Pediatric septal dysembryoplastic neuroepithelial tumor (sDNT): case-based update.

ABSTRACT

PURPOSE: The purpose of this study was to review a case of a septal dysembryoplastic neuroepithelial tumor (sDNT) and compare it to cases reported in the current literature. METHODS: We review a case of sDNT and compare with 7 other previously noted cases in the literature. RESULTS: The mainstay treatment is gross total resection, and most patients achieve full clinical resolution. Septal dysembryoplastic neuroepithelial tumor (sDNT) is a rare pediatric disease most commonly presenting as intractable epilepsy or headache. sDNT has been recognized as a genotypically distinct entity from DNT. A high frequency (~ 80%) of mutations of platelet-derived growth factor receptor A (PDGFRA) has been isolated in sDNT and could form the basis for future therapy. MRI is most commonly used to radiographically diagnose the disease and usually demonstrates a lobular interventricular mass involving the septum, potentially extending to the third ventricle. CONCLUSIONS: Our case and literature review validates endoscopic biopsy as a diagnostic and therapeutic intervention.