Primary vitreoretinal lymphoma: prevalence, impact, and management challenges.

Primary vitreoretinal lymphoma (PVRL) is a rare and potentially fatal intraocular malignancy. More than half of PVRL cases eventually involve the central nervous system (CNS). PVRL frequently masquerades as chronic uveitis. Advanced imaging tests, such as optical coherence tomography and fundus autofluorescence, have been applied in the diagnosis of PVRL. Histology and immunohistochemistry, in combination with molecular tests and IL-10 analysis, have been demonstrated as reliable in diagnosing PVRL. Mortality is high in patients with PVRL associated with CNS involvement, and relapses are common. The use of systemic chemotherapy in addition to the local therapies has proved to extend the mean survival time of these patients. Local therapies, including intravitreal injections of methotrexate and/or rituximab and low-dose radiotherapy to the eye, have been shown to be extremely effective in controlling intraocular lymphoma.