Neurofibromatosis 1-associated optic pathway gliomas
- Childs Nerv Syst. 2020 Oct;36(10):2351-2361. doi: 10.1007/s00381-020-04697-1.
- 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. [email protected].
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.