Orbital and ocular adnexal lymphoma

Lymphomas result from neoplastic transformation of cells that reside predominantly within lymphoid tissues. Ocular adnexal lymphoma (OAL) refers to lymphoma that involves the conjunctiva, lacrimal gland, eyelid, or orbit. Most OALs are low-grade B-cell non-Hodgkin lymphoma, and approximately half of OALs are of the mucosa-associated lymphoid tissue (MALT) type [1–3]. Orbital lymphoma is the most common primary orbital malignancy in adults and accounts for approximately 11 % of all orbital masses and 34 % of orbital malignancies [2]. The majority of OALs are non-Hodgkin B-cell lymphoma and are seen primarily in adults in the sixth to eighth decade of life. OAL accounts for 8 % of all extranodal lymphomas and only 1–2 % of all non-Hodgkin lymphomas [2–7]. While OAL is often viewed as a localized disease process, studies have demonstrated that up to 36 % of patients with primary OAL have systemic involvement at the time of diagnosis, depending on the elements of their staging work-up [8–10]. This chapter discusses the classification, staging, treatment options, and outcomes for OAL.