Dysembryoplastic Neuroepithelial Tumor (DNET) - Temporal Lobe
Overview
Dysembryoplastic Neuroepithelial Tumor (DNET) is a benign, WHO grade I glioneuronal tumor typically presenting in children and young adults with long-standing, pharmacoresistant epilepsy. The temporal lobe is the most common location.
Epidemiology
- Age: Childhood to young adulthood (most < 20 years)
- Slight male predominance
- ~80% of cases involve the temporal lobe
Clinical Presentation
- Chronic, intractable focal epilepsy
- Often normal neurological exam
- Rarely causes increased intracranial pressure or neurological deficits
Diagnosis
Treatment
- Surgical resection is the treatment of choice
- Gross total resection often cures epilepsy
- Recurrence is rare
Prognosis
- Excellent long-term prognosis
- Very low risk of malignant transformation
- Seizure freedom in >80% after complete resection
Differential Diagnosis
- Ganglioglioma
- Oligodendroglioma (especially low-grade)
- Cortical dysplasia without tumor
- Low-grade astrocytoma
References
- WHO Classification of Tumors of the CNS, 2021
- Guerrini R, et al. Epilepsy surgery and DNETs. Epilepsia. 2012.
- Blumcke I, et al. Histopathological findings in long-term epilepsy surgery. Brain Pathol. 2007.