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This 78YO female was referred for asymptomatic findings in her right fundus. Vision was 20-40 OD and 20-30 OS.
Optos color RG imaging shows diffuse submacular creamy-yellow discoloration with chorioretinal folds. Numerous drusen are noted outside the macula. OCT scanning shows diffuse, irregular choroidal thickening with overlying irregular RPE folds. Shallow subretinal fluid is noted temporally. Fundus autofluorescence (FAF) shows variable hyper-FAF along the periphery of this lesion. Fluorescein angiography shows variable speckled subretinal leakage. The choroidal folds are visible more peripherally as linear hypofluorescent lines.
A presumed diagnosis of uveal lymphoma was made, and she was referred to oncology for a systemic workup to rule out systemic lymphoma.
Unfortunately, our patient was immediately lost to follow-up.
Learning Points:
Uveal lymphoma is a rare, typically indolent, B-cell non-Hodgkin lymphoma that most often involves the choroid. Secondary choroidal lymphoma from systemic disease is morphologically high-grade and associated with more severe ocular findings, thus requiring a metastatic workup before a diagnosis of primary uveal lymphoma can be made (Mashayekhi et al, Ophthalmology 2014;121:342-351).
The diagnosis is often difficult to establish due to a broad differential diagnosis that includes amelanotic uveal melanoma, uveal metastases, uveal effusion syndrome, posterior scleritis, and miscellaneous uveitides (Aronow et al., Ophthalmology 2014;121:334-341). Enhanced depth imaging OCT shows a pathognomonic pattern of calm, rippled, or undulating topography (Shields et al, Retina 2014;34:1347-1353). Treatment is most often with external beam radiotherapy for localized disease, while systemic or combined therapies are considered for more aggressive or disseminated cases.
Ophthalmol Retina. 2026 Aug;10(8):844-851. doi: 10.1016/j.oret.2026.03.019.
Paclitaxel cumulative incidence for macular edema and cystic maculopathy in 1.4%. More common with older age, female sex, dyslipidemia, liver disease, and greater cumulative dose — Retrospective, US HIRA database.
Purpose: To determine the incidence and risk factors for maculopathy among new paclitaxel users in South Korea.
Design: Retrospective, nationwide cohort study.
Participants: Adults who initiated paclitaxel therapy between 2015 and 2023 without a prior diagnosis of maculopathy, as recorded in the Health Insurance Review and Assessment database.
Methods: Incidence rate ratios (IRRs) were calculated by comparing pretreatment and posttreatment periods, whereas cumulative incidence was assessed using Kaplan-Meier analysis. Multivariable Cox regression models were applied to estimate adjusted hazard ratios (HRs) for demographic and clinical risk factors, including a dose-response analysis.
Main outcome measures: The main outcome measures were as follows: (1) cumulative incidence of macular edema and associated cystic maculopathy, (2) IRRs comparing posttreatment with pretreatment periods, and (3) adjusted HRs for demographic and clinical predictors.
Results: The number of annual paclitaxel users gradually increased during the study period, rising from 7375 in 2015 to 18 337 in 2023. Among 98 246 patients treated with paclitaxel, the cumulative incidence of macular edema or associated cystic maculopathy was 1.4% by the end of the study period. The IRR for the posttreatment versus pretreatment period was 4.08 (95% confidence interval [CI], 3.64-4.56) for macular edema and 4.11 (95% CI, 3.72-4.55) for macular edema or associated cystic maculopathy. Independent risk factors for maculopathy included duration of use (HR, 1.01; 95% CI, 1.00-1.01), age (HR, 1.01; 95% CI, 1.01-1.02), female sex (HR, 1.47; 95% CI, 1.22-1.77), dyslipidemia (HR, 1.47; 95% CI, 1.24-1.74), and liver disease (HR, 1.32; 95% CI, 1.11-1.57). Patients in the highest tertile of cumulative dose had a higher risk compared with those in the lowest tertile (HR, 1.33; 95% CI, 1.08-1.65 for overall maculopathy and HR, 1.28; 95% CI, 1.03-1.59 for macular edema).
Conclusions: Paclitaxel therapy is associated with a measurable increase in the risk of macular edema or associated cystic maculopathy. Older age, female sex, dyslipidemia, liver disease, and greater cumulative dose further amplified this risk.
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