Children with optic pathway gliomas (OPGs) frequently experience vision loss from their tumors. Standard front-line treatment using carboplatin-based chemotherapy typically produces only a modest benefit (eg, stabilization or 0.2 logMAR improvement) in visual acuity (VA). Bevacizumab is a monoclonal antibody that targets vascular endothelial growth factor and acts primarily as an anti-angiogenic agent. Recent reports suggest a qualitative improvement in vision after bevacizumab-based treatment in children with OPGs.
We report 4 cases of pediatric OPGs (2 neurofibromatosis type 1–related and 2 sporadic cases) that received treatment with bevacizumab due to progressive VA or visual field (VF) loss despite prior treatment with chemotherapy or proton-beam radiation. All 4 subjects demonstrated a marked improvement in their VA, VF, or both while receiving bevacizumab-based therapy. Three patients had complete resolution of their VA or VF loss in at least 1 eye—2 of whom had previously received bevacizumab therapy.
Conclusions and Relevance
Given that most patients with OPG-related visual impairment will show modest or no visual improvement with standard treatment, the incorporation of bevacizumab in these cases may greatly improve visual outcomes and should be considered in appropriate clinical situations.
Chiasmatic optic pathway glioma in an 11-year-old girl (case 1) with neurofibromatosis type 1 with dense temporal visual field loss (left panel). Improvement was seen in the visual field and tumor size/enhancement within 7 months of treatment with bevacizumab/irinotecan (middle panel). The visual field continued to improve and remained stable 6 months after treatment was discontinued (right panel).
Sporadic (non–neurofibromatosis type 1–related) optic pathway glioma in a 13-year-old girl (case 2) with previously normal visual acuity (20/20, OD/OS) that presented with a decline in visual acuity and a left hemifield defect respecting the vertical meridian 7 months after proton-beam therapy (left panel). Improvement in vision and tumor size/enhancement occurred within 2 months of starting bevacizumab treatment (middle panel) and remained stable at 12 months (right panel).
Sporadic chiasmatic/hypothalamic optic pathway glioma in a 6-year-old girl (case 4), with a decline in visual acuity 4 months after proton-beam therapy (left panel). Within 5 months of starting bevacizumab, her visual acuity improved, along with a noticeable decrease in tumor size and enhancement (middle panel). Bevacizumab was discontinued because of a positive treatment response. Vision remained stable despite an increase in size and enhancement (right panel) 9 months after discontinuation of bevacizumab. CF indicates counting fingers.
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