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Show Poster 291 Visual Outcomes in Neurofibromatosis Type-1 Patients with Optic Pathway Gliomas at a Tertiary Cancer Center Beena Shah1, Sruti Rachapudi1, John Slopis2, Zsila Sadighi2, Wafik Zaky2, Dan Gombos2, Andrew Whyte2, Nagham Al-Zubidi2 1 University of Texas Medical Branch, Galveston, Texas, USA, 2MD Anderson Cancer Center, Houston, Texas, USA Introduction: Investigate visual outcomes of optic nerve gliomas based on different treatment modalities Methods: Retrospective review of the institutional database between January 2000 to February 2021. Patients’ ophthalmic exams, imaging, oncology care, genetic testing, and progression of disease were analyzed. Primary outcome measures were visual outcomes (visual acuity (VA), visual fields deficits, and optic nerve atrophy). Secondary outcome measure was progression of disease. Presentation symptoms and age of diagnosis of NF1 were analyzed. Visual outcomes and disease progression was analyzed by treatment modality. Results: Out of 279 patients, we identified 66 patients who met inclusion criteria. The most common presentation of OPG was after routine screening following diagnosis of NF1. We found a final normal VA is greater among those under observation compared to other treatments (P value: 0.0143). 95% of observation patients maintained their VA over multiple exams. A statistically significant association between treatment status and optic atrophy (P value = 0.0069). The best outcome for optic nerve atrophy was with chemotherapy treatment (23% normal nerves) while surgery and multimodal treatments had the worst outcome (100% with moderate or severe optic atrophy). Surgery had a significantly greater progression rate and observation has a significantly lower progression rate (P <0.001). Overall, patients who underwent surgery alone had the highest progression rate (100%). Conclusions: Our data supports routine screening for OPGs with neuro-imaging in all patients at the time of NF1 diagnosis. Patients undergoing observation maintained an 81% progression-free disease course. In traditionally treated patients, surgery had the worst visual outcomes and progression rates while radiation had the lowest progression rates and better visual outcomes. Immunotherapy holds a promising future for progression-free disease and visual outcomes. Future prospective study would help find causal factors for differences in treatment modalities and visual outcomes. References: 1) Thirunavu VM, Mohammad LM, Kandula V, Beestrum M, Lam SK. Vision Outcomes for Pediatric Patients With Optic Pathway Gliomas Associated With Neurofibromatosis Type I: A Systematic Review of the Clinical Evidence. J Pediatr Hematol Oncol. 2021 May 1;43(4):135-143. 2) Fisher MJ, Loguidice M, Gutmann DH, Listernick R, et al. Visual outcomes in children with neurofibromatosis type 1-associated optic pathway glioma following chemotherapy: a multicenter retrospective analysis. Neuro Oncol. 2012 Jun;14(6):790-7. Epub 2012 Apr 3. 3) José P, Couceiro R, Passos J, Jorge Teixeira F. Visual Outcomes of Optic Pathway Glioma Treated With Chemotherapy in Neurofibromatosis Type 1. J Pediatr Ophthalmol Strabismus. 2021 Sep 1:1-8. Epub ahead of print. 4) Acharya S, Quesada S, Coca K, et al Long-term visual acuity outcomes after radiation therapy for sporadic optic pathway glioma. J Neurooncol. 2019 Sep;144(3):603-610. Epub 2019 Aug 14. Abstract Type: Epidemiological Study (cohort, case-control, and cross-sectional studies, including electronic health record analyses and chart reviews with subgroup comparisons) Keywords: tumors, optic neuropathy, chemotherapy and radiation injury, visual fields Financial Disclosures: The authors had no disclosures. Grant Support: None. Contact Information: Beena Shah, bmshah@utmb.edu 450 | North American Neuro-Ophthalmology Society |
