PerspectiveOcular Adnexal Lymphoid Tumors: Progress in Need of Clarification
Section snippets
General Features and Anatomy
Among 1,269 autopsied cases of systemic lymphomas,4 0.24% patients initially sought treatment for an OAL; by the time of death, ocular adnexal involvement had developed in a total of 1.3%. Another series puts ocular adnexal involvement at 5% of non-Hodgkin systemic lymphomas.5 Of all tumors with a propensity for extranodal sites, 8% settle in the ocular adnexa.6
There are two fundamental ways of looking at OALs: from the perspective of putatively predictive features that may be distinctive to
Overview of Ocular Adnexal Lymphoid Tumors or Lymphomas
The following clinicopathologic and immunophenotypic generalizations about OALs continue to be valid, although they include occasional exceptions. While the most common orbital tumors of adults, they are extremely rare in children, in whom a leukemic infiltrate instead should be suspected. They are generally indolent clinically, painless, functionally well tolerated, and mold on imaging studies to adjacent structures like the sclera and tissue planes, usually without bone destruction (in
General Pathologic Background
The single most valuable study covering OALs over the past decade is that by Ferry and associates, which intensively analyzed a staggering 353 cases by means of a plethora of immunophenotypic markers in conjunction with the World Health Organization (WHO) morphologic classification of lymphopoietic and hematopoietic neoplasms.7, 30 It offers a wealth of clinical and anatomic correlations and a panoply of immunologic, genotypic, and cytogenetic findings. Because it was published in the pathology
Histopathologic Findings
Follicular architecture (Figure, Top) to various extents and at different levels of clear definition is seen in overt follicular lymphoma, marginal zone lymphoma, mantle cell lymphoma, and small cell lymphoma or chronic lymphocytic leukemia (which have pseudofollicles or growth centers containing paraimmunoblasts).7, 30 Prominent marginal zones in nonneoplastic lymphoid tissue can be discerned in Waldeyer oropharyngeal tonsillar tissue, Peyer patches, and splenic follicles, but are less
Immunophenotypic Findings
The combination of meticulous architectural and cytologic evaluation with immunophenotypic profiling is a powerful approach because it tremendously reduces intraobserver and interobserver discordances of morphologic interpretations. As a shorthand, marginal zone lymphoma cells are B-antigen positive, cyclin D1 negative, and CD5 negative (also rarely CD5 positive, bcl-6 negative, and bcl-2 positive). The aberrant expression of a T-cell antigen (CD5, CD43) on a cell of B-lymphocytic lineage is
Clinical Correlations
The clinical implications of properly using the diagnosis of extranodal marginal zone lymphoma (EMZL) are apparent. At the time of diagnosis, OALs of EMZL type are the most likely to be restricted locally, and on follow-up the least likely to have spread7, 9, 25 (Ferry J. Unpublished Data from a Clinical Follow-up Paper in Preparation, personal communication, February 2008). Rosado and associates diagnosed 89% of EMZLs among 62 OALs (53% in orbit, 26% in conjunctiva, and 19% in lacrimal gland).9
Conclusions
Until we have the results of such a study, the best advice that can be offered at the moment is to become deeply familiar with the results of Ferry and associates' landmark article and to stage clinically all patients with OALs regardless of their diagnosis.7 Fifteen years ago, Knowles and associates established that there were no incontestable pathologic or immunophenotypic criteria for predicting the full extent of an OAL's spread and that clinical staging transcended tissue studies in this
References (37)
Ocular inflammatory disease: the lymphocyte redivivus
Am J Ophthalmol
(1983)- et al.
Immunologic characterization of ocular adnexal lymphoid neoplasms
Am J Ophthalmol
(1979) - et al.
Homing of lymphocytes to non-lymphoid tissues
Lancet
(1974) - et al.
Ocular adnexal lymphoma: a clinicopathologic study of a large cohort of patients with no evidence for an association with Chlamydia psittaci
Blood
(2006) - et al.
B- and T- lymphocytes in ocular disease
Ophthalmology
(1984) - et al.
Lymphocytic subpopulations in the normal conjunctiva: a monoclonal antibody study
Ophthalmology
(1986) - et al.
The immunoarchitecture of the normal lacrimal gland
Ophthalmology
(1988) - et al.
Survey of 1,264 patients with orbital tumors and simulating lesions
Ophthalmology
(2004) - et al.
Clinicopathologic characteristics of orbital lymphoid hyperplasia
Ophthalmology
(1979) - et al.
Genotypic monoclonality in immunophenotypically polyclonal orbital lymphoid tumors: a model of tumor progression in the lymphoid system
Ophthalmology
(1987)
Ocular adnexal monoclonal lymphoid tumors with a favorable prognosis
Ophthalmology
Mantle cell lymphoma in the ocular adnexal region
Ophthalmology
Ocular adnexal lymphoma: a review of clinicopathologic features and treatment options
Blood
High incidence of auto-immune disease in Japanese patients with ocular adnexal reactive lymphoid hyperplasia
Am J Ophthalmol
Lymphoid hyperplasia and malignant lymphoma occurring in the ocular adnexa (orbit, conjunctiva, eyelid): a prospective multiparametric analysis of 108 cases during 1977–1987
Hum Pathol
Extranodal non-cutaneous lymphoid hyperplasias represent a continuous spectrum of B-cell neoplasia: demonstration by molecular genetic analysis
Blood
Identification of T-lymphocytes in ocular adnexal neoplasms by hybridoma monoclonal antibodies
Am J Ophthalmol
Lymphosarcoma: a review of 1269 cases
Medicine
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See accompanying Article on page 1002.