Vitreoretinal Lymphoma: Presentation, Diagnosis, and Initial Treatment

Journal Highlights

Ophthalmology Retina, January 2024

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Given the rarity of vitreoretinal lymphoma, there is insufficient medical evidence to guide clinical decisions. To address this gap, Smith et al. created an international vitreoretinal lymphoma registry for reporting clinical characteristics, diagnostic experiences, and initial treatments. Data for the first 80 cases suggest that bilateral presentation and visual loss are common with vitreoretinal lymphoma. Determining the diagnosis usually involved cellular tests. Initial treatments varied but often included intravitreal chemotherapy.

The electronic registry was launched in January 2020 and contains reports from ophthalmologists worldwide who manage patients with vitreoretinal lymphoma. The registry data include logMAR VA at presentation, diagnostic techniques, and initial treatments.

By Dec. 31, 2022, information was available for 80 patients with vitreoretinal lymphoma (48 women, 32 men), reported from Asia, Oceania, Europe, North America, and the Middle East. Altogether, 132 eyes were affected. The median age at diagnosis was 64 years (range, 32-91 years). At presentation, the lymphoma was bilateral in 52 patients (65%). The eye was an initial site of the lymphoma in 62 patients (78%). Among 127 eyes with lymphoma at presentation, the vitreous was involved in 113 (89%) and was the only affected eye tissue in 51 (40%). The retina was involved in 58 eyes (46%) and was the only affected eye tissue in 11 (9%). The median logMAR VA of the worse-seeing eye was 0.50.

In most cases (80%; n = 64), the lymphoma was identified in ocular specimens, usually of the vitreous (89%). Cellular studies were conducted in 92%, the most common of which was cytology. Tumor gene analysis was performed for 21 patients (33% of 64), and cytokine assays were used in 13 cases (20% of 64). For 76 patients (95%), treatment was begun within six months of diagnosis and included ocu­lar (48%), extraocular (21%), and ocular plus extraocular (26%) approaches. The most common ocular treatment was intravitreal methotrexate (95%).

Diagnosing vitreoretinal lymphoma can be challenging, said the authors. The gold standard has been cytopathologic evidence of malignant lymphocytes, but molecular analyses as an adjunct is encouraged in recent consensus guidelines. Moreover, there are calls to move beyond cytology to ensure timely treatment. Ophthalmic imaging, particularly OCT, is improving confidence in clinical assessments, said the authors. As the registry grows, it should shed more light on the diagnostic tech­niques, management decisions, and clinical outcomes associated with this malignancy.

The original article can be found here.