An update of ocular adnexal lymphomas

The ocular adnexa (OA) include the eyelids, conjunctiva, lacrimal apparatus, and orbital soft tissue. One percent of all lymphomas and approximately 8% of all extranodal lymphomas arise in the OA and the incidence is increasing. The relative frequencies of ocular adnexal lymphoma presentation are orbit, 38%, conjunctiva, 31%, lacrimal apparatus, 18% and eyelid, 13%. The most frequent primary lymphoma types of the ocular adnexa are extranodal marginal zone lymphoma, 63%, follicular lymphoma, 16%, diffuse large B cell lymphoma 10%. The eyelids show the highest proportion of secondary lymphoma involvement, 47% of all eyelid lymphoproliferative lesions, compared with 20% in the other ocular adnexa. The specific aspects of the site, histologic, immunohistochemical, cytogenetic and molecular findings of the most relevant lymphoma types occurring in the various parts of the ocular adnexa will be discussed in relation to clinical parameters and relevance for therapy choice.