PURPOSE: To analyze the clinical features, treatment outcomes, and prognostic factors associated with lymphoproliferative lesions of the ocular adnexa in a Korean population. METHODS: Data from 69 patients treated for adnexal lymphoproliferative disease in the 12-year period from 1991 to 2002 were retrospectively evaluated. RESULTS: The 69 patients had a median age of 46 years (range, 15-73 years); 60 of these patients were diagnosed with extranodal marginal zone B cell lymphomas (MALT lymphomas) and had 10 year cause specific survival and relapse free survival rates of 95.6% and 82.6%, respectively. Of 6 patients (8.7%) with concurrent systemic lymphoma, including 4 diagnosed with stage IV disease, 3 died from lymphoma. Only one patient with a primary ocular adnexal MALT lymphoma developed systemic lymphoma, which was treated with surgical resection. Local recurrence either at the primary site or in the fellow eye occurred in 11.6% of patients at a median follow-up time of 102 months (range, 79-132 months), and was controlled using repeat irradiation in all cases. Statistical analysis showed the presence of concurrent systemic lymphoma, bilateral disease, and an advanced stage at diagnosis, were linked to lymphoma-related death (Log-rank test, p < 0.05) and systemic progression (Fisher's exact test, p < 0.05), and that the tumor location was not a prognostic factor for lymphoma-related death or relapse at any site. CONCLUSIONS: Ocular adnexal lymphoproliferative disease in Koreans occurred at a relatively young age, and was mostly orbitally located and of the MALT subtype, which is highly localized and rarely associated with extraorbital relapse. Primary or secondary status, stage at presentation, and bilaterality were found to be prognostic factors.
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