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.

• Authors: Ben Shofty, Liat Ben Sira, Shlomi Constantini
• Journal: Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery (Childs Nerv Syst) Vol. 36 Issue 10 Pg. 2351-2361 (10 2020) ISSN: 1433-0350 [Electronic] Germany
• PMID: 32524182 (Publication Type: Journal Article)

Topics

• Eye
• Humans
• Nerve Fibers
• Neurofibromatosis 1 (complications)
• Optic Nerve
• Optic Nerve Glioma (complications, therapy)
• Optic Nerve Neoplasms (complications, therapy)