Aims To compare the clinical characteristics and prognosis of primary conjunctival versus other ocular adnexal extranodal marginal zone B-cell lymphoma (EMZL).
Methods Retrospective review of clinical records for all consecutive patients with primary ocular adnexal EMZL treated from March 1995 to December 2015.
Results 198 patients were evaluated including 81 with primary conjunctival and 117 with other ocular adnexal EMZL. Conjunctival EMZL was found at a younger age (40.3±10.3vs 54.3±13.4 years, p<0.0001) with a female predilection (75.3%vs35.9%, p<0.0001) and had a higher rate of bilaterality (40.7%vs11.1%, p<0.0001) and a lower rate of systemic involvement (1.2%vs9.4%, p=0.030) compared with other ocular adnexal EMZLs. Conjunctival EMZL also showed a higher rate of complete response to primary treatment (98.8%vs89.5%, p=0.016) than other ocular adnexal EMZLs; however, recurrence and lymphoma-related death rates were not different between the two groups (p>0.05). Kaplan-Meier estimates for disease-specific survival at 5 and 10 years were 98.2% and 98.2% in conjunctival and 98.6%, respectively, and 95.2% in other ocular adnexal EMZLs. Univariate analysis showed that systemic involvement was negatively associated with conjunctival tumour location and positively associated with age (OR=0.35 and OR=1.05, p=0.045 and p=0.012, respectively), and treatment response was positively associated with conjunctival tumour location and negatively associated with age (OR=3.02 and OR=0.95, p=0.035 and p=0.009, respectively).
Conclusions Conjunctival EMZL shows unique demographic characteristic compared with other ocular adnexal EMZLs. Long-term follow-up is required due to late recurrence in ocular adnexal EMZL.
- Conjunctival lymphoma
- ocular adnexal lymphoma
- Extranodal Marginal Zone B-cell Lymphoma (EMZL)
- survival analysis
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Contributors Each author took part in the design of the study, contributed to the data collection, participated in writing the manuscript and all agreed to accept equal responsibility for the accuracy of the content of the paper.
Competing interests None declared.
Patient consent Detail has been removed from this case description/these case descriptions to ensure anonymity. The editors and reviewers have seen the detailed information available and are satisfied that the information backs up the case the authors are making.
Ethics approval Samsung Medical Center’s institutional review board and ethics committee.
Provenance and peer review Not commissioned; externally peer reviewed.