Clinical patterns and prognostic outcomes of Asian ocular adnexal marginal zone lymphoma

Background Ocular adnexal marginal zone lymphoma (OAMZL) is the most common subtype of primary ocular lymphoma and has been rising in incidence in Asian populations. Methods We conducted a retrospective review of 95 patients diagnosed with OAMZL within a multi-ethnic cohort from Singapore. Clinical characteristics, survival outcomes including overall survival (OS) and progression-free survival (PFS), and SUVmax values on staging 18-FDG-PET/CT were investigated. Results The cohort comprised 60 males and 35 females, with a median age of 58 years (25–88). Median follow-up was 92 months. The most common sites involved were the orbit (49.5%) and lacrimal gland (23.2%). Most patients presented with stage 1 disease (72.6%). 5-year OS and PFS for the whole cohort was 94.9% and 84.1% respectively. Factors significantly associated with poorer OS included advanced (stage 2–4) disease (HR 6.26, 95% CI: 1.69–23.19, p  = 0.0061), older age above 56 years (HR 13.48, 95% CI: 3.98–45.74, p  < 0.0001), and higher MALT-IPI scores of 2–3 compared to low (0) and intermediate (1) scores (HR 9.28, 95% CI: 1.24–69.11, p  < 0.0001 and HR 10.99, 95% CI: 1.34–89.94, p  < 0.0001), respectively. Older age (HR 2.49, 95% CI: 1.12–5.55, p  = 0.0260) and advanced disease (HR 2.47, 95% CI: 1.07–7.03, p  = 0.0348) were significantly associated with poorer PFS. Median SUVmax of the lesions was 5.6 (2.1–9.6), with significantly higher values in advanced disease. Conclusion Our study illustrates the favourable prognosis of OAMZL in an Asian cohort, although particular factors may portend worse survival outcomes.