Diagnosis of a primary uveal extranodal marginal zone B-cell lymphoma by chorioretinal biopsy: case report

Purpose To report the clinical, histopathological and molecular biological findings of a primary extranodal marginal zone B-cell lymphoma (EMZL) of the uvea.Methods Conventional histology, immunohistochemistry and polymerase chain reaction for immunoglobulin heavy chain gene rearrangement (IgH-PCR) and GeneScan analysis were performed on a chorioretinal biopsy.Results Histological examination of the chorioretinal biopsy demonstrated a dense infiltrate of small centrocyte-like cells, plasmacytoid tumour cells and occasional blasts. The tumour cells were positive for CD20, showed monotypical expression for Ig-kappa and IgM, and a growth fraction of 10%. Clonality analysis using IgH-PCR disclosed a monoclonal B-cell population. Following localised irradiation of 35 Gy, a complete remission was achieved.Conclusion This report describes a rare uveal EMZL diagnosed following investigation of a chorioretinal biopsy. Despite its rarity, ophthalmic pathologists should consider the diagnosis of a primary uveal EMZL when reviewing chorioretinal biopsies.