AI Article Synopsis

  • Lymphoma in the ocular adnexa is usually extranodal marginal zone (ENMZ) lymphoma; involved-field radiotherapy (IFRT) is the standard treatment for unilateral cases, while optimal care for more extensive cases remains uncertain.
  • A study of 95 patients identified key risk factors affecting progression-free survival, such as age, female gender, and a history of lymphoma, with IFRT improving outcomes for unilateral ENMZ OAL significantly.
  • Single-agent rituximab showed effectiveness for bilateral or systemic ENMZ OAL, but ocular progression requires careful monitoring, highlighting a need for more research on combined therapies for complex cases.

Article Abstract

Background: Lymphoma rarely presents in the ocular adnexa but is usually extranodal marginal zone (ENMZ) lymphoma when it does. Involved-field radiotherapy (IFRT) is the standard of care for unilateral disease, but the optimal management of more extensive disease is unclear.

Patients And Methods: We retrospectively evaluated the clinical characteristics and outcomes of 95 patients with ocular adnexal lymphoma (OAL) or uveal lymphoma treated or diagnosed at our institution. All patients identified were included in the risk factor analysis for progression-free survival (PFS). The initial treatment-related outcomes were assessed for ENMZ OAL only (n = 62).

Results: With a median follow-up of 32 months, significant risk factors for PFS after initial treatment were age (hazard ratio, 1.33; 95% confidence interval, 1.02-1.74), female gender (hazard ratio, 2.04; 95% confidence interval, 1.04-4.00), and a history of lymphoma (hazard ratio, 2.31; 95% confidence interval, 1.12-4.78). In ENMZ, IFRT was associated with improved PFS (median, 5.4 years; P < .001). Progression occurred in 7 of 39 (23%), with 6 of the 7 (86%) at systemic sites. Single-agent rituximab was typically used for bilateral ocular or systemic presentations of ENMZ OAL. Progression occurred in 7 of 11 (64%), with no progression at systemic sites. All progression events in those initially treated with rituximab occurred in the ocular adnexa.

Conclusion: The results of the present study have confirmed IFRT as the standard for unilateral ENMZ OAL. Single-agent rituximab was an effective agent for bilateral ocular or systemic ENMZ OAL, particularly for systemic control, but ocular progression should be closely monitored. Combined modality therapy should be studied further in bilateral and systemic ENMZ OAL.

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Source
http://dx.doi.org/10.1016/j.clml.2013.10.011DOI Listing

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