Rapidly progressive primary vitreoretinal lymphoma with optic disc involvement and retinal detachment.

[PURPOSE] This report presents a diagnostically challenging and aggressive case of primary vitreoretinal lymphoma, demonstrating the potential for recovery following early diagnostic vitrectomy and prompt intravitreal methotrexate.

[OBSERVATIONS] A 42-year-old woman presented with a 1-week history of temporal visual field loss in the right eye and was referred to our hospital for suspected retinal detachment. At presentation, best-corrected visual acuity was 20/16 in the right eye. Fundus examination revealed vitreous cells, retinal hemorrhages, retinal vasculitis, and extensive subretinal fluid. Optical coherence tomography showed preretinal hyperreflective deposits with a level-like appearance. Fluorescein angiography demonstrated vascular leakage and peripheral non-perfusion, while Goldmann perimetry revealed a central scotoma. Despite empiric antimicrobials for presumed infectious uveitis, vision rapidly deteriorated to light perception with worsening of the central scotoma. This profound functional decline was associated with marked optic disc swelling and progressive retinal detachment. Contrast-enhanced orbital magnetic resonance imaging demonstrated subtle retrobulbar enhancement, supporting infiltrative optic neuropathy consistent with the central dysfunction. Diagnostic vitrectomy identified a monoclonal B-cell population with an elevated interleukin-10/-6 ratio and clonality of the immunoglobulin heavy-chain gene, confirming primary vitreoretinal lymphoma. Intravitreal methotrexate was initiated the following day. Over eight injections, vision dramatically improved from light perception to 20/20, with resolution of the central scotoma, vitreous opacity, and optic disc swelling.

[CONCLUSIONS AND IMPORTANCE] This case illustrates a fulminant presentation of primary vitreoretinal lymphoma, characterized by rapidly progressive optic disc involvement and extensive serous retinal detachment. Despite the severity, early diagnostic vitrectomy followed by prompt intravitreal methotrexate enabled anatomical and functional recovery.