title: Clinical characteristics of 95 patients with ocular adnexal and uveal lymphoma: treatment outcomes in extranodal marginal zone subtype.
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.
  
  
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Hematologic Oncology and Blood Disorders, Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH. Electronic address: cportell@virginia.edu.
authors.forename: Craig A
authors.initials: CA
authors.lastname: Portell
authors.affiliation: Cole Eye Institute, Cleveland Clinic, Cleveland, OH.
authors.forename: Mary E
authors.initials: ME
authors.lastname: Aronow
authors.affiliation: Department of Hematologic Oncology and Blood Disorders, Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH.
authors.forename: Lisa A
authors.initials: LA
authors.lastname: Rybicki
authors.affiliation: Department of Radiation Oncology, Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH.
authors.forename: Roger
authors.initials: R
authors.lastname: Macklis
authors.affiliation: Cole Eye Institute, Cleveland Clinic, Cleveland, OH.
authors.forename: Arun D
authors.initials: AD
authors.lastname: Singh
authors.affiliation: Department of Hematologic Oncology and Blood Disorders, Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH.
authors.forename: John W
authors.initials: JW
authors.lastname: Sweetenham
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D000970:Antineoplastic Agents / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D003131:Combined Modality Therapy; D005134:Eye Neoplasms / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy*; D005260:Female; D006801:Humans; D018442:Lymphoma, B-Cell, Marginal Zone / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D009362:Neoplasm Metastasis; D009367:Neoplasm Staging; D011379:Prognosis; D012189:Retrospective Studies; D000069283:Rituximab; D016896:Treatment Outcome; D047368:Tumor Burden; D014604:Uveal Neoplasms / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy*; D055815:Young Adult
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents; D000069283:Rituximab
keywords: Extranodal marginal zone lymphoma of mucosal associated lymphoid tissue; Ocular adnexal lymphoma; Radiation; Rituximab