title: Ocular adnexal lymphoma: assessment of a tumor-node-metastasis staging system.
abstract: OBJECTIVE
  To assess distribution, correlations, and prognostic effect of tumor (T), node (N), and metastasis (M) staging on relapse and survival.
  
  
  METHODS
  Retrospective clinical review.
  
  
  METHODS
  Sixty-three patients diagnosed with primary ocular adnexal lymphoma (OAL) between January 1986 and November 2011.
  
  
  METHODS
  Complete ocular examination and systemic evaluation were performed. Patients were staged according to the American Joint Committee on Cancer (AJCC) seventh edition tumor-node-metastasis (TNM) clinical staging system for OAL and followed every 6 to 12 months (median follow-up, 27.9 months).
  
  
  METHODS
  Relapse defined as lymphoma recurrence in the initial site of presentation, the contralateral ocular adnexal structures, or other systemic site and overall survival.
  
  
  RESULTS
  There were 40 men (63.5%). The median age was 65 years (range, 24-85 years). The affected site was the conjunctiva in 27 patients (42.9%), orbit in 38 patients (60.3%), and eyelid in 3 patients (4.8%). The histologic subtype was extranodal marginal zone lymphoma (EMZL) in 51 patients (81.0%). A total of 14 patients (23.3%) had T1, 42 patients (70.0%) had T2, 1 patient (1.7%) had T3, and 3 patients (5.0%) had T4 disease. A total of 48 patients (82.8%) had N0 disease, and 10 patients (17.2%) had N1-4 disease. M stage was M0 in 47 patients (81.0%) and M1 in 11 patients (19.0%). With advanced T stage, there was an increase in both N1-4 (P = 0.045) and M1 disease (P = 0.041). M1 disease was greater among patients with N1-4 disease compared with N0 stage (50.0% vs. 12.5%, P = 0.003). Overall, 18 patients (28.6%) relapsed and 6 patients (9.5%) died. In Cox analysis, relapse was not associated with T stage (hazard ratio [HR], 1.14 per 1 level increase, P = 0.71), N stage (HR, 1.47; P = 0.51 N1-4 vs. N0), or M stage (HR, 1.22; P = 0.76 M1 vs. M0). T stage was not associated with survival (HR, 0.86; P = 0.81), whereas N1-4 had marginally worse survival than N0 (HR, 5.35; P = 0.07), and M1 had worse survival than M0 (HR, 9.27; P = 0.008).
  
  
  CONCLUSIONS
  The TNM staging system for primary OAL is useful for precise characterization of extent of local disease. Although T stage does not predict relapse or survival, N1-4 and M1 stages indicated less favorable survival.
  
  
  BACKGROUND
  The author(s) have no proprietary or commercial interest in any materials discussed in this article.
authors.affiliation: Department of Ophthalmic Oncology, Cole Eye Institute, Cleveland Clinic, Cleveland, Ohio 44195, USA.
authors.forename: Mary E
authors.initials: ME
authors.lastname: Aronow
authors.forename: Craig A
authors.initials: CA
authors.lastname: Portell
authors.forename: Lisa A
authors.initials: LA
authors.lastname: Rybicki
authors.forename: John W
authors.initials: JW
authors.lastname: Sweetenham
authors.forename: Arun D
authors.initials: AD
authors.lastname: Singh
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D003230:Conjunctival Neoplasms / Q000401:mortality / Q000473:pathology*; D005134:Eye Neoplasms / Q000401:mortality / Q000473:pathology*; D005260:Female; D006801:Humans; D008207:Lymphatic Metastasis; D018442:Lymphoma, B-Cell, Marginal Zone / Q000401:mortality / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D009918:Orbital Neoplasms / Q000401:mortality / Q000473:pathology*; D011379:Prognosis; D012189:Retrospective Studies; D015996:Survival Rate; D014057:Tomography, X-Ray Computed; D014463:Ultrasonography; D055815:Young Adult