Purpose of review: The goal of this review is to update ophthalmologists and orbital and adnexal specialists with the emerging role of targeted monoclonal antibody therapy and radioimmunotherapy for orbital and adnexal non-Hodgkin's lymphoma and other lymphoproliferative disorders of the orbit.
Recent findings: Rituximab as monotherapy for indolent lymphoma of the orbit and conjunctiva may be associated with good initial response, but the risk of distant relapse is about 50%. Rituximab may be used in combination with conventional chemotherapy, and such combination therapy may be associated with a higher durable response rate and improved progression-free survival compared with chemotherapy alone. Radioimmunotherapy using targeted monoclonal antibodies with radioactive conjugates is also associated with a more durable response rate than monotherapy with rituximab alone and may be an alternative for treatment of ocular adnexal lymphoma. Radioimmunoconjugates are associated with transient pancytopenia during the first 3 months after treatment. Rituximab monotherapy may be considered as an alternative to systemic steroids for treatment of orbital benign lymphoid hyperplasia.
Summary: Targeted monoclonal antibody therapy is an intriguing new modality for treatment of orbital, eyelid, or conjunctival lymphoma and can be used in combination with radioimmunotherapy or standard chemotherapy for a more durable response.