Primary ocular adnexal MALT lymphoma: A long-term follow-up study of 114 patients

Background: Although primary ocular adnexal MALT (mucosa-associated lymphoid tissue) lymphoma (POAML) is a recently recognized unique entity, its natural history, prognostic factors, behavior of progression and death, and standard initial management have not been fully elucidated. Methods: The data of 114 patients with histologically verified POAML who were treated at our institution between 1970 and 2003 were retrospectively analyzed. Results: With a median follow-up duration of 5.7 years (0.6-34.0), estimated overall survival (OS) rate and progression-free survival (PFS) rate at 10 years was 89% and 57%, respectively. Thirteen (11 %) patients died, but only three (3%) of them due to progressive lymphoma. Thirty-one (27%) patients progressed: eight who progressed at contra lateral sites were limited to those who had initially involved in the orbit (P = 0.036) and their OS and PFS were significantly longer (P = 0.035 and 0.039, respectively). Patients who initially received radiation-containing therapy were superior in PFS but not in OS to those initially treated with other modalities (P = 0.016 and 0.091, respectively). When we compared the outcomes of the observation cohort and the immediate therapy cohort, there were no significant differences in OS and PFS (P = 0.499 and 0.073, respectively). Conclusions: The majority of patients with POAML showed the behaviors of localized and indolent diseases. Our preliminary observation that no initial therapy is an acceptable approach for selected patients was confirmed. Considering the possible heterogeneity of POAML among initial sites, further investigations are warranted.