Intraocular Lymphoma

Intraocular lymphoma can be divided into three main forms: primary vitreoretinal lymphoma (PVRL), secondary intraocular lymphoma, and primary uveal lymphoma (PUL). PVRL is thought of as an intraocular manifestation of multicentric primary central nervous system (CNS) lymphoma, and it is associated with a worse ocular and systemic prognosis. Clinically, it manifests with vitreous cells and sb-RPE infiltrates. Definitive diagnosis is achieved with vitreous biopsy and cytology. PUL commonly involves extranodal marginal zone non-Hodgkin lymphoma, which generally has excellent prognosis. Secondary intraocular lymphoma generally involves the eye following disease arising in an extraocular organ. Treatment strategies for PVRL include external beam radiotherapy (EBRT) for bilateral disease with or without intravitreal chemotherapy. For unilateral disease, intravitreal chemotherapy with methotrexate or rituximab is an option. Additional systemic chemotherapy regimens have been suggested to improve overall outcomes in PVRL with CNS involvement. However, overall prognosis remains poor when the CNS is involved. PUL usually responds to low-dose EBRT. For secondary intraocular lymphoma, treatment and prognosis depend on the primary type of lymphoma.