Ocular adnexal lymphoma

This is the authors' abstract. We don't add key points for this paper.

PURPOSE: To characterize demographic, clinical, and histopathologic features of ocular adnexal lymphoma (OAL) at a single institution. METHODS: Retrospective review of all patients with pathologic diagnosis of OAL between 2015 and 2020. RESULTS: There were 133 patients with OAL, with a median age of 65 years (range 23–97) and a slight female predominance (male: female = 1:1.46), ( n = 79, 59%). The majority of tumors were non-Hodgkin B-cell lymphomas ( n = 131, 99%), most frequently Extranodal Marginal Zone B-Cell Lymphoma (EMZL, n = 93, 70%), followed by follicular lymphoma ( n = 21, 16%), chronic lymphocytic leukemia/small lymphocytic lymphoma ( n = 7, 5%), diffuse large B-cell lymphoma ( n = 5, 4%), and mantle cell lymphoma ( n = 5, 4%). The most frequently involved sites included the orbit ( n = 85, 64%) and conjunctiva ( n = 43, 32%). Information was available on oncologic staging in 78 (59%), treatment in 82 (62%), and follow-up in 75 (56%) patients. By the Ann-Arbor classification system, patients were classified as IE (54/78, 69%), IIE (9/78, 12%), IIIE (6/78, 8%), and IVE (9/78, 12%). The most common treatments included external beam radiotherapy (standard and ultra-low-dose) (48/82, 59%), biologics (22/82, 27%), and surgical excision with cryotherapy (14/82, 17%) (some patients had >1 therapy). Median follow-up time was 24 months (range 0–221 months). Recurrence was observed in 13% (10/75) with a median time to recurrence of 60 months (95% confidence interval 47–73 months). Excision with cryotherapy as a sole treatment modality was associated with earlier recurrence ( P = 0.003). CONCLUSION: In this largest single-center study of OAL, we found that most OAL were Ann-Arbor Stage IE EMZL, occurring in older patients with a female predominance. Early recurrence was noted in tumors treated with excision and cryotherapy alone.