Review article Extranodal marginal zone lymphoma of the ocular adnexa

Lymphomas of the ocular adnexa are a heterogeneous group of malignancies, composing approximately 1 % to 2 % of non-Hodgkin lymphomas (NHLs) and 8% of extranodal lymphomas. The most com-mon subtype, accounting for up to 80 % of cases of primary ocular adnexal lym-phoma, is marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT) type. In the recent past, there have been significant advances in our understanding of the clinical characteris-tics, morphology and phenotype, etiol-ogy, pathogenesis, diagnosis, natural his-tory, treatment approaches, outcome, and prognostic factors of this disease entity. Novel immunologic and molecular tech-niques have aided in the distinction be-tween MALT lymphoma and other lympho-proliferative disorders and led to the identification of tissue markers of prog-nostic significance. Modern imaging mo-dalities provide invaluable tools for accu-rate staging and treatment planning. Besides radiotherapy and chemotherapy, a variety of new treatment options have emerged in the management of patients with ocular adnexal MALT lymphoma, es-pecially monoclonal antibody therapy and antibiotic therapy against Chlamydia psittaci, which has been associated with the pathogenesis of ocular adnexal lym-phomas in some parts of the world. In this review, we present a state-of-the-art sum-mary of ocular adnexal MALT lympho-mas. (Blood. 2009;114:501-510