Primary central nervous system lymphoma (PCNSL) may manifest initially in the eye (termed vitreoretinal lymphoma or VRL) or in non-ocular CNS compartments, or in both. The nature of the onset of PCNSL implies two clinical specialists — ophthalmologists and neuro-oncologists — independently may assess the primary presentation of this rare malignancy. Clinically relevant perspectives on expectations of PCNSL manifestation in both ocular and non-ocular CNS compartments would help inform management practices in each specialty, which should impact clinical outcomes. A recent increase in the number of published PCNSL cohort studies provides new opportunity to review the current prevalence rates of ocular involvement, and the timing of this involvement over the course of disease. In PCNSL cohorts defined by non-ocular CNS compartment involvement, with or without ocular involvement (termed “PCNSL ± ocular involvement” cohorts), mean rates of concomitant VRL at diagnosis, or at any time during the course, are 10% and 16%, respectively. Only a few individuals within this cohort group present with exclusive eye disease (<5%), and the rate of secondary ocular involvement is only 5–9%. In PCNSL cohorts defined by the involvement of the ocular compartment, with or without non-ocular CNS involvement (termed “VRL ± non-ocular CNS involvement” cohorts), 58% of persons have a primary ocular diagnosis, which carries a 50% risk of secondary involvement in the CNS beyond the eye. Rates of non-ocular CNS involvement with VRL at diagnosis or over the course of disease are 41% and 69%, respectively.
- primary central nervous system
- vitreoretinal lymphoma
- diffuse large B-cell lymphoma
- systematic review