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Bilateral orbital tumors can increase long-term risk of developing systemic lymphoma

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A long-term study has found that patients with orbital lymphoid tumors and no systemic disease at presentation were at a 33% risk for developing systemic lymphoma at 10 years that increased to 72% among patients with bilateral tumors.

In order to determine the risk for systemic disease progression, Hakan Demirci, MD, and colleagues retrospectively reviewed clinical data obtained for 209 eyes of 160 patients with orbital lymphoproliferative tumors. Of these 160 patients, 106 who had no systemic disease at presentation were followed at 6-month intervals.

Of these 106 patients, systemic lymphoma subsequently developed in 17 patients (16%) over an average interval of 152 months.

Nine of these patients (53%) had identical systemic lymphoma and orbital tumor cell types, and eight patients (47%) had different systemic and orbital lymphoma cell types, the authors noted.

Of these eight patients with dissimilar systemic and orbital cell types, four (50%) with orbital atypical lymphoid hyperplasia and two (25%) with orbital mucosa-associated lymphoid tissue lymphoma subsequently developed systemic small lymphocytic lymphoma, according to the study.

In addition, two patients (25%) with orbital small lymphocytic lymphoma subsequently developed systemic diffuse large B-cell lymphoma, the authors reported.

Kaplan-Meier survival analysis of the 106 patients with orbital lymphoma and no systemic lymphoma at presentation revealed a 33% incidence of systemic disease at 10 years.

However, among 24 of these patients who had bilateral tumors, the analysis showed a 72% incidence of systemic disease at 10 years, the authors noted.

Among the 82 patients with unilateral orbital tumors alone, systemic lymphoma subsequently developed in 12% of patients at 3, 5 and 10 years.

"Multivariate analysis showed that bilateral involvement at presentation was the only significant factor predictive of [systemic lymphoma]," the authors said in the September issue of Ophthalmology.