Lymphomas of the Ocular Adnexa

Lymphoid tumors are the most common primary orbital tumors. The majority of ocular adnexal lymphomas (OAL) are low grade. In order of frequency, the most common histologic subtypes of lymphoma in the orbit and ocular adnexa are marginal zone lymphoma (“MALT”), follicular lymphoma, diffuse large B-cell lymphoma, and mantle cell lymphoma. The American Joint Committee on Cancer (AJCC) classification for ocular adnexal lymphoma is more detailed in terms of the extent of involvement of the orbital and ocular structures, while the Ann Arbor staging may be more relevant to systemic staging of lymphoma. The role of positron emission tomography (PET)/computed tomography (CT) scans and bone marrow biopsy in staging of ocular adnexal lymphoma are better defined. Treatment options depend on the stage of lymphoma and the histologic subtype of lymphoma. Treatments that may include very low-dose radiation therapy, monoclonal antibody treatment, radioimmunotherapy, and systemic chemotherapy may be considered depending on the stage and histologic subtype of OAL.