The eye and lymphoma: New concepts of an old acquaintance

Abstract

AbstractIntraocular lymphoma (IOL) is a life‐threatening condition that results from the proliferation of malignant lymphocytic cells arising from either the central nervous system (CNS) (primary CNS lymphoma) or outside the CNS with secondary dissemination and metastasis to the eye (secondary IOL). Primary intraocular lymphoma (PIOL), also known as primary vitreoretinal lymphoma (PVRL), is a subset of PCNSL where the findings are ocular.PVRL is particularly feared because it can manifest as non‐specific uveitis, leading to a potentially fatal diagnostic delay. Although clinical and ancillary parameters can provide valuable diagnostic clues, the only valid confirmation comes from cytological analysis and molecular studies of clonality based on vitreous and/or (sub)retinal biopsies performed during vitrectomy. Standard three‐port twenty‐five‐gauge transconjunctival vitrectomy is the usual sampling technique; however, it is important to remember that the diagnostic yield of vitreous cells depends on the way samples are harvested, transported and analysed. Even when surgical recommendations are strictly followed and analyses performed by trained pathologists, the diagnostic yield of vitrectomy is not 100%.The objective of this course is to describe the clinical features of PVRL and its characteristics on ancillary exams in both typical and more challenging situations, and present the diagnostic procedures that are or will be available in the near future. Therapeutic management will be discussed later during this course.