Fact-sheet: Dysembryoplastic neuroepithelial tumor
Updated on 10/07/2017 at 9:46 AM
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Definition
Rare benign brain tumors classified among glial tumors. Classically discovered in the setting of drug-resistant temporal lobe epilepsy in a young patient.
Clinical features
Epilepsy classically:
-drug-resistant.
-typically beginning before the age of 20.
CT
Hypodense.
Sometimes density close to CSF = may be mistakenly attributed to a sequela or atrophy.
May erode the inner table of the skull vault.
Contrast enhancement (see MRI).
Variable calcifications.
MRI
Cortico-subcortical lesion, sometimes triangular with a cortical base, well-defined Hyposignal on T1 Hypersignal on T2 Possible multiple cystic components with low T1 signal and high T2 signal in a soap-bubble pattern Negative signs: no perilesional edema Variable contrast enhancement: Classically no contrast enhancement In 40-50% of cases: contrast enhancement (nodular, more rarely ring-like). The appearance of contrast enhancement during follow-up is possible. Spectroscopy nonspecific, however absent or slight increase in choline (DD: glioma).
Management
Surgical resection, not mandatory (only if drug-resistant epilepsy)