Good survival prognosis for most unilateral ocular adnexal lymphoma patients

This retrospective series of primary unilateral ocular adnexal lymphoma (OAL) cases found that most patients had indolent lymphomas with no progression and a good prognosis for survival. However, a minority of patients presented with more aggressive lymphomas, such as diffuse large B-cell lymphoma, mantle cell lymphoma, immunoblastic lymphoma and diffuse large cell lymphoma, and had a more guarded prognosis.

This study, published in the November/December issue of Ophthalmic Plastic and Reconstructive Surgery, included all 122 consecutive biopsy-confirmed unilateral OAL (unilateral stage IE) cases seen at one institution during a 30-year period.

Indolent B-cell lymphomas (mucosa-associated lymphoid tissue (MALT)-type marginal zone, follicular and small lymphocytic lymphoma) represented 80 percent of cases. Among these patients, following initial therapy, their progression-free and disease-specific survival was 71 percent and 98 percent at five years and 61 percent and 90 percent at 10 years, respectively. Typically aggressive lymphomas were found in 7 percent of patients. These lymphomas were more likely to progress (P < 0.01), progress earlier (log rank, P < 0.01) and succumb to disease (P < 0.01) in a shorter interval (log rank, P < 0.01). Overall, 24.4 percent of patients experienced disease progression after initial therapy, with most of this occurring within five years. Approximately 75 percent of patients remained relapse free for at least 10 years.

The authors found that indolent lymphomas tended to respond well to radiation as the sole primary treatment and relapse in the contralateral orbit or head and neck region where they are easy to detect and treat. However, the authors caution that, in rare cases, more aggressive tumors can present in this manner and should be managed accordingly.