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Background/purpose To investigate the clinical characteristics of intraocular lymphoma and to evaluate two protocols of intravitreal methotrexate injection. Methods A retrospective chart review was conducted of newly-diagnosed intraocular lymphoma patients between January 2013 and January 2018 at National Taiwan University Hospital. Patients were divided into two groups. In Group A, intravitreal methotrexate was administered weekly for the initial 8 weeks, every 2 weeks for the following 12 weeks, and then monthly for 7 months. In Group B, intravitreal methotrexate was administered twice a week for the initial 2 weeks, weekly for the subsequent 2 weeks, once every 2 weeks for the next 1 month, and monthly for the last 10 months. Results A total of 12 patients were analyzed in the study; seven of these patients were allocated to Group A. Differences in the overall survival and progression-free survival between the two groups did not yield statistical significance. The median visual acuity was improved from LogMAR 0.46 to LogMAR 0.30 with borderline significance in Group A (p = 0.053). Two of seven patients in Group A and five of five patients in Group B developed punctate keratitis during intravitreal methotrexate injection treatment. Conclusion Intravitreal methotrexate is an effective and repeatable treatment for intraocular lymphoma. A new protocol with reduced frequency of intravitreal injections as shown in this study could potentially produce similar results without a worse prognosis, along with a decrease in the incidence of keratitis.

Vitreous biopsy is essential for establishing a definitive diagnosis and guiding potential therapy in patients with refractory vitritis and posterior uveitis, among other indications. Especially in cases where one must rule out intraocular lymphoma, obtaining a significant amount of undiluted vitreous increases diagnostic yield. We demonstrate our technique for obtaining a significant amount of undiluted vitreous using pars plana vitrectomy with air infusion.

Purpose To report a rare case of secondary intraocular lymphoma treated with intravitreal rituximab, following pars plana vitrectomy. Observations A 74-year-old female with history of parotid gland large B-cell lymphoma presented bilateral intraocular recurrence 10 years after the onset of the primary malignancy. Systemic work-up including PET/CT Scan, bone marrow biopsy, brain MRI and CSF analysis were unremarkable, and the patient declined to undergo systemic chemotherapy. Vision loss in her left eye was severe due to significant sub-retinal pigment epithelium (RPE) infiltration involving the macula; this eye was treated with external beam radiation therapy. On the right eye, the relapse manifested with vitreous involvement and fovea-sparing multifocal, sub-RPE infiltration for which the patient received monthly intravitreal rituximab injections, following pars plana vitrectomy. Through the course of therapy, the patient achieved good local control and maintained 20/20 visual acuity on her right eye. Brain magnetic resonance imaging (MRI) surveillance, every 3 months, was performed and revealed a cerebellar recurrence 24 months into the course of therapy. Conclusions and importance Our case illustrates how intravitreal immunotherapy with rituximab may provide local control of CD-20 positive secondary intraocular lymphoma; particularly in cases where systemic therapy is not amenable. In our case, a prior vitrectomy, did not appear to interfere with the therapeutic effect of intravitreal rituximab. Close quarterly surveillance with Brain MRI may help disclose central nervous system recurrences in such cases.

Primary vitreoretinal lymphoma (PVRL) is a rare ocular lymphoid malignancy, which consists a subset of primary central system lymphoma (PCNSL) and the most common type of intraocular lymphoma. The involvement of eyes is estimated to be approximately 20% of PCNSL, but the brain involvement may be up to 80% of PVRL. Typically, PVRL is a high grade B-cell malignancy of the retina and needs to be assorted from choroidal low-grade B-cell lymphomas. Very often PVRL masquerades and can be erroneously diagnosed as chronic uveitis, white dot syndromes or other neoplasms. Establishing an accurate diagnosis may involve cytology/pathology, immunohistochemistry, flow cytometry, molecular pathology and cytokine profile analysis. There is inadequate information about PVRL’s true incidence, ethnic/geographical variation and pathogenetic mechanisms. The therapeutic approach of PVRL involves aggressive chemotherapy and radiation therapy. Although PVRL tends to have a good response to the initial treatment, the prognosis is poor and the survival restricted due to the high relapse rates and CNS involvement. Keywords: B-cell lymphoma, Primary vitreoretinal lymphoma, Intraocular lymphoma, Primary CNS lymphoma, Masquerade syndrome