Primary and Secondary Ocular Adnexal Lymphoma: A Single Institutional Experience

The incidence of ocular adnexal lymphoma (OAL) is rare and usually presents in the setting of central nervous system (CNS) involvement. There are no rigid guidelines for the treatment of OAL, most probably because of the variety of characteristics of the disease.

To analyze clinical pathological features, therapy and outcomes of patients with primary and secondary OAL.

Retrospective chart review of 17 consecutive patients diagnosed with OAL at Moffitt Cancer Center from 2004–2011. Characteristics of the participants were median age 68 years, 15 (88%) white, 2 (12%) Hispanic, and 11 (65%) male. Chlamydia serology testing was negative in all patients tested. Secondary OAL patients were staged per the Ann Arbor Staging System, 2 (22%) stage III and 7 (78%) stage IV. The primary OAL patients were staged utilizing the TNM staging system for OALs (Coupland et al, Arch Pathol Lab Med, 2009). Six (75%) patients were stage T1 and 2 (25%) patients were stage T2.

Seventeen patients with a diagnosis of OAL were evaluated in our institution. Patients with OAL are commonly stratified into 2 groups, primary and secondary. Eight (50%) of the patients were diagnosed with primary OAL; of these there were 4 (50%) marginal zone, 3 (37%) diffuse large B cell, and 1 (12%) follicular lymphoma. Nine (50%) patients were diagnosed with secondary OAL; 4 (44%) marginal zone, 1 (11%) diffuse large B cell, 1 (11%) mantle cell, 1 (11%) CLL, and 2 (22%) progressed from low grade to diffuse large B cell lymphoma. In the primary OAL, radiation in combination with systemic chemotherapy was the preferred treatment in diffuse large B cell lymphoma and radiation was preferred in patients with low-grade lymphoma. In secondary OAL, systemic chemotherapy was the preferred treatment for aggressive lymphoma. The choice of systemic Rituximab, radiation, or observation was the preferred treatment of low-grade lymphoma. Aggressive primary OAL had a relapse rate of 2 (66%) patients with a median time to progression of 8 months. Aggressive secondary OAL demonstrated a relapse rate of 50% with median time to progression 6 months. All patients who experienced relapsed disease received salvage chemotherapy. No cases of relapse were observed in the low-grade, primary or secondary, OAL patients. Median duration of response in low-grade primary lymphoma was 6 months and the low-grade secondary lymphoma was 54 months.

In our patient population diffuse large B cell lymphoma and marginal zone lymphoma were the most common diagnoses. Ocular adnexal lymphoma has been associated with the presence of CNS disease and it is estimated that 80–90% of patients diagnosed with OAL will experience progression to the CNS. Treatment depends on the extent of disease and the subtype of lymphoma that is histologically identified. Treatment may consist of involved field radiation in localized disease and has approximately less than 10% local recurrence rate. Intravitreal methotrexate and itraorbital injections of Rituximab or a combination of localized radiation and systemic high dose methotrexate are also options for treatment. In the event that there is systemic disease, single agent IV Rituximab or standard chemotherapy regimens such as CHOP-R or CVP-R in conjunction with ocular directed therapy. When disease is localized to the ocular compartment, the burden of disease is low and there is a greater chance of eradicating the disease. The delay in diagnosis increase the risk of CNS involvement and decreases overall survival.

No relevant conflicts of interest to declare.