Dysembryoplastic neuroepithelial tumors in pediatric patients

Objective: Dysembryoplastic neuroepithelial tumors (DNTs) are benign cortical tumors that are frequently associated with the medically intractable focal epilepsy. In this study, the authors delineate the clinical characteristics of DNTs in children and evaluate the role of cortical dysplasia (CD) in the epileptogenicity to find out the optimum surgical strategy. Methods: A retrospective analysis was performed for clinical data of children with DNT, who underwent surgery between 1996 and 2006. The adopted surgical methods were uniform according to the tumor location and included intraoperative electrocorticography (ECoG)-guided resection. The prognostic factors were evaluated for the two prognostic group categorized by the seizure outcome at one year after surgery. Results: Of 22 patients, the overall seizure free rate was 90.9% and the other two patients belonged to Engel class II during the mean follow-up period of 44.1 months. There was no worsening of the seizure after one year of surgery. Associated CD was found in 18 cases (81.8%) and in the 80% (8 of 10 cases) of the additionally resected areas according to the electrophysiologic studies. Conclusions: The CD associated with DNT appears to have its own epileptogenicity. Therefore, complete removal of the CD with tumor itself is important for patient outcome. A thorough surgical approach can be accomplished by comprehensive presurgical evaluations and extensive surgery with the aid of the intraoperative ECoG or intracranial recording.