Clinical characteristics of 95 patients with ocular adnexal and uveal lymphoma: Treatment outcomes in extranodal marginal zone subtype

Craig A. Portell, Mary E. Aronow, Lisa A. Rybicki, Roger Macklis, Arun D. Singh, John W. Sweetenham

Research output: Contribution to journalArticle

18 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)203-210
Number of pages8
JournalClinical Lymphoma, Myeloma and Leukemia
Issue number3
Publication statusPublished - Jun 2014
Externally publishedYes



  • Extranodal marginal zone lymphoma of mucosal associated lymphoid tissue
  • Ocular adnexal lymphoma
  • Radiation
  • Rituximab

ASJC Scopus subject areas

  • Hematology
  • Oncology
  • Cancer Research

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