Ocular adnexal lymphoid tumors: Progress in need of clarification

PURPOSE: To evaluate recent ophthalmic publications on ocular adnexal lymphomas (OALs) according to his, topathologic and immunophenotypic criteria used in the diagnosis of systemic lymphomas (World Health Organization classification). DESIGN: Summary and critical analysis of recent clinical and pathologic studies. METHODS: Literature review and interpretation. RESULTS: In 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). CONCLUSIONS: Rigorous diagnostic criteria and a proposal for a prospective multicenter study may bring further clarification to the emerging order in this set of tumors.