Ocular adnexal lymphomas: a review

Ocular adnexal lymphomas comprise 1% to 2% of all non-Hodgkin lymphomas and about 8% of extranodal lymphomas. They are a heterogeneous group of malignancies, the majority of which are primary extranodal lymphoma with most (up to 80%) of the marginal zone of mucosa associated lymphoid tissue type (MALT lymphoma). This review will encompass the incidence, histology, immunophenotyping, recent advances in molecular and cytogenetics, clinical features including outcome, and prognostic factors. The association with Chlamydia psittaci and the very recently recognized occurrence in the context of IgG4-related sclerosing disease will be discussed. Finally, traditional (surgery, radiotherapy, chemotherapy) and newer forms of therapy (immunotherapy and radioimmunotherapy) will be reviewed.