Presenting Clinical Features of Vitreoretinal Lymphoma

BACKGROUND: Vitreoretinal lymphoma is a rare cancer, widely recognised to present diagnostic challenges. We aimed to report information that would support the clinician in considering this diagnosis. METHODS: We interrogated the International Vitreoretinal B-Cell Lymphoma Registry for clinical data from patients newly diagnosed with vitreoretinal lymphoma from January 1, 2020. Demographics, tumour features, optical coherence tomography (OCT) signs, and visual acuities were analysed with descriptive and comparative statistics. RESULTS: The study cohort included 57 men and 81 women, averaging 65.8 years of age. Men presented at a younger mean age than women, including 40.4% who presented before 60 years (versus 21.0% of women). Diagnosis was by cell-based and molecular tests on ocular samples in 80.4% and from indirect clinical evidence in 19.6%; if known, lymphoma type was diffuse large B-cell in 99.0%. The tumour was bilateral in 65.0% of patients, and involved vitreous in 90.6% and retina in 60.1%. Extraocular tissues were involved concurrently in 26.8%, within the central nervous system in 23.2% and outside in 5.1%. 96.4% of patients had ocular imaging by OCT: abnormalities were present in 80.5%, including subretinal and sub-retinal pigment epithelial nodules. Snellen visual acuity of 227 eyes ranged widely, with a median of 6/12. Visual acuities were ≥ 6/12 in 54.6% of eyes, < 6/19 in 33.0%, and ≤ 6/60 in 22.9%. CONCLUSIONS: We have comprehensively described features that characterise the presentation of vitreoretinal lymphoma. Future research using the International Vitreoretinal B-Cell Lymphoma Registry will focus on visual acuity and survival outcomes.