Dysembryoplastic neuroepithelial tumor

Case contributed by Dr Hisham Alwakkaa

Presentation

Complex partial seizure for the first time in otherwise healthy child.

Patient Data

Age: 5 years
Gender: Male

Hypo-dense mass in the posterior inferior aspect of the left temporal lobe. Minimal mass effect on the adjacent left temporal horn of the lateral ventricle is present. No peri-lesional edema noted.

The left temporal lobe mass shows hypo-signal intensity on T1-Weighted images, hyper-signal intensity on T2-weighted images, and mixed signal intensity on FLAIR images with predominantly hypo-signal center and hyper-signal peripheral margins giving the appearance of bright rim sign. No diffusion restriction or susceptibility artifact seen. No enhancement identified.

Case Discussion

This is a pathology-confirmed case of dysembryoplastic neuroepithelial tumor. The case is typical in terms of patient age, clinical complaint (partial seizure), CT & MRI findings. The patient underwent surgery to remove the tumor and the pathology confirmed the diagnosis of DNET.

Histology

MICROSCOPIC DESCRIPTION: The largest tumor component comprises small round nuclei in a fibrillary background with some suggestion of perinuclear clearing. Astrocytes with eosinophilic cytoplasm are also present. This area transitions to similar cells in a loose, microcytic background. Some of the background matrix is basophilic and also staining with Alcian blue. No definite mature ganglion cells can be seen floating in the mucoid matrix. There is co-expression of GFAP, Synaptophysin, and Neurofilament. No mitoses or necrosis is present.

DIAGNOSIS: Microscopic foci of low-grade glioneuronal tumor.

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Case information

rID: 57641
Published: 4th Jan 2018
Last edited: 16th Jul 2018
Inclusion in quiz mode: Included
Institution: Women and children Hospital of Buffalo

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