Primary vitreoretinal
lymphoma (PVRL), previously called primary
intraocular lymphoma (PIOL), is a rare and fatal ocular
malignancy. PVRL is a subset of primary central nervous system
lymphoma (PCNSL), mostly a
diffuse large B-cell lymphoma. The diagnosis of PVRL is often challenging as it often masquerades as chronic
uveitis. PVRL requires invasive procedures for tissue diagnosis. Cytology/pathology, molecular pathology (immuno-
globulin or T-cell receptor gene rearrangement), immunohistochemistry, biophysical technology (flow cytometry), and
cytokine analysis (interleukine-10) are often required. The
therapies that have been successful in systemic
lymphomas have not been reliably effective in PVRL and PCNSL. Current management of PVRL involves aggressive
chemotherapy (
methotrexate and
rituximab) and
radiation therapy. PVRL normally responds well to initial treatment; however, relapse rate and CNS involvement are high, resulting in poor prognosis and limited survival. A professional team of medical experts in ophthalmology, oncology (particularly neuro-oncology), and pathology is essential for optimizing patient management.