Dysembryoplastic Nueroepithelial Tumors of Childhood: Ege University Experience

Objectives: Dysembryoplastic neuroepithelial tumors (DNETs) are rare, low-grade tumors of childhood CNS. It is an important cause of intractable epilepsy, which is surgically curable. We aimed to review our institutional experience with DNET. Methods: Medical records of children <18 years of age diagnosed with DNET between 2009-2020 were reviewed. Clinical features of the patients, including initial symptoms, duration of symptoms, medical treatments, age at the time of surgery, tumor location, surgical resection, and outcome of the patients, were documented. Results: We reviewed the records of 17 patients. Twelve were male(70%), 5 of them female(30%). The median age was 11 years(19 months-17 years). The primary symptom was a seizure in all of the patients. Thirteen patients presented with complex partial seizures, whereas 2 had a simple partial seizure and two generalized tonic-clonic seizures. Seven patients had intractable epilepsy and received at least two anti-epileptic drugs. The median duration of symptoms was 6.6 months(0-48 months). The surgical strategy was determined according to symptoms of the patient, MRI findings, and EEG results. Total surgical resection was performed in 15 patients, and two patients underwent partial resection. Of these 15 patients, seven patients underwent lesionectomy, while the other eight patients had extended lesionectomy. The mean follow-up time was 107 months(54-144 months), the seizure control was achieved in 14 patients(82.4%) after surgery, but three patients experienced recurrence. Conclusion: In DNETs, the complete resection of the lesion is generally associated with seizure-free outcomes. In patients with partial resection and lesionectomy, MRI follow-up is recommended for recurrence.