Abstract
Dysembryoplastic neuroepithelial tumors (DNETs) are benign cortical tumors frequently associated with medically incurable focal epilepsy. These tumors occur most commonly in children. Given the fact that they rarely become malignant, the long-term prognosis in terms of mortality is excellent, however its similar appearance with other tumors of the central nervous system increases the potential for misdiagnosis and the risk of a pejorative clinical evolution. Our patient underwent partial resection of the lesion with a pathology study revealing a WHO grade 2 cortical ependymoma, reflecting the malignant transformation of the tumor.