Abstract: Objective To investigate the clinicopathological features and prognosis of intraocular lymphoma. Design Retrospective case series. Participants 12 cases of enucleation patients with intraocular lymphoma diagnosed from 2012 to 2019. Methods Clinical features, histopathological morphology and prognosis in medical records were reviewed. Main Outcome Measures Tumor site, pathological classification and prognosis. Results There were 7 males in 12 cases. Mean age was 58.2±10.9 years. None of the patients had a history of lymphoma in other parts of the body. The most common presentation was visual acuity loss (11/12), with a mean course of 12.6±10.3 months. The most common site of intraocular lymphoma was choroid (9/12), in which the retina was involved in 1 case (1/9) , iris and ciliary body were involved in 6 cases (6/9). Only 1 case (1/12) was located in both the ciliary body and the iris. Only the retina involved was found in 2 cases (2/12). The pathologic classification of primary choroidal lymphoma was mainly diffuse large B-cell lymphoma (DLBCL) (5/9), followed by mucosa-associated lymphoid tissue marginal B-cell lymphoma (MALT) (3/9). 1 case of primary iridociliary body lymphoma was DLBCL. 2 cases of primary retinal lymphoma were DLBCL. Extraocular diffusion occurred in 4 patients, 3 with MALT and 1 with mantle cell lymphoma (MCL). Both of the 2 patients with central nervous metastasis were DLBCL. All cases were followed up for 11-99 months. All the DLBCL patients received systemic chemotherapy with methotrexate drug after enucleation. All the 6 patients with tumors located in the choroid and iridociliary body were survived, and 1 of the 2 patients with tumors located only in the retina died and 1 was lost to follow-up. None of the 3 MALT patients with tumors located in choroid received systemic chemotherapy, and all of them have survived without disease at present. Of the 2 DLBCL patients with central nerve metastasis, 1 patient with whole uveal and retinal involvement survived after systemic chemotherapy, while 1 patient with retinal involvement only died after chemotherapy. Conclusion Choroidal lymphoma was the most common intraocular lymphoma. DLBCL was the most common pathological type, followed by MALT.  The survival prognosis of choroidal DLBCL patients after systemic chemotherapy was good. Choroidal MALT patients are prone to extraocular metastasis, and the survival prognosis is good despite no postoperative chemotherapy. All retinal lymphomas are DLBCL with poor survival and prognosis. (Ophthalmol CHN, 2021, 30: 295-299)

Key words: intraocular lymphoma, diffuse large B-cell lymphoma, mucosa-associated lymphoid tissue marginal B-cell lymphoma