Ocular adnexal lymphoid tumors: progress in need of clarification.
OBJECTIVE
To 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).


METHODS
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.
Jakobiec
Frederick A
FA
Harvard Medical School, Massachusetts Eye and Ear Infirmary, David G. Cogan Ophthalmic Pathology Lab, 243 Charles Street, Boston, MA 02114, USA. Fred_Jakobiec@meei.harvard.edu
D003230:Conjunctival Neoplasms / Q000473:pathology*; D005134:Eye Neoplasms / Q000473:pathology*; D005142:Eyelid Neoplasms / Q000473:pathology*; D006801:Humans; D016130:Immunophenotyping; D007766:Lacrimal Apparatus Diseases / Q000473:pathology*; D008223:Lymphoma / Q000473:pathology*; D009801:Oculomotor Muscles / Q000473:pathology*