Samanthila Waduthantri, Anita Sook Yee Chan, Soon-Phaik Chee
Dendritiform keratic precipitates and large vitreous cells aid early recognition of VRL. The SRPEI size and presence of CIs may help infer the disease extent. The CIs may be associated with lower CNS dissemination risk and improved DSS.Overall, DSS is primarily influenced by systemic and CNS involvement rather than diagnostic delay.
PURPOSE: To characterize the clinical features of vitreoretinal lymphoma (VRL) and their prognostic significance in patients presenting to a tertiary eye center.
METHODS: We retrospectively reviewed medical records of 49 patients diagnosed with VRL between January 1997 and January 2022.
RESULTS: At presentation, 25 patients had primary VRL (PVRL), 7 had concurrent primary CNS lymphoma (PCNSL), and 17 had concomitant systemic lymphoma (CSL). The majority presented with dendritiform keratic precipitates (46.9%) and large vitreous cells (87.8%). Large sub-retinal pigment epithelium infiltrates (SRPEIs) (≥3-disc diameters [DD]) were common in PVRL (52.0%) and PCNSL (57.1%) but were absent in CSL. In PVRL, large SRPEIs were associated with shorter presentation time (Spearman's ρ = -0.57; p = 0.004) but were not associated with CNS dissemination (OR = 0.76; p = 0.65) or disease-specific survival (DSS) [HR = 1.26; p = 0.217]. The CSL typically presented with small SRPEIs ≤ 1-DD (64.7%) and choroidal infiltrates (CIs) (47.1%). The CIs were inversely associated with CNS dissemination (OR = 0.01; p = 0.03) and demonstrated a non-significant trend toward improved DSS (HR = 0.51; p = 0.3). Five-year DSS was 65.5% in PVRL, 42.9% in PCNSL, and 49.3% in CSL. The time to diagnosis was not associated with DSS (PVRL, p = 0.48; PCNSL, p = 0.97; CSL, p = 0.24).
CONCLUSION: Dendritiform keratic precipitates and large vitreous cells aid early recognition of VRL. The SRPEI size and presence of CIs may help infer the disease extent. The CIs may be associated with lower CNS dissemination risk and improved DSS.Overall, DSS is primarily influenced by systemic and CNS involvement rather than diagnostic delay.