This is a multicentre retrospective study of seven eye centres of 689 patients with ocular adnexal extranodal marginal zone b-cell lymphoma (OA-EMZL). The median follow-up time was 42 months and the median age was 62 years. Fifty-five percent were women; 82% were primary OA-EMZL with Ann Arbor stage 1E of 90% and 61% with American joint committee on cancer stage T2 at the time of diagnosis. Sixty-six percent involved the orbit and 37% involved the conjunctiva. The 5, 10 and 20 year disease-specific survival (DSS) were 96%, 91% and 90% respectively. Stage 1E patients treated with external beam radiation (EBRT) as monotherapy had a 95% of 10-year DSS; better than those treated with chemotherapy (10-year DSS of 86%). Stage 3E/4E treated with rituximab-based chemotherapy had a better DSS (10-year of 96%) than those treated with chemotherapy only (10-year DSS of 63%). The authors concluded that EMZL is an indolent tumour and long-term survival is good. Poor outcome was associated with secondary disease, relapses or progression of OA, larger sample size, standardised staging and treatment regimens, prospective study and longer follow-up period are needed.
Ocular adnexal marginal zone b-cell lymphoma
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