Poster: ABCL-312: Survival Outcomes of Primary Intraocular Lymphoma

Objectives To report the clinical presentation, treatment, and outcome of patients diagnosed with primary intraocular lymphoma (PIOL) at a single institution in the time period between July 1996 and December 2020. Materials and Methods We identified 38 patients who were consecutively diagnosed or seen between 1996 and the present and had pathology-confirmed PIOL. Data regarding initial presentation, staging, treatment, progression, and survival were collected and analyzed for trends related to survival. Results In our cohort of 38 patients, the mean age is 65 years, although it is not limited by age and can present in other age groups. Most patients (33 cases, 85%) were diagnosed with vitrectomy. At initial presentation, bilateral eye involvement was present in 11/38 cases (25%), progression to the CNS was present in 10 cases (23%), and 4 patients had CSF involvement (37 had CSF evaluation; therefore, 9% of those evaluated, 5 patients had atypical CSF results). Thirty-five (80%) patients had B-cell lymphomas and 20% T-cell lymphomas. Tumors commonly expressed B-cell markers (CD20, PAX5, CD19, or/and CD79). Twenty-nine patients were treated with ocular targeted external beam radiation or whole-brain radiation (66%). Methotrexate-based systemic plus intrathecal regimen with an average of 3.5 cycles was given to 33 patients (75%). Although 19 patients (47%) achieved complete remission after first-line therapy, 17 relapsed (39%) and received second-line treatment, and 3 received third-line therapy, which includes chemotherapy with or without radiation. Those who relapsed were less likely to have undergone radiation and methotrexate chemotherapy. Of the 38 patients, 30 (76%) underwent autologous stem cell transplant. The overall survival is 75% (29 patients), while progression-free survival is 41% (16 patients), with a median follow-up of 49.7 months. Conclusion PIOL remains a rare disease, which, in opposition to its scarcity, has a poor prognosis, being potentially fatal. The use of ocular radiotherapy, combined with HD MTX-based systemic chemotherapy, has become the mainstay of ocular lymphoma treatment, highlighting the highly therapeutic effects through significantly improved patient outcomes. Prospective studies are needed to improve outcomes in this rare disease.