Ocular adnexal lymphoma: assessment of a tumor-node-metastasis staging system.

PURPOSE: To assess distribution, correlations, and prognostic effect of tumor (T), node (N), and metastasis (M) staging on relapse and survival. DESIGN: Retrospective clinical review. PARTICIPANTS: 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). MAIN OUTCOME MEASURES: 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. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Population-based assessment of clinical characteristics predicting outcome of conjunctival melanoma in whites.

PURPOSE: To identify the clinical determinants of prognosis and the incidence of malignant conjunctival melanoma in whites. METHODS: A nationwide search identified 85 patients in whom primary conjunctival melanoma was diagnosed in Finland between 1967 and 2000, all of whom were enrolled. Data were collected from the Finnish Cancer and Population Registries and from patients' charts in all involved hospitals. The age-specific and age-adjusted incidences were calculated. Clinical characteristics of the tumors were recorded and time to local recurrence and melanoma-specific survival were analyzed by Kaplan-Meier analysis and univariate and multivariate extended Cox regression. RESULTS: The annual crude incidence of conjunctival melanoma in Finland was 0.51 per million inhabitants, and the age-adjusted incidence (mean, 0.54) increased from 0.4 to 0.8 during the 34-year study period. The median age at diagnosis was 60 years (range, 20-90). Clinically detectable primary acquired melanosis preceded or accompanied the primary tumor in 61% of patients. The 5-year cumulative proportion of cases with local recurrence was 0.36 (95% confidence interval [CI], 0.25-0.48). The melanoma-specific 5-and 10-year mortalities were 0.20 (95% CI, 0.12-0.32) and 0.38 (95% CI, 0.26-0.53), respectively. By multiple-event Cox regression, nonlimbal location of the primary tumor predicted a short time to local recurrence (hazard ratio [HR] 1.81, P = 0.024). Nonlimbal location of the primary tumor (HR 4.08, P = 0.023) and increasing tumor thickness (HR 1.19 for each millimeter change, P=0.063) were associated with increased mortality. Local recurrence, analyzed as a time-dependent covariate, also increased mortality (HR 1.39 for each recurrence, P = 0.014). CONCLUSIONS: The incidence of conjunctival melanoma in the white population of Finland increased analogous to cutaneous melanoma. Nonlimbal tumors recur more often and are associated with decreased survival, independent of their greater thickness. Local recurrence contributes to mortality, whereas primary acquired melanosis was not associated with either outcome.