Objective: To investigate the clinical features of conjunctival lymphoid tumors and factors predictive of systemic lymphoma.
Design: Retrospective, observational case series.
Setting: Clinical practice of ocular oncology.
Participants: The participants included 117 consecutive patients with lymphoid tumors of the conjunctiva treated at the Oncology Service of Wills Eye Hospital between 1974 and 1999.
Main outcome measure: The main outcome measure was the development of systemic lymphoma. Cox proportional regression models were used to calculate the risk of eventual systemic lymphoma. Kaplan-Meier survival estimates were used to analyze the development of systemic lymphoma as a function of time.
Results: There were 55 males (47%) and 62 females (53%); 110 patients (94%) were white and 7 patients (6%) were African AMERICAN: The mean age at ocular presentation was 61 years, and the conjunctival lymphoid infiltrate was unilateral in 72 patients (62%) and bilateral in 45 patients (38%). In 8 patients (7%), initial unilateral conjunctival disease evolved into bilateral involvement over a mean of 32 months. Additional sites of ocular involvement were found in 27 patients (23%) and included lymphoid tumor in the eyelid in 3 cases, orbit in 18, choroid in 5, and vitreous in 1. Systemic lymphoma was known to exist before ocular diagnosis in 16 patients; (14%; for a mean of 51 months) and was found subsequent to ocular diagnosis in 20 patients (17; at a mean of 21 months). Therefore, of 117 patients with conjunctival lymphoid infiltration, 36 (31%) had or eventually developed systemic lymphoma, and 81 (69%) did not manifest systemic lymphoma during the mean follow-up of 38 months. Of the 64 patients with unilateral conjunctival involvement, 11 (17%) manifested systemic lymphoma, and of the 53 patients with bilateral involvement, 25 (47%) manifested systemic lymphoma. By univariate analysis, the clinical factors at date first seen predictive of the presence or development of systemic lymphoma included location of the tumor at an extralimbal site (fornix or midbulbar conjunctiva; P = 0.02) and increasing number of conjunctival tumors (P = 0.02). Using Kaplan-Meier life table analysis of those 101 patients who had conjunctival lymphoid tumor(s) and no evident systemic lymphoma at presentation, systemic lymphoma was eventually discovered in 7% of patients at 1 year, 12% at 2 years, 15% at 5 years, and 28% at 10 years. Overall, only one patient (<1%) died of systemic lymphoma, at 28 months after the diagnosis of the ocular disease.
Conclusions: Lymphoid tumors of the conjunctiva are associated with systemic lymphoma in 31% of patients. Systemic lymphoma is found more often in those patients with forniceal or midbulbar conjunctival involvement and in those with multiple conjunctival tumors. Long-term systemic follow-up is advised, because related systemic lymphoma can manifest many years later.