Child's Nervous System
URL with Digital Object Identifier
Purpose: In children and adolescents, dysembryoplastic neuroepithelial tumors (DNETs) of the brain present with seizures almost 100 % of the time, potentially creating significant long-term morbidity and disability despite the generally indolent course of the lesion. These tumors also tend to be quite resistant to anti-epileptic drugs which, themselves, can be associated with long-term side effects and resultant disability. Many clinicians advocate early surgical resection of these lesions, but how effective this approach is, and how aggressive tumor removal should be, continues to be debated. Methods: We performed a systematic review of the relevant literature to identify all reports of DNET resections in pediatric patients published over the past 20 years. In all, over 3000 MEDLINE abstracts were reviewed, ultimately resulting in 13 studies with 185 pediatric DNET patients to review. Results: Surgical resection of the lesion was effective at improving seizures in over 98 % of patients and at achieving long-term seizure freedom in 86 %. Surgical resection of DNETs also appeared to be quite safe, with no reported perioperative deaths and an overall rate of postoperative complications of 12 %; the vast majority of these complications were transient. Conclusions: Total gross resection of the lesion was the only factor statistically correlated with long-term seizure freedom (r = 0.63, p = 0.03). However, data remain lacking regarding whether this translates into more extensive procedures—like brain mapping and partial lobectomies—being any more effective than simple lesionectomies alone. Further research is clearly needed to address this and other crucial questions.