Spinal Intramedullary Ganglioglioma in Children: An Unusual Location of a Common Pediatric Tumor

Pediatr Neurosurg. 2019;54(4):245-252. doi: 10.1159/000500427. Epub 2019 Jun 18.

Abstract

Ganglioglioma is a common CNS tumor in children, mostly found in the temporal lobe, causing epilepsy. Spinal gangliogliomas are very rare, accounting for 1.1% of all intramedullary spinal tumors. The management principles and the need for adjuvant therapy are not yet well defined in this cohort. BRAF V600E mutation in spinal ganglioglioma has been described in a few series recently. In this report, we describe 3 children with spinal ganglioglioma at different locations, and their expression of BRAF V600E mutation and follow-up. In addition, we review the recent literature on pediatric spinal ganglioglioma management.

Keywords: BRAF mutation; Ganglioglioma; Management; Pediatric tumor; Spinal tumor.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Female
  • Ganglioglioma / pathology
  • Ganglioglioma / surgery*
  • Ganglioglioma / therapy
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Mutation
  • Proto-Oncogene Proteins B-raf / genetics
  • Spinal Cord Neoplasms / pathology
  • Spinal Cord Neoplasms / surgery*
  • Spinal Cord Neoplasms / therapy

Substances

  • BRAF protein, human
  • Proto-Oncogene Proteins B-raf