Hindsø TG, et al. - Researchers conducted this largest cohort study to characterise the clinical features of ocular adnexa (OA)-extranodal marginal zone B-cell lymphoma (EMZL). Participants in the study were 689 patients (median age was 62 years) with OA-EMZL. From January 1, 1980, through December 31, 2017, data were gathered. Data reported that the median follow-up time was 42 months. At the time of diagnosis, the majority of patients were diagnosed with primary OA-EMZL with Ann Arbor stage IE and American Joint Committee on Cancer stage T2. The most frequently involved anatomical structures were the orbit and the conjunctiva. Data reported that the 5-year, 10-year and 20-year disease-specific survival were 96%, 91% and 90%, respectively. According to findings, EMZL is a tumor that grows slowly and has great long-term survival. In localised OA-EMZL, low-dose EBRT as monotherapy should be considered. In those patients with disseminated disease, rituximab-based chemotherapy should be chosen.