Survival and Recurrence in Vitreoretinal Lymphoma Simulating Uveitis at Presentation: The Possible Role of Combined Chemotherapy

ABSTRACT

Purpose

To investigate the role of combined systemic and local chemotherapy in improving the survival of patients with vitreoretinal lymphoma (VRL).

Methods

Patients with VRL consecutively seen from 2006 to 2020 were retrospectively reviewed; data on the presence and time of central nervous system (CNS) involvement and treatment regimen (systemic, local or combined chemotherapy) were collected. Overall survival (OS) and progression-free survival (PFS) were calculated for each group.

Results

Forty-three eyes of 22 subjects with histology-proven VRL were included. Mean time of survival was 64.8 months (SE±10.8). Twelve patients (57%) presented CNS involvement, which was significantly associated with progression (r = 0.48, P = .03) and death (r = 0.56, P = .009). The isolated primary VRL group had a 5-year OS of 80%. Combined systemic and local chemotherapy reduced the risk of death by 82% (hazard ratio 0.18[0.04– 0.85]) in the entire cohort.

Conclusion

Combined systemic and local chemotherapy significantly improved OS but not PFS of patients affected by VRL.

KEYWORDS:

• Vitreoretinal lymphoma
• uveitis masquerade syndrome
• CNS involvement
• survival
• chemotherapy

Acknowledgments

To Jacqueline Costa for English proofreading.To Chantal Adani for support in patients visits.

Author contributions

FG, RA, JM, LDS and LC wrote the draft of the manuscript. RA performed the statistical analyses. LC, VM, FG, LDS, DI, MC, EB, FI, AF, SL, EF, DN, IT and RV contributed to the writing of the protocol and researched data. LC, RA, SC, AZ, FM, AC, LF and CS interpreted data and critically revised the manuscript. LC is the guarantor of this work and, as such, had full access to all the data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis.

Declaration of interest

All authors certify that they have no affiliations with or involvement in any organization or entity with any financial interest (such as honoraria; educational grants; participation in speakers’ bureaus; membership, employment, consultancies, stock ownership, or other equity interest; and expert testimony or patent-licensing arrangements) or non- financial interest (such as personal or professional relationships, affiliations, knowledge, or beliefs) in the subject matter or materials discussed in this manuscript.