Ocular adnexal lymphomas: a review

ABSTRACT

Introduction: Lymphoma is the commonest malignancy of the ocular adnexa. Its relatively low incidence and significant heterogeneity pose unique clinical, diagnostic and therapeutic challenges, requiring a multidisciplinary approach to its management. We present an overview of the literature regarding the epidemiological and clinico-pathological features of ocular adnexal lymphoma (OAL), current staging methods and therapeutic approaches.

Areas covered: We searched MEDLINE for publications on OALs from 1990 to the present day and conducted a manual search of the reference sections of relevant articles and book chapters. Radiotherapy and chemo-immunotherapy are widely used current treatment options in the most common subtypes of OAL and generally offer excellent long-term outcomes. We discuss the emerging role of anti-microbial therapy in marginal zone lymphoma associated with Chlamydia psittaci, the prevalence of which shows significant geographical variation. Several novel and targeted therapeutic agents have emerged in the setting of relapsed and refractory B-cell non-Hodgkin lymphomas, and are likely to become future treatment options in OALs.

Expert commentary: The recognition of new pathways and prognostic molecular markers in lymphomas is also expected to result in better prognostication, novel therapeutic targets and personalised therapies in OAL. It is important that multi-center collaborative approaches are pursued for continued progress in the field.

KEYWORDS:

• Ocular adnexal lymphoma
• chlamydia psittaci
• non hodgkin lymphoma
• MALT lymphoma
• orbital lymphoma
• extranodal marginal zone lymphoma

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Additional information

Funding

No funding to declare.