Daumas-Duport C, Varlet P
Laboratoire d'Anatomie Pathologique, Hôpital Sainte-Anne, Paris.
Rev Neurol (Paris). 2003 Jul;159(6-7 Pt 1):622-36.
Dysembryoplastic neuroepithelial tumors DNTs are highly polymorphic tumors that arise during embryogenesis. They are preferentially, but not exclusively, located in the supratentorial cortex. Histologically they may mimic any categories of low-grade or even of high-grade gliomas, but from a carcinological point of view, they behave as stable lesions. Their differential diagnosis from gliomas is obviously important to spare these young patients with a normal life expectancy the long- term deleterious effect of radiation or chemotherapy. The diagnosis of DNT must be considered when all the following criteria are present: partial seizures with or without secondary generalization, no neurological deficit or a stable congenital deficit, cortical topography on MRI, absence of peri-tumoral edema and of mass effect. In other locations, the diagnosis of DNT has to be suspected in case of discordance between the neurological status of the patient and the topography of the tumor or of unusual radiological features such as contrast enhancement but no mass effect and no edema. Supratentorial cortical DNTs tend now to be detected more systematically by imaging soon after first seizures. In most instances, the epilepsy can be cured by gross total surgical removal. Surgery also allows to prevent the risks of intratumoral hematoma or infarct. DNTs should therefore be operated soon after diagnosis. However, excellent results can also be obtained by epilepsy surgery in patients with long term drug resistant partial seizures.
