Ocular Adnexal Lymphoid Tumors: Progress in Need of Clarification

PurposeTo evaluate recent ophthalmic publications on ocular adnexal lymphomas (OALs) according to histopathologic and immunophenotypic criteria used in the diagnosis of systemic lymphomas (World Health Organization classification).DesignSummary and critical analysis of recent clinical and pathologic studies.MethodsLiterature review and interpretation.ResultsIn the largest study of 353 cases of OALs published in the pathology literature, extranodal marginal zone lymphoma (EMZL) constituted 52%, follicular lymphoma 23%, mantle cell lymphoma 5%, and small cell lymphocytic and chronic lymphocytic leukemia 4%—plus a residuum of arcane entities. In smaller series of less intensively studied OALs in the ophthalmic literature, EMZLs had a higher preponderance and also were associated with a favorable prognosis. Many EMZLs seemed to arise primarily in the ocular adnexa (mucosa-associated lymphoid tissue [MALT] lymphoma should be restricted for EMZLs involving epithelial tissues).ConclusionsRigorous diagnostic criteria and a proposal for a prospective multicenter study may bring further clarification to the emerging order in this set of tumors.