Recent progress in the diagnosis and treatment of primary vitreoretinal lymphoma.

Primary vitreoretinal lymphoma (PVRL) was previously termed primary intraocular lymphoma. PVRL is a potentially fatal intraocular malignancy, and 65-90% of PVRL cases eventually involve the central nervous system (CNS). The incidence of PVRL has been rising in both immunocompromised and immuno-competent populations worldwide. PVRL frequently masquerades as chronic uveitis. Advanced auxiliary examinations, 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 interleukin-10 analysis have been demonstrated as reliable in diagnosing PVRL. Despite early initiation of treatment, mortality is high with PVRL associated with CNS involvement and relapses are common. The use of systemic chemotherapy has not been proven to prevent CNS involvement; however, local therapies including intravitreal injections of methotrexate and/or rituximab and low-dose radiotherapy to the eye, has shown to be extremely effective in controlling intraocular lymphoma with encouraging results.