Primary, Unilateral Ocular Adnexal Lymphoma: Disease Progression and Long-Term Survival

Abstract

This investigation aimed to improve our understanding of the disease characteristics and clinical course for primary unilateral ocular adnexal lymphoma (OAL). In this retrospective case series, all consecutive biopsy confirmed cases of lymphoma confined to a single ocular adnexa (unilateral stage 1E) during a 30-year period were included. All histologic classification conformed to the current World Health Organization classification. Descriptive statistics and standard survival analyses were performed. 122 patients were included in this study. The most common primary unilateral OAL were indolent B-cell lymphomas (mucosa-associated lymphoid tissue (MALT)-type marginal zone, follicular and small lymphocytic lymphoma) representing 80% of cases. Typically aggressive lymphomas were found in only 7% of patients (diffuse large B-cell lymphoma, immunoblastic lymphoma, diffuse large cell lymphoma, and mantle cell lymphoma). Overall, 24.4% of patients experienced progression of their disease after initial therapy, the majority occurring within 5 years. For the 80% of OAL of indolent B-cell type, progression free and disease specific survivals were 71 and 98% at 5 years and 61 and 90% at 10 years, respectively. Diffuse large B-cell lymphoma was more likely to progress overall (p < 0.01) and progress earlier (log rank, p < 0.01). Additionally, these patients were also more likely to succumb to disease (p < 0.01) in a shorter interval (log rank, p < 0.01). Most primary unilateral OALs are indolent lymphomas with good prognosis for survival and freedom from progression. However, a minority present with more aggressive lymphomas (diffuse large B-cell lymphoma, mantle cell lymphoma, immunoblastic lymphoma, and diffuse large cell lymphoma), carrying a more guarded prognosis.