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

Childs Nerv Syst. 2020 Jun;36(6):1127-1130. doi: 10.1007/s00381-020-04581-y. Epub 2020 Apr 5.

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.

Keywords: Dysembryoplastic neuroepithelial tumor (DNT); Pediatric tumor; Septal dysembryoplastic neuroepithelial tumor (sDNT); Ventricular tumor.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Brain Neoplasms* / diagnostic imaging
  • Brain Neoplasms* / surgery
  • Child
  • Glioma*
  • Humans
  • Magnetic Resonance Imaging
  • Neoplasms, Neuroepithelial* / diagnostic imaging
  • Neoplasms, Neuroepithelial* / surgery
  • Third Ventricle*