Treatment Strategies in Primary Vitreoretinal Lymphoma

Abstract

IMPORTANCE The best treatment option for primary vitreoretinal lymphoma (PVRL) without signs of central nervous system lymphoma (CNSL) involvement determined on magnetic resonance imaging or in cerebrospinal fluid is unknown. OBJECTIVE To evaluate the outcomes of treatment regimens used for PVRL in the prevention of subsequent CNSL. DESIGN, SETTING, AND PARTICIPANTS A retrospective cohort study was conducted at 17 referral ophthalmologic centers in Europe. We reviewed clinical, laboratory, and imaging data on 78 patients with PVRL who did not have CNSL on presentation between January 1, 1991, and December 31, 2012, with a focus on the incidence of CNS manifestations during the follow-up period. INTERVENTIONS The term extensive treatment was used for various combinations of systemic and intrathecal chemotherapy, whole-brain radiotherapy, and peripheral blood stem cell transplantation. Therapy to prevent CNSL included ocular radiotherapy and/or ocular chemotherapy (group A, 31 patients), extensive systemic treatment (group B, 21 patients), and a combination of ocular and extensive treatment (group C, 23 patients); 3 patients did not receive treatment. A total of 40 patients received systemic chemotherapy. MAIN OUTCOMES AND MEASURES Development of CNSL following the diagnosis of PVRL relative to the use or nonuse of systemic chemotherapy and other treatment regimens. RESULTS Overall, CNSL developed in 28 of 78 patients (36%) at a median follow-up of 49 months. Specifically, CNSL developed in 10 of 31 (32%) in group A, 9 of 21 (43%) in group B, and 9 of 23 (39%) in group C. The 5-year cumulative survival rate was lower in patients with CNSL (35% [95% CI, 50% to 86%]) than in patients without CNSL (68% [95% CI, 19% to 51%]; P = .003) and was similar among all treatment groups (P = .10). Adverse systemic effects occurred in 9 of 40 (23%) patients receiving systemic chemotherapy; the most common of these effects was acute renal failure. CONCLUSIONS AND RELEVANCE In the present series of patients with isolated PVRL, the use of systemic chemotherapy was not proven to prevent CNSL and was associated with more severe adverse effects compared with local treatment.

High frequency of MYD88 mutations in vitreoretinal B-cell lymphoma: a valuable tool to improve diagnostic yield of vitreous aspirates

“…Most cases are classified as diffuse large B-cell lymphomas (DLBCLs) and represent either primary VRL or secondary ocular involvement by primary central nervous system lymphoma (PCNSL). [1][2][3][4][5] VRLs are highly aggressive tumors that need to be distinguished from the second most common intraocular lymphoma, low-grade choroidal extranodal marginal zone B-cell lymphomas, which are not associated with central nervous system (CNS) disease but which do occasionally extend extraocularly. 2 VRL is usually diagnosed by cytologic, immunocytochemical, and molecular examination of vitreous body aspirates.…”

Combined treatment of primary vitreoretinal lymphomas significantly prolongs the time to first relapse

“…A multicentre study by Riemens et al 24 stated that incidence of CNS relapse was similar in patients treated with only local ocular therapy as in patients with systemic treatment (various combinations of systemic and intrathecal chemotherapy, whole-brain radiotherapy and peripheral blood stem cell transplantation). Riemens et al 24 emphasised the severe adverse effects accompanying systemic chemotherapy.…”

Insights from a Case of Vitreoretinal Lymphoma

“…A multicenter study showed no evidence that systemic therapy prolongs survival in patients presenting with vitreoretinal lymphoma [6]; however, this situation may be improving thanks to therapeutic advances. Whether the systemic therapy has prevented overt CNS disease in our patient is uncertain.…”

“…In most centers, vitreoretinal lymphoma is treated focally, with ocular radiotherapy or intravitreal chemotherapy [3-5]. About one-third of patients develop CNS disease within 5 years [6]. At the University of California San Francisco (UCSF), we treat vitreoretinal lymphoma with systemic chemotherapy, usually in combination with rituximab and lenalidomide, a second-generation immunomodulatory agent [7].…”