Ocular adnexal non-Hodgkin's lymphoma: a review of epidemiology and risk factors.

Ocular adnexal non-Hodgkin's lymphoma (NHL), the most common form of ophthalmic NHL, has a unique incidence pattern showing a steady and rapid increase in the past few decades, nearly equal rates among both genders, and predominance among Asians/Pacific Islanders. No major cause for ocular adnexal NHL has been identified, although infectious agents, immune disorders and genetic/epigenetic factors have all been implicated in its etiology. Identifying putative risk factors and biologic mechanisms leading to carcinogenesis in ocular adnexal NHL may enable implementation of effective preventive and/or therapeutic approaches for this malignancy. This article summarizes current knowledge on epidemiology of ocular adnexal NHL and the role of various potential risk factors in its etiology.