Classification, diagnosis, and management of conjunctival lymphoma

Lymphoma is a malignant lymphoproliferative tumor that can involve the conjunctiva. Approximately 5–15% of all extranodal lymphomas are found in the ocular adnexal region, with approximately 25% of those involving the conjunctiva. Ninety-eight percent of conjunctival lymphomas arise from B-lymphocytes. The most common subtype of conjunctival lymphoma is extranodal marginal zone lymphoma (80%), followed by follicular lymphoma (8%), diffuse large B-cell lymphoma (3%) and mantle cell lymphoma (3%). Natural killer and T cells (NK/T) are rare causes of lymphoma. While most conjunctival lymphomas are localized to the ocular adnexa at the time of presentation, systemic examination and management are of key importance in the long-term care of the patient. This review outlines the classification, etiology, presentation, diagnosis, and management of conjunctival lymphoma. The novel use of high resolution optical coherence tomography, both as a diagnostic tool and as a means for ongoing evaluation during treatment, is illustrated. Treatment options discussed include external beam radiation, chemotherapy, immunotherapy, antibiotic therapy, and combination regimens. Future investigation of the etiology and pathogenesis of conjunctival lymphoma is expected to reveal opportunities for innovative and individualized therapeutic agents. Collaboration between multiple disciplines is key in the advancement of the field.