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We present a large single\u2010institution retrospective study of 72 patients. The median age was 64\u2009years, 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\u2010treated patients. With a median follow\u2010up of 6.7\u2009years, relapse\/progression and death occurred in 19.4% each, with one relapse within the RT field. The 10\u2010year progression\u2010free survival (PFS) and overall survival (OS) were 68.4% (95% CI 52.8%\u201379.8%) and 89.4% (95% CI 77.4%\u201395.2%), respectively. The 10\u2010year rate for time to progression from diagnosis was 22.5% (95% CI 11.6%\u201335.7%). The 10\u2010year PFS and OS of MALT\u2010IPI 0 versus 1\u20132 were 83.3% versus 51.3%, (<jats:italic>p<\/jats:italic>\u00a0=\u00a0.022) and 97.6% versus 76.6%, (<jats:italic>p<\/jats:italic>\u00a0=\u00a0.0052), respectively. The following characteristics were associated with shorter survival: age\u2009&gt;\u200960\u2009years (PFS: HR\u00a0=\u00a02.93, 95% CI 1.08\u20137.95; <jats:italic>p<\/jats:italic>\u00a0=\u00a0.035, OS: HR\u00a0=\u00a09.07, 95% CI 1.17\u201370.26; <jats:italic>p<\/jats:italic>\u00a0=\u00a0.035) and MALT\u2010IPI 1\u20132 (PFS: HR\u00a0=\u00a02.67, 95% CI 1.12\u20136.31; <jats:italic>p<\/jats:italic>\u00a0=\u00a0.027, OS: HR\u00a0=\u00a06.64, 95% CI 1.45\u201330.37; <jats:italic>p<\/jats:italic>\u00a0=\u00a0.015). CR following frontline therapy was associated with longer PFS (HR\u00a0=\u00a00.13, 95% CI 0.04\u20130.45; <jats:italic>p<\/jats:italic>\u00a0=\u00a0.001), 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\u00a0=\u00a00.34, 95% CI 0.12\u20130.96 <jats:italic>p<\/jats:italic>\u00a0=\u00a0.041 and SHR\u00a0=\u00a00.11, 95% CI 0.03\u20130.36; <jats:italic>p<\/jats:italic>\u2009&lt;\u2009.001, respectively). Patients with PCEMZL treated with frontline RT exhibit excellent long\u2010term survival, and the MALT\u2010IPI score appropriately identifies patients at risk for treatment failure.<\/jats:p>","DOI":"10.1002\/ajh.26591","type":"journal-article","created":{"date-parts":[[2022,5,13]],"date-time":"2022-05-13T03:59:47Z","timestamp":1652414387000},"page":"148-158","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Long\u2010term outcomes of patients with conjunctival extranodal marginal zone lymphoma"],"prefix":"10.1002","volume":"98","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8909-5194","authenticated-orcid":false,"given":"Eduardo Edelman","family":"Saul","sequence":"first","affiliation":[{"name":"Department of Internal Medicine Jackson Memorial Hospital\/University of Miami, Miller School of Medicine  Miami Florida 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School of Medicine  Miami Florida USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2507-9347","authenticated-orcid":false,"given":"Sunil G.","family":"Iyer","sequence":"additional","affiliation":[{"name":"Division of Hematology, Department of Medicine, Sylvester Comprehensive Cancer Center University of Miami, Miller School of Medicine  Miami Florida USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gregor","family":"Rodriguez","sequence":"additional","affiliation":[{"name":"Division of Hematology\u2010Oncology, Department of Medicine University of Florida  Gainesville Florida USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Amrita","family":"Desai","sequence":"additional","affiliation":[{"name":"Department of Hematology\u2010Oncology, OHSU Knight Cancer Institute Oregon Health Sciences University  Portland Oregon USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jennifer 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