Primary intraocular lymphoma

Abstract

Primary intraocular lymphoma (PIOL) is a neoplasm, most frequently of B-cell and rarely T-cell origin arising from or initially presenting in the eye. PIOL of the B-cell type is a non-Hodgkin’s lymphoma of the diffuse large B-cell type that belongs within the category of primary central nervous system lymphoma (PCNSL). The lymphoma always is located in the subretinal space, retina, vitreous, and/or optic nerve. Since the retina, retinal pigment epithelium (RPE), and optic nerve are derived from the same neuroectoderm as the CNS during embryogenesis, and both the eye and CNS are immune-privileged organs, PIOL is often considered to be a subtype of PCNSL. Although a subtype of PCNSL, PIOL may arise de novo in the neuroretina rather than secondary involvement from a PCNSL.