A Reappraisal of the Diagnostic and Therapeutic Management of Uncommon Histologies of Primary Ocular Adnexal Lymphoma

Abstract

Lymphoma is the most common malignancy arising in the ocular adnexa, which includes conjunctiva, lachrymal gland, lachrymal sac, eyelids, orbit soft tissue, and extraocular muscles. Ocular adnexal lymphoma (OAL) accounts for 1%-2% of non-Hodgkin lymphoma and 5%-15% of extranodal lymphoma. Histology, stage, and primary localizations are the most important variables influencing the natural history and therapeutic outcome of these malignancies. Among the various lymphoma variants that could arise in the ocular adnexa, marginal zone B-cell lymphoma (OA-MZL) is the most common one. Other types of lymphoma arise much more rarely in these anatomical sites; follicular lymphoma is the second most frequent histology, followed by diffuse large B-cell lymphoma and mantle cell lymphoma. Additional lymphoma entities, like T-cell/natural killer cell lymphomas and Burkitt lymphoma, only occasionally involve orbital structures. Because they are so rare, related literature mostly consists of anecdotal cases included within series focused on OA-MZL and sporadic case reports. This bias hampers a global approach to clinical and molecular properties of these types of lymphoma, with a low level of evidence supporting therapeutic options. This review covers the prevalence, clinical presentation, behavior, and histological and molecular features of uncommon forms of primary OAL and provides practical recommendations for therapeutic management. The Oncologist 2013;18: 876 -884 Implications for Practice: The vast majority of literature's data on ocular adnexa lymphomas focus on the most frequent herein encountered histotype, i.e., marginal zone B-cell lymphoma. However, when a physician encounters less common histologies in this anatomical site, difficulties in their recognition and management may arise, because these tumors are usually reported as minor groups within large series describing marginal zone B-cell lymphomas or under case report format. The present review aims to offer a comprehensive, easily accessible review on these uncommon histologies in order to collect and present available pathologic, clinical, and therapeutic data other than providing own authors' experience in individual considered entities.

Burkitt Lymphoma Leading to Isolated Conjunctival Relapse.

“…When eye involvement is present, lymphomas cause proptosis and swelling due to the pressing effect of the mass, and also minimal pain and inflammation; salmon colored areas in the conjunctiva, ptosis, exophthalmus, loss of vision, and epiphora may be more widely seen. 1,2 In the present study, we have discussed our patient with Burkitt lymphoma (BL) occurring with isolated conjunctival relapse, and reported this uncommon case. The patient did not undergo radiotherapy, and was accepted as cured in January 2012.…”

“…However, radiotheraphy-related xerophthalmia, corneal ulceration, cataract formation,and vasculopathy of the retina and the optic nerve may occur. 1,6 We didn't need to give radiotherapy to our patient because of complete complete remission after chemotherapy.…”

“…The T-cell/natural killer-cell lymphoma and BL cause rare orbital involvement. 1,2 The BLs that lead to orbital involvement occur sporadically in children, and they are therefore reported as case reports. 6 Our patient had abdominal involvement at diagnosis, and there was no conjunctival involvement.…”

“…Conjunctival lymphomas generally has a good prognosis; however, these cases have to be followed-up for several years because of the risk of relapse. 1 Orbital lymphomalar is associated with an increased risk of CNS dissemination either by hematogenous route or by direct infiltration through the skull bones. Cancer Network guidelines recommend prophylaxis of intrathecal methotrexate and/ or cytarabine given during primary chemotherapy.…”

“…Cancer Network guidelines recommend prophylaxis of intrathecal methotrexate and/ or cytarabine given during primary chemotherapy. 1,6 We have given to our patient CNS prophylaxis with intrathecal methotrexate during primary chemotherapy.…”