Systemic workup recommended for orbital and ocular adnexal lymphoma patients

This study is a retrospective review of follow-up data from all patients with orbital and ocular adnexal lymphoma treated by one of the authors during a seven-year period at The University of Texas M.D. Anderson Cancer Center in Houston. Forty-three patients were included, with 19 classified as having mucosa-associated lymphoid tissue (MALT) lymphoma, nine follicular lymphoma, nine diffuse large-cell lymphoma, three mantle cell lymphoma, two small lymphocytic lymphoma/B-chronic lymphocytic leukemia, and one large T-cell/natural killer cell lymphoma. Only 18 of the patients had disease limited to the local site at the time of orbital diagnosis; three of these patients subsequently developed systemic disease during the follow-up period. For the entire cohort, the five-year recurrence-free survival rate (both local and systemic) was 64.6%

The paper's authors emphasized that more than half of the patients included in the study had evidence of extraocular disease at diagnosis. Therefore, they recommended an extensive systemic workup of all patients with orbital and ocular adnexal lymphoma, continued surveillance by an oncologist, and consideration of systemic therapy.

Orbital specialists agree that the diagnosis of orbital and ocular adnexal lymphoma should prompt a referral to an oncologist so that a systemic workup can be performed. However, the high prevalence of multifocal disease demonstrated in this series of orbital and ocular adnexal lymphomas from a national cancer center likely reflects the referral nature of the practice, as well as the inclusion of patients who had already been diagnosed with systemic disease at the time of the orbital presentation.

The histopathologic subtype of the lymphoma cases in this series is somewhat atypical for orbital and ocular adnexal lymphomas, since only 19 of the 43 patients had MALT lymphomas. Analysis of other orbital lymphoma series suggests that up to 90% of patients have MALT lymphomas, with more aggressive subtypes, such as follicular and diffuse large-cell, seen less frequently. Additionally, 15 of the patients in this series had already been diagnosed with lymphoma prior to receiving a diagnosis of ocular adnexal involvement.