Comprehensive information on clinical features and long-term outcomes of primary conjunctival extranodal marginal zone lymphoma (PCEMZL) is scarce. We present a large single-institution retrospective study of 72 patients. The median age was 64?years, and 63.9% were female. Stage I was present in 87.5%. Radiation therapy (RT) alone was the most common treatment (70.8%). Complete response (CR) was 87.5%, and 100% in RT-treated patients. With a median follow-up of 6.7?years, relapse/progression and death occurred in 19.4% each, with one relapse within the RT field. The 10-yr progression-free survival (PFS) and overall survival (OS) were 68.4% (95%CI 52.8-79.8%) and 89.4% (95%CI 77.4-95.2%), respectively. The 10-year rate for time to progression from diagnosis was 22.5% (95%CI 11.6-35.7%). The 10-yr PFS and OS of MALT-IPI 0 vs. 1-2 were 83.3% vs. 51.3%, (p=0.022) and 97.6% vs. 76.6%, (p=0.0052), respectively. The following characteristics were associated with shorter survival: age >60?years (PFS: HR=2.93, 95%CI 1.08-7.95; p=0.035, OS: HR=9.07, 95%CI 1.17-70.26; p=0.035) and MALT-IPI 1-2 (PFS: HR=2.67, 95%CI 1.12- 6.31; p=0.027, OS: HR=6.64, 95%CI 1.45-30.37; p=0.015). CR following frontline therapy was associated with longer PFS (HR=0.13, 95%CI 0.04-0.45; p=0.001), but not OS. Using the Fine and Gray regression model with death without relapse/progression as a competing risk, RT and CR after frontline therapy were associated with lower risk of relapse (SHR=0.34, 95%CI 0.12-0.96 p=0.041 and SHR=0.11, 95%CI 0.03-0.36; p<0.001, respectively). Patients with PCEMZL treated with frontline RT exhibit excellent long-term survival, and the MALT-IPI score appropriately identifies patients at risk for treatment failure.
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