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


CONCLUSIONS
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.
Coupland
Sarah E
SE
Department of Pathology, Charité University Hospital, Campus Benjamin Franklin, Hindenburgdamm 30, 12200 Berlin, Germany. sarah.coupland@charite.de
Joussen
Antonia
A
Anastassiou
Gerasimos
G
Stein
Harald
H
D001707:Biopsy, Needle; D002829:Choroid / Q000473:pathology*; D015326:Gene Rearrangement, B-Lymphocyte, Heavy Chain / Q000235:genetics; D005803:Genes, Immunoglobulin / Q000235:genetics; D006801:Humans; D007143:Immunoglobulin Heavy Chains / Q000235:genetics; D016130:Immunophenotyping; D016393:Lymphoma, B-Cell / Q000175:diagnosis* / Q000235:genetics / Q000532:radiotherapy; D008297:Male; D008875:Middle Aged; D016133:Polymerase Chain Reaction; D012160:Retina / Q000473:pathology*; D014604:Uveal Neoplasms / Q000175:diagnosis* / Q000235:genetics / Q000532:radiotherapy; D014821:Vitrectomy
D007143:Immunoglobulin Heavy Chains