Neurofibromatosis 1–associated optic pathway gliomas

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.

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Correspondence to Shlomi Constantini.

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Shofty, B., Ben Sira, L. & Constantini, S. Neurofibromatosis 1–associated optic pathway gliomas. Childs Nerv Syst (2020). https://doi.org/10.1007/s00381-020-04697-1

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Keywords

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