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 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.