Clinical Patterns and Prognostic Outcomes of Asian Ocular Adnexal Marginal Zone Lymphoma

Abstract

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 maximum standardized uptake value (SUVmax) on staging F-18 fluorodeoxyglucose positron emission tomography/computed tomography (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%). Five-year OS and PFS for the whole cohort were 94.9% and 84.1%, respectively. Factors significantly associated with poorer OS included advanced (stage 2-4) disease (hazard ratio (HR) 6.26, 95% confidence interval (CI): 1.69 - 23.19, P = 0.0061), older age above 58 years (HR = 15.29, 95% CI: 4.47 - 52.3, P < 0.0001), and higher mucosa-associated lymphoid tissue International Prognostic Index (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 = 2.41, 95% CI: 1.07 - 5.43, P = 0.0330) 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.

Conclusions: Our study illustrates the favorable prognosis of OAMZL in an Asian cohort, although particular factors may portend worse survival outcomes.