Primary Central Nervous System and Retinal Lymphoma

Abstract

Primary lymphoma of the central nervous system (CNS) is considered a variant of extra-nodal non-Hodgkin’s lymphoma (NHL), a high-grade B-cell malignancy associated with a median survival ranging from one to 8 years depending on factors such as age and Karnofsky performance status [1]. Primary CNS lymphoma (PCNSL) originates in the brain parenchyma, spinal cord, leptomeninges, and eyes [2]. Formerly used descriptors such as “reticulum cell sarcoma” and “microgliomatosis” are no longer preferred as both misleadingly imply that the lymphoma arises from transformed reticulum or microglial cells. Primary intraocular lymphoma (PCNSL-O) is a variant of PCNSL with predominantly ophthalmic involvement. As vitreoretinal manifestations are the dominant feature, the term primary vitreoretinal lymphoma (PVRL) is commonly used. In contrast, other forms of ocular lymphoma typically affect the adnexal structures or uveal tract. The distinction is important as uveal and ocular adnexal lymphoma are usually low-grade, indolent, B-cell lymphomas that behave similarly to extra-nodal marginal zone lymphoma(EMZL) found elsewhere in the body [3].