ABSTRACT

Clinically, dysembryoplastic neuroepithelial tumor (DNET) is associated with pharmaco-resistant chronic epilepsy, with partial complex seizures as the main presentation. Based on its mixed cellularity, preponderance in the temporal lobe, and association with focal cortical dysplasia (FCD), DNET may have a developmental origin from the secondary germinal layer and pluripotent precursor cells. DNET is a rare glioneuronal tumor that tends to present with medically intractable epileptic seizures in childhood, adolescence, or young adulthood. Clinical diagnosis of DNET is essentially based on the following criteria: partial seizures, with or without secondary generalization before age 20; no neurological deficit or presence of a stable and likely congenital neurological deficit; cortical topography of the lesion on MRI; and no mass effect on CT or MRI. Prognosis of DNET is related to complete tumor resection, young age at surgery, short epilepsy duration, and absence of cortico–subcortical damage.