Treatments for Ocular Adnexal Lymphoma: A Report by the American Academy of Ophthalmology.

PURPOSE: To review the literature to determine the efficacy of available treatments for ocular adnexal lymphoma (OAL) and to evaluate the outcomes and complications of treatments in patients older than 13 years.

METHODS: A literature search was conducted last in March 2017 in the PubMed and Cochrane Library databases for English-language original research investigations that evaluated treatment outcomes for OAL. The searches identified 307 unique citations, and 27 studies were selected according to the criteria outlined for this assessment.

RESULTS: The 27 studies reviewed comprised 2009 patients. Seventy-five percent of the cases reported were extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT). Twenty-five studies reported results using radiotherapy with or without chemotherapy or surgery. The local control rate of MALT lymphomas with treatments involving radiotherapy averaged 95.9%. Distant and local relapses can occur, and in studies reporting only on MALT lymphomas (884 patients), the 5-year and 10-year disease-free survival rates were reported to be 86.4% and 78.7%, respectively. However, overall survival in patients receiving radiotherapy remained very good, with the 5-year and 10-year survival rates reported to be 93.8% and 84.9%, respectively. Studies that included data on multiple histologic subtypes of lymphoma or non-MALT lymphomas (988 patients) reported local control rates to be 93.1%; 5-year and 10-year disease-free survival rates to be 75.7% and 71.0%, respectively; and 5-year and 10-year overall survival rates to be 78.9% and 73.5%, respectively. Studies on the use of doxycycline for MALT lymphomas (137 patients) reported complete responses of between 4.4% and 13%. Complete and partial responses combined were between 26.7% and 65%. Disease-free survival was not reported for these 2 studies, although progression-free survival was reported to be between 55% and 60.9%. The most frequently reported complications of treatment were cataracts (12.1%) and dry eye (8.5%).

CONCLUSIONS: For MALT lymphomas, local control, disease-free survival, and overall survival are good with radiation treatment. The results of treatment of non-MALT lymphomas using radiotherapy also were good, but they were not as favorable as the treatment results of MALT lymphomas.