Ocular and Adnexal Lymphoma by Arun D. Singh

Author:Arun D. Singh
Language: eng
Format: epub
Publisher: Springer Berlin Heidelberg, Berlin, Heidelberg

5.2.4 Ocular Adnexal Lymphoma

OAL encompasses a heterogeneous group of malignancies, the vast majority of which are of the non-Hodgkin’s type and are primarily of B-cell origin. Rarely, other forms such as T-cell (Ferry et al. 2007) and natural killer (NK)-cell lymphomas (Coupland et al. 1999; Mendenhall et al. 2006) can affect the ocular adnexal structures. The majority of OAL belong to one of five subtypes: EMZL, follicular lymphoma, DLBCL, mantle cell lymphoma, and lymphoplasmacytic lymphoma (Shields et al. 2001). Approximately 80 % are of the EMZL type (Freeman et al. 1972). Among affected sites, the frequencies of involvement are conjunctiva 20–33 %, orbit/lacrimal gland 46–74 %, and eyelid 5–20 % (Coupland et al. 2004; Knowles et al. 1990). Disease may be limited to a single, localized tumor, or it may be multifocal. Overlap with ocular adnexal sites is common (10–20 % of cases), and coexisting uveal involvement has been observed (Fuller et al. 2008). OAL can also affect regional, central, and peripheral lymph nodes as well as other distant extranodal sites.

OAL has a slight female predilection (60 % of cases in most series) (Shields et al. 2001). Individuals with OAL may present with a broad range of symptoms depending upon the site and extent of involvement. Common symptoms include a mass within the ocular adnexal structures, exophthalmos, ptosis, ophthalmoplegia, pain, decreased vision, or diplopia. If the lacrimal gland is involved, dry eye symptoms may occur. On clinical examination, OAL has site-specific presentations that affect how the diagnosis is made. In the conjunctiva, lesions typically present as a mass with a “salmon patch” appearance (Fig. 5.4). In the orbit, lacrimal gland, and eyelid, the lymphoma presents as a mass, which, if palpable, is typically firm. Mobility is variable depending upon attachment to other ocular adnexal structures. Diplopia may occur depending upon how rapidly the mass develops. Exophthalmos and decreased retropulsion of the globe may be the only clinical signs. Involvement of the nasolacrimal drainage system can occur. Compression or invasion of the optic nerve can lead to vision loss. Regardless of the clinical findings, these features do not allow distinction of benign from malignant lymphoproliferative disease.

Fig. 5.4Ocular adnexal lymphoma. Typical appearance of subconjunctival infiltrate presenting as “salmon” patch (a) and diffuse orbital extension without compression of the orbital structures (b). Bilateral asymmetric involvement (milder OS)

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