Predictors in Optic Pathway Gliomas in Neurofibromatosis Type 1: A Single Center Study

Area of Science:

• Neuro-oncology
• Ophthalmology
• Genetics

Background:

• Optic pathway gliomas (OPGs) are common NF1-dependent tumors.
• Retrospective analysis of NF1-OPG course, treatment indications, and outcomes is crucial.

Purpose of the Study:

• To analyze the clinical course, treatment indications, and therapeutic effects in 92 patients with NF1-OPGs.
• To identify factors influencing treatment decisions and outcomes in NF1-OPGs.

Main Methods:

• Retrospective analysis of demographics, clinical, genetic, imaging, and ophthalmological data.
• Multivariate analysis to identify significant factors for treatment and amblyopia.
• Evaluation of first and subsequent line oncological treatments.

Main Results:

• OPGs were unilateral in 55.4% and bilateral in 44.6% of patients.
• Oncological treatment was needed for 16.3%, with amblyopia and proptosis as significant predictors.
• Factors like strabismus, epilepsy, and optic nerve thickness influenced amblyopia development.

Conclusions:

• Modified MRI protocols are suggested: first MRI after age 1, reduced follow-up frequency for isolated cases, and no contrast for routine follow-ups.
• Caution is advised before initiating oncological therapy for visual acuity loss without confirmed progression on OCT, VEP, and MRI in specific patient groups.