Comprehensive analysis of uveal mucosa-associated lymphoid tissue lymphomas: Epidemiological insights and radiotherapy utilization from the Surveillance, Epidemiology and End Results database

Uveal mucosa-associated lymphoid tissue lymphomas are rare, indolent ocular tumours affecting the choroid, ciliary body, or iris. Due to their rarity, demographic and treatment data remain limited. This retrospective study analysed uveal mucosa-associated lymphoid tissue lymphoma cases diagnosed between 2000 and 2021 from the Surveillance, Epidemiology and End Results (SEER) database across 17 registries. Twenty-seven cases of uveal mucosa-associated lymphoid tissue lymphoma were identified from the SEER database (2000-2021), representing 0.12 % of 22,246 extra-nodal MALT lymphomas, with an age-adjusted incidence of 0.014 per 1,000,000 person-years. Median age was 65-69 years, with a near-equal sex ratio and 74 % of patients being Caucasian. Most cases were stage I (74.1 %) at diagnosis. Radiotherapy alone was the most common treatment (55.6 %). At a median follow-up of 69 months, the 5-year overall survival rate was 78.9 %, with no relapses or lymphoma-related deaths, yielding 100 % cancer-specific and relapse-free survival. Radiotherapy proved highly effective, supporting its role as a primary treatment. This is the largest analysis to date, highlighting the excellent prognosis and suggesting potential for radiotherapy dose reduction to lower long-term toxicity.