ABSTRACT
Marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) is the most common lymphoma category arising in these anatomical structures. As for other MALT lymphomas, polymerase chain reaction analysis of immunoglobulin heavy-chain gene rearrangement shows a clonal B-cell population in 55% of Ocular Adnexal MALT Lymphoma (OAML) and somatic hypermutations in two-thirds of these cases. OAML shares several clinicopathological features with other MALT lymphomas. It arises in tissues normally devoid of innate immunore-active cells, often develops on a background of preexisting chronic inflammation, and shows an indolent clinical course. Lymphomas can infiltrate any orbital and ocular adnexal tissue, and the clinical picture depends on the structures involved. Neuroimaging techniques are fundamental for differential diagnosis, staging, and definition of therapeutic response in patients with OAML. Surgical resection is a necessary diagnostic step and, in selected cases, a part of the therapeutic approach to OAML. Only a small proportion of OAML patients in reported series have been treated with chemotherapy alone.
