Orbital lymphomas: a clinicopathologic study of a rare disease

To evaluate the clinicopathologic features and prognosis of patients with orbital lymphomas.

Clinical and pathologic data of 35 patients with biopsy-proven orbital lymphoma diagnosed at a tertiary care hospital from 1992 to 2001 were reviewed. Lymphomas were divided into low-grade and high-grade lymphomas. Survival of patients was compared according to age, gender, disease site, extent of disease, tumor grade, and treatment modality by using log rank test.

Median patient age was 75 years (23-94) and the male-to-female ratio was 1:2.9. Twenty-three patients (66%) were diagnosed with low-grade lymphoma, and 12 patients (34%) were found to have high-grade lymphoma. Among low-grade lymphomas, marginal zone lymphoma (n=6), follicle center cell lymphoma (n= 6), and small lymphocytic lymphoma (n=5) were common entities, whereas diffuse large cell B-cell lymphoma (n=5) was the most common entity in patients with high-grade lymphoma. Disease was clinically localized in 74% of patients at the time of diagnosis. Radiation alone or with chemotherapy was the primary treatment modality in 83% of patients. All except one patient had an objective response to therapy. Over the median follow-up period of 47 months (range, 1.5-141 months), disease recurred in 37% patients who achieved a complete response. The estimated 5- and 10-year survival rates were 64% and 42%, respectively. Overall, 13 (37%) patients died, 6 with high-grade and 7 with low-grade lymphoma. No clinical variable was found to be prognostically significant with respect to survival.

Orbital lymphoma is a disease of the elderly with a female preponderance. It tends to be localized to the orbit at the time of diagnosis and responds well to local or systemic therapy.