Neurofibromatosis 1–associated optic pathway gliomas

Ben Shofty, Liat Ben Sira, Shlomi Constantini*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

10 Scopus citations

Abstract

Background: Optic Pathway Gliomas (OPG) are the most common brain tumor in Neurofibromatosis 1 patients (NF1). They are found along the optic pathway and may involve the optic nerves, chiasm, retro-chiasmatic structures, and the optic radiations. NF1 associate OPG (NF1-OPG) have variable presentation, disease course and response to treatment. The optimal management is patient-specific and should be tailored by a multidisciplinary team. Age, sex, histology, and molecular markers may be important factors in the individualized decision-making process. Chemotherapy is the first-line treatment in cases of progressive tumors, and visual preservation is the main goal of treatment. Purpose: In this paper we will review the disease, practical management, and recent advances of NF1-OPG.

Original languageEnglish
Pages (from-to)2351-2361
Number of pages11
JournalChild's Nervous System
Volume36
Issue number10
DOIs
StatePublished - 1 Oct 2020

Keywords

  • Chemotherapy
  • Low-grade glioma
  • NF1
  • Neurofibromatosis 1
  • Neurosurgery
  • OPG
  • Optic pathway glioma
  • Radiotherapy

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