Primary vitreoretinal lymphoma (PVRL) is a rare subtype of primary central nervous system lymphoma (PCNSL). Most cases are classified as high-grade, large B-cell, non-Hodgkin lymphoma, and they are associated with poor prognosis. Its clinical presentation can masquerade as intermediate/posterior uveitis. Characteristic features include the presence of creamy subretinal lesions and/or vitritis, the lack of macular edema, and a paucity of leakage on fluorescein angiography.
Clinical suspicion of PVRL is raised based on the presence of characteristic features and the exclusion of common causes of intermediate/posterior uveitis. The standard diagnostic criterion is vitreous cytology on samples from pars plana vitrectomy (PPV). The scarcity and fragility of the malignant cells render the diagnostic procedure highly operator and observer dependent. A 20-year review of cases at our institution indicates clinical diagnosis alone has sensitivity, specificity, and positive predictive values of 77%, 73%, and 13%, respectively.1 Cytological sensitivity was 87%, but has been reported to be as low as 38% to 81% in other reports.1
Pakdel A, Mammo Z, Hollands H, Forooghian F. Regression of Subretinal Lymphoma After Diagnostic Vitrectomy. JAMA Ophthalmol. Published online April 06, 2017. doi:10.1001/jamaophthalmol.2017.0464