Dysembryoplastic neuroepithelial tumor (DNET)

Case contributed by A.Prof Frank Gaillard

Presentation

Seizures

Patient Data

Age: 40
Gender: Female

Mass effect involves the hippocampal head, amygdala, adjacent portion of the anterior temporal lobe, with the thin section axial T2 scans and the previous coronal T2 scans demonstrating a micro cystic appearance. The lesion is centered on grey matter with some extension into the white matter. There is no abnormal contrast enhancement. No vasogenic edema extends beyond the lesion. The left cerebral hemisphere is normal.

Case Discussion

The patient went on to have a resection. 

Histology

MICROSCOPIC DESCRIPTION:

Paraffin sections show fragments consisting predominantly of cerebral cortex with white matter included. These show loss of normal laminar architecture with a haphazard arrangement of enlarged, mildly dysmorphic neurons. Many of these lack discernible apical dendrites. Many smaller neurons are maloriented. No binucleate or balloon neurons are identified. There is moderate astrocytic gliosis. The features are of malformation of cortical development (focal cortical dysplasia - Taylor Type IB). No evidence of tumor is seen.

Additional sections shows a fagment of cerebral cortex in which there is a small focus of dysembryoplastic neuroepithelial tumor (DNET). This measures 3mm in greatest dimension and consists of a circumscribed collection of small immature neuronal cells which are dispersed in a myxomatous stroma. Scattered large mature neurons are admixed with the small immature neuronal cells. Poorly formed glioneuronal elements are noted. The small immature neuronal cells show strong granular immunostaining for synaptophysin. The Toposiomerase IIb labeling index is <1%. The adjacent cerebral cortex shows dyslamination and malorientation of neurons.

DIAGNOSIS:

  1. Malformation of cortical development (focal cortical dysplasia - Taylor Type Ib);
  2. Dysembryoplastic neuroepithelial tumor (DNET) in deep temporal specimen
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Case information

rID: 32955
Published: 23rd Jan 2015
Last edited: 22nd Sep 2015
Inclusion in quiz mode: Included

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