Primary Central Nervous System and Vitreoretinal Lymphoma

Primary central nervous system lymphoma (PCNSL) is typically a B-cell extranodal non-Hodgkin’s lymphoma (NHL). This high-grade malignancy affects the brain parenchyma, spinal cord, and leptomeninges. It is best to consider vitreoretinal lymphoma as a subset of primary lymphoma of the central nervous system that may occur synchronously or as an isolated abnormality (primary CNS lymphoma–ophthalmic [PCNSL-O]), predominantly affecting the subretinal space, retina, and vitreous (Singh AD, Raval VR. Primary central nervous system lymphoma: terminology and outcome measures. In: Ocular and adnexal lymphoma. Springer International Publishing, Cham, 2023. pp 1-5). PCNSL-O is distinct from other forms of ocular lymphoma such as ocular adnexal and uveal lymphoma, which are usually low-grade, indolent, B-cell lymphomas that behave similarly to their systemic counterparts. In this chapter, the presenting signs, clinical features, diagnostic studies, treatment options, and prognosis of PCNSL are discussed.