Visual prognosis and survival outcomes in patients with ocular adnexal diffuse large B-cell lymphoma.

[AIM] To investigate the clinical characteristics and treatment outcomes, including visual function and overall survival (OS) of patients with ocular adnexal diffuse large B-cell lymphoma (OA-DLBCL).

[METHODS] This retrospective cohort study enrolled 29 patients diagnosed with OA-DLBCL based on histopathological biopsy between 2006 and 2023. Patients were stratified into two subgroups: primary OA-DLBCL (no prior history of lymphoma) and secondary OA-DLBCL (history of DLBCL at non-ocular adnexal sites). OS was defined as the time interval from OA-DLBCL diagnosis to death from any cause. Survival analysis was performed using the Kaplan-Meier method, and prognostic factors affecting OS were identified using multivariate Cox proportional hazards regression with a stepwise selection approach.

[RESULTS] The cohort included 24 patients with primary OA-DLBCL (13 males, 11 females; mean age: 61.36±18.29y) and 5 patients with secondary OA-DLBCL (2 males, 3 females; mean age: 50.94±18.17y). Among the primary OA-DLBCL subgroup, 12 patients (50%) presented with advanced disease (Ann Arbor stage IIIE-IV), and 16 patients (66%) were classified as T4 disease according to the tumor-node-metastasis (TNM) staging system. The mean final visual acuity was 1.72±1.10 in the primary group and 0.90±1.18 in the secondary group. The 5-year OS rate for the entire cohort was 27.7%. Multivariate analysis identified five factors significantly associated with poor survival outcomes: epiphora [adjusted hazard ratio (aHR), 36.95], atherosclerotic cardiovascular disease (aHR, 10.08), human immunodeficiency virus (HIV) infection (aHR, 12.47), M1 stage (aHR, 6.99), and secondary OA-DLBCL (aHR, 6.03; all <0.05). The median OS was 1.68y for primary OA-DLBCL and 1.12y for secondary OA-DLBCL.

[CONCLUSION] A substantial proportion of patients with primary OA-DLBCL present with advanced-stage disease at diagnosis. Epiphora, atherosclerotic cardiovascular disease, HIV infection, M1 stage, and secondary OA-DLBCL are independent prognostic factors for poor survival outcomes. These findings emphasize the urgent need for optimized therapeutic strategies and early screening protocols to improve the management of OA-DLBCL, particularly in developing countries.