Abstract
Primary vitreoretinal lymphoma (PVRL) is a high-grade extranodal non-Hodgkin lymphoma, with limited prospective data to inform practice. High rates of central nervous system (CNS) relapse contribute to its poor prognosis. This international multicentre retrospective cohort study aimed to characterise real-world contemporary practice and outcomes in PVRL (2010-2022). Sixty patients were included from 11 centres across Australia, Singapore, Canada and the United Kingdom. Most patients had systemic therapy included in their initial management (63%) either alone or in combination with local therapies; 13% had upfront autologous stem cell transplantation (ASCT). The overall response rate was 78%. With a median follow-up of 68 months, the median progression-free survival (PFS) was 25 months, with a median overall survival (OS) of 73 months. Neither incorporation of systemic therapy into initial treatment nor upfront ASCT demonstrated a statistically significant impact on PFS or OS. The 5-year cumulative incidence of CNS relapse was 33%, with front-line systemic therapy being the only predictive factor for CNS relapse in a multivariate model, hazard ratio of 0.30 (95% CI 0.09-0.98, p = 0.05). Concerning heterogeneity in real-world approaches to diagnosis, staging and management approaches were identified. Further international collaborative efforts are required to address the unmet need in this rare entity.