Orbital and ocular adnexal lymphoma: a review of epidemiology and prognostic factors in Taiwan.

PURPOSE: To investigate the clinical features, prognostic outcomes of patients with orbital and ocular adnexal lymphoma (OALs) in Taiwanese cohort.

METHODS: Total 112 patients with OALs were retrospectively reviewed. Demographic information such as age, gender, lymphoma subtype, tumor location and treatment modalities were recorded. The primary endpoints were disease-specific survival (DSS), and progression-free survival (PFS).

RESULTS: The mean patient age was 59.0 ± 15.5 years (range, 23-92 years). The major histopathologic subtypes were mucosa-associated lymphoid tissue (MALT) lymphoma in 76 patients (67.9%), followed by diffuse large B-cell lymphoma (DLBCL) (9.8%), follicular cell lymphoma (FL) (8.0%), and small lymphocytic lymphoma (SLL) (5.4%). The anatomical locations for OALs were the orbit (44 patients, 39.3%), the conjunctiva (31 patients, 27.7%), the lacrimal gland (28 patients, 25.0%), and the eyelid (8 patients, 7.1%). With a mean follow-up time of 74.5 ± 59.8 months (range 6-342 months), the DSS for all patients were 93.1%, 87.7%, and 68.8% at 60, 120, and 180 months' follow-up, respectively. The PFS at 60, 120, and 180 months' follow-up were 86.2%, 61.2%, and 44.6%, respectively. Regarding the analysis of prognostic factors, patients with high grade lymphoma subtype and advanced stage exhibited a worse prognosis.

CONCLUSIONS: MALT type lymphoma constitutes most of OALs in Taiwan and occurs more frequently than in Western countries. Patients with MALT lymphoma, FL, SLL and earlier stage have favorable outcomes compared with patients of high grade lymphoma and Ann Arbor stage IV lymphoma.