprised both small neurocytic rosettes and astrocytic components. However, a few studies have reported supratentorial RGNTs arising in the cerebral hemispheres. Here, we report an unusual case involving a 9-year-old boy with a supratentorial RGNT who presented with intractable epilepsy and behavioral changes. Brain MRI revealed a well-circumscribed space-occupying lesion with septae in the right inferomedial parietal lobe. Electroencephalography showed multifocal spikes over the right frontal, temporal and parietal regions. The seizure frequency decreased dramatically after tumorectomy. Histopathological examination revealed prominent neurocytic rosette formation appearing with the specific glioneuronal element of a dysembryoplastic neuroepithelial tumor (DNT). Although the pathogenesis has not been elucidated, a supratentorial RGNT presenting with epilepsy may exhibit a rosette component, which is the major feature of this tumor, against the background of a specific glioneuronal element mimicking DNT. However, RGNT arising in regions other than the fourth ventricle is rare, and the pathogenesis of epilepsy due to RGNT has not been fully elucidated. Further clinical and histological studies are required to understand the pathology underlying epilepsy caused by RGNT.

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Section: Discussionmentioning

confidence: 77%

Intractable epilepsy due to a rosette‐forming glioneuronal tumor with a dysembryoplastic neuroepithelial background

et al. 2017

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“…However, the majority of OLCs are strongly positive for S-100 protein and Oligo-2, while the "floating neurons" express neuronal markers including synaptophysin neurofilament, NeuN, neuron-specific enolase, MAP2, and class-III beta-tubulin [5]. Given its benign nature is usual to find a low proliferative index Ki67 [6]. This tumor showed diffuse reactivity for GFAP and widely perikaryal synaptophysin reaction, whereas no expression of chromogranin and CD34 was detected.…”

Section: Discussionmentioning

confidence: 91%

A Case of Dysembryoplastic Neuroepithelial Tumor in an Adolescent Male

Yibirin¹,

Oliveira²,

Suarez³

et al. 2021

Cureus

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Dysembryoplastic neuroepithelial tumors (DNETs) are benign mixed glioneuronal neoplasms that frequently occur in children and young adults. We present the case of a 17-year-old male who arrived at the hospital following seizure-like activity. A magnetic resonance imaging (MRI) scan showed a 10 x 8 x 10 mm, ovalshaped, non-enhancing, well-defined mass within the right hippocampus. The patient underwent a transcortical approach via the middle temporal gyrus for resection of the mass; histopathological examination demonstrated the presence of round, uniform cells in an extensively myxoid background with diffuse reactivity to glial fibrillary acidic protein (GFAP). DNETs are considered benign, non-recurring lesions. Complete surgical resection is associated with a seizure-free outcome in 80% to 100% of cases.

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“…It has been related to abnormal embryological cortical development of the brain, and its occurrence is often linked to focal cortical dysplasia (FCD). This type of tumor resides typically in the brain cortex and often involves only one gyrus, with neuronal elements that form columnar structures perpendicular to the cortical surface 2 …”

Section: Introductionmentioning

confidence: 99%

“…This type of tumor resides typically in the brain cortex and often involves only one gyrus, with neuronal elements that form columnar structures perpendicular to the cortical surface. 2 DNET is primarily found in patients with epilepsy. Confident diagnosis based on magnetic resonance imaging (MRI) can often be made due to its signal profile and its relationship with the cortex.…”

Section: Introductionmentioning

confidence: 99%

Intraventricular dysembryoplastic neuroepithelial tumor in the temporal horn with Broad involvement of the ependyma

et al. 2021

Clinical Case Reports

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Dysembryoplastic neuroepithelial tumour: insight into the pathology and pathogenesis

Abstract: A b s t r a c t Dysembryoplastic neuroepithelial tumour (DNT) is categorized as a benign glioneuronal neoplasm affecting children

Cited by 21 publications

References 60 publications

Intractable epilepsy due to a rosette‐forming glioneuronal tumor with a dysembryoplastic neuroepithelial background

Intractable epilepsy due to a rosette‐forming glioneuronal tumor with a dysembryoplastic neuroepithelial background

A Case of Dysembryoplastic Neuroepithelial Tumor in an Adolescent Male

Intraventricular dysembryoplastic neuroepithelial tumor in the temporal horn with Broad involvement of the ependyma

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