Presentation, Diagnostic Testing and Initial Treatment of Vitreoretinal Lymphoma

Vitreoretinal lymphoma is a malignancy with high mortality. Incidence is rare, and there is a lack of medical evidence to direct management. This work describes presentation, diagnostic testing and first treatment approaches in a recently diagnosed and treated patient cohort.Clinical registry-based observational study.48 women and 32 men (32 to 91 years, median of 64 years) diagnosed with vitreoretinal lymphoma.An international network of ophthalmologists reported clinical features and management of patients presenting with vitreoretinal lymphoma between January 1, 2020 and December 31, 2022 via an electronic platform.Visual acuity at presentation (LogMAR); basis for diagnosis; first treatment.Vitreoretinal lymphoma was bilateral at presentation in 65% of patients (N = 52) and an initial site of lymphoma in 78% (N = 62). In 127 eyes with lymphoma at presentation, vitreous was involved in 89% (N = 113) and the only involved eye tissue in 40% (N = 51), and retina was involved in 46% (N = 58) and the only involved eye tissue in 9% (N = 11). Median LogMAR visual acuity of the worse-seeing eye was 0.50. The lymphoma was diagnosed from ocular specimens in 80% (N = 64), usually vitreous (N = 57, 89% of 64 patients), and on other clinical information in 20% (N = 16). Cellular studies were performed in 92% (59 of 64 patients), most often cytology. Tumor gene analysis was used in 21 patients (33% of 64) and cytokine assays were used in 13 patients (20% of 64). For 76 patients (95%), treatment was initiated within 6 months of diagnosis and included ocular (48%, N = 38 of 76 patients), extraocular (21%, N = 17 of 76), and ocular plus extraocular (26%, N = 21 of 76) approaches. Intravitreal methotrexate was the most common ocular treatment (N = 83 of 87 eyes, 95%).Using data collected from 80 patients diagnosed with vitreoretinal lymphoma since 2020, we show that visual impairment is common, and that management commonly involves diagnosis by cellular tests and treatment with intravitreal chemotherapy.