2. Academic Validation
  3. Neurofibromatosis 1-associated optic pathway gliomas

Neurofibromatosis 1-associated optic pathway gliomas

  • Childs Nerv Syst. 2020 Oct;36(10):2351-2361. doi: 10.1007/s00381-020-04697-1.
Ben Shofty 1,  Liat Ben Sira 2,  Shlomi Constantini 3
Affiliations

Affiliations

  • 1 Department of Neurosurgery, Tel-Aviv Medical Center, The Gilbert Israeli International Neurofibromatosis Center (GIINFC), Tel Aviv University, Tel Aviv, Israel.
  • 2 Pediatric Radiology, Tel-Aviv Medical Center, The Gilbert Israeli International Neurofibromatosis Center (GIINFC), Tel Aviv University, Tel Aviv, Israel.
  • 3 Department of Pediatric Neurosurgery, Dana Children's Hospital, Tel-Aviv Medical Center, The Gilbert Israeli International Neurofibromatosis Center (GIINFC), Tel Aviv University, 6th Weizmann St., 64239, Tel-Aviv, Israel. sconsts@netvision.net.il.
PMID: 32524182 DOI: 10.1007/s00381-020-04697-1
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.

Keywords

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

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