Incidence, Prevalence, and Ocular Comorbidities of Primary Vitreoretinal Lymphoma in the TriNetX Global Collaborative...

To characterize the epidemiology of primary vitreoretinal lymphoma (PVRL), a rare malignancy of the central nervous system (CNS) that presents with vitritis and chorioretinal infiltrates and is closely associated with primary CNS lymphoma. Limited literature describes an incidence between 0.023 and 0.46/100,000 that has been increasing. The epidemiology of PVRL remains poorly characterized in large-scale datasets. We evaluated the baseline demographics, incidence, and prevalence of PVRL and ocular comorbidities using the TriNetX Global Collaborative Network database.

A retrospective cohort study was conducted using TriNetX using the 10^th revision of the International Classification of Diseases codes for chorioretinal inflammation (H30) and other nonfollicular lymphoma (C83.8) to identify cases of PVRL in the TriNetX database. Data collected included demographics and comorbid ocular complications. The ten-year cumulative prevalence was calculated using TriNetX software.

Among 152,281,229 patients in the TriNetX network, 175 were identified as probable PVRL cases. The 10-year cumulative prevalence was estimated at 0.076 per 100,000 persons. The mean age at diagnosis was 59.4 years (standard deviation: 15.6; range: 19-90), and 59.52% were Caucasian. Postdiagnosis comorbidities included glaucoma (15.0%), cystoid macular edema (13.6%), and low vision (13.2%).

This study provides an updated, large population-based assessment of the 10-year cumulative prevalence of PVRL using a global electronic health record database. These findings contribute to the limited literature on PVRL prevalence and offer insights into disease burden across diverse international populations.