Abstract

PurposeTo describe the visual acuity and anatomic outcomes of intravitreal methotrexate (MTX) for the treatment of primary vitreoretinal lymphoma (PVRL).MethodsSingle-center retrospective case series of patients with a diagnosis of PVRL treated with intravitreal MTX. Patient records were reviewed for demographic information, ocular exam findings, and treatment regimens including number of MTX injections. Clinical outcomes recorded included visual acuity (VA), time to partial (PR) or complete response (CR), disease-free survival, time to relapse, and any CNS progression.ResultsTen eyes of 7 patients (4 male, 6 female) were reviewed. The mean age ± standard deviation (SD) was 70 ± 12 years. Five patients had prior or concomitant diagnosis of primary CNS lymphoma with a history of systemic chemotherapy including MTX. Three eyes (30%) exhibited isolated vitreous involvement, four (40%) had subretinal lesions, and three (30%) presented with both vitreous and subretinal disease. Mean initial logMAR VA was 0.38 ± 0.52 (Snellen visual equivalent 20/50), while mean final logMAR VA ± SD was 0.34 ± 0.27 (Snellen visual equivalent 20/40) with a mean follow-up time of 26 months (Range, 3–49 months). Patients received an average of 6 intravitreal MTX injections (Range 1–10) over the course of treatment. Two patients received concomitant systemic chemotherapy. Mean time to either PR or CR was 57 days, and 6 eyes (60%) exhibited regression with no relapse after local treatment. For the 4 eyes that eventually relapsed, the mean time ± SD to first relapse was 193 days ± 155 days, and one eye experienced a second relapse. Two of 3 patients with subretinal disease showed complete regression with extended follow-up of 1 and 4 years following treatment with less than 3 doses of intravitreal MTX. One patient with PVRL developed CNS lymphoma during the study period. VA remained stable overall between the initial treatment visit, 3, 6, and 12-months (P > 0.05 for paired comparisons of VA over time).ConclusionsIntravitreal methotrexate was well-tolerated and led to local disease response in the majority of patients at approximately 2 months after initiation of treatment of intraocular lymphoma. Further studies on the efficacy of intravitreal treatment alone versus combined systemic and intravitreal treatment are warranted.

Highlights

  • Primary vitreoretinal lymphoma (PVRL), termed primary intraocular lymphoma (PIOL) occurs when lymphoma cells, most commonly diffuse large B-cellAnthony et al International Journal of Retina and Vitreous (2021) 7:72 non-Hodgkin lymphoma, proliferate within the posterior segment of the eye, with tissue invasion that may involve the retina, subretinal space, vitreous and/or optic nerve [1, 2]

  • Early PVRL or PIOL is known as a masquerade syndrome, often presenting with vague complaints and insidious onset that can mimic other conditions such as chronic uveitis [5]

  • We describe our tertiary referral experience center experience regarding the disease presentation and treatment response of patients with PVRL who were treated with intravitreal methotrexate

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Summary

Introduction

Primary vitreoretinal lymphoma (PVRL), termed primary intraocular lymphoma (PIOL) occurs when lymphoma cells, most commonly diffuse large B-cellAnthony et al International Journal of Retina and Vitreous (2021) 7:72 non-Hodgkin lymphoma, proliferate within the posterior segment of the eye, with tissue invasion that may involve the retina, subretinal space, vitreous and/or optic nerve [1, 2]. Primary vitreoretinal lymphoma (PVRL), termed primary intraocular lymphoma (PIOL) occurs when lymphoma cells, most commonly diffuse large B-cell. 20% of patients have concurrent central nervous system (CNS) involvement, or primary CNS lymphoma (PCNSL), and 90% of patients PVRL will develop CNS lymphoma within 29 months [3]. While considered a rare disease, the incidence of PCNSL has risen over the past decade, contributing to increasing disease prevalence and importance of recognition and treatment [4]. Early PVRL or PIOL is known as a masquerade syndrome, often presenting with vague complaints and insidious onset that can mimic other conditions such as chronic uveitis [5]. Protean symptoms and signs, delayed referrals, and the need for invasive vitrectomy surgery to establish a diagnosis, may contribute to a delay in the diagnosis of PVRL and treatment

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