Purpose: Although systemic or eyelid involvement by ocular adnexal lymphoma carries a worse prognosis, there have been few reports of the outcome in relation to clinical presentation. The outcome of malignant ocular adnexal lymphoma was, therefore, related to presenting clinical symptoms and signs.
Design and methods: A retrospective, noncomparative case-note review of 326 patients treated in the Orbital Clinic at Moorfields Eye Hospital. The associations between presenting symptoms or signs and three outcome measures (v.i.) were assessed by univariate and multiple variable regression together with Kaplan-Meier analysis.
Main outcome measures: (i) Presence of extra-orbital disease at the time of presentation; (ii) development of systemic lymphoma after new presentation with solely ocular adnexal disease; and (iii) death attributable to widespread lymphoma.
Results: Presentation with disseminated disease was rarer with over 1 year's ophthalmic symptoms (odds ratio (OR) 0.7; 95% CI 0.5-0.9) and much more frequent with bilateral adnexal disease (OR 5.8; 95% CI 3.0-11.2). With solely adnexal disease at presentation, subsequent extra-orbital lymphoma was more frequent and earlier with lacrimal gland disease (as compared to those without; hazard ratio (HR) 1.9; 95% CI 1.2-4.5) or with eyelid disease (compared to those without; HR 2.4; 95% CI 1.2-4.5), or with bilateral disease (compared to unilateral disease; HR 2.6; 95% CI 1.4-5.2). Prior or concurrent systemic disease was the most significant predictive factor for lymphoma-related death (HR 6.8; 95% CI 4.3-10.9), but tumour-related death was also commoner and earlier with bilateral disease (HR 2.4; 95% CI 1.4-4.0) or where a relative afferent papillary defect was present (HR 2.8; 95% CI 1.6-4.9). Similarly, the rate of tumour-related death was slightly less where symptoms had been present for more than a year (HR 0.8; 95% CI 0.7-1.0) and slightly greater in the elderly (HR 1.03; 95% CI 1.01-1.05). Conjunctival lymphoma had the lowest rate of extra-orbital spread and lymphoma-related death, the rate of these two events being sequentially greater for patients with predominantly deep orbital lymphoma, lacrimal gland lymphoma, or eyelid lymphoma.
Conclusion: These data suggest that presenting symptoms and signs of patients with ocular adnexal lymphoma are significantly associated with the risk of systemic disease at orbital presentation, the rate of subsequent spread, and the rate of lymphoma-related death.