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BMC Ophthalmology
Published in: BMC Ophthalmology 1/2015

Open Access 01-12-2015 | Case report

Primary extranodal marginal zone B-cell lymphoma with diffuse uveal involvement and focal infiltration of the trabecular meshwork: a case report and review of literature

Authors: Xinxiao Gao, Bin Li, Qisheng You, Xiaoyan Peng

Published in: BMC Ophthalmology | Issue 1/2015

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Abstract

Background

Primary extranodal marginal zone lymphoma (EMZL) of the uvea is a rare condition and diagnosis may be challenging. We aim to report the clinical, histopathologic and immunohistochemical findings in a case of primary EMZL with diffuse uveal involvement and focal infiltration of the trabecular meshwork.

Case presentation

A 38-year-old male presented with 2-year progressive vision loss in the right eye. Fundus examination showed choroidal thickening with diffuse retinal pigment epithelium (RPE) changes and inferior exudative retinal detachment. Ultrasonography revealed low-reflective masses with diffuse thickening of the choroid involving the optic nerve and orbit. Despite treatment with steroids, his symptoms progressed over time. One year later, visual acuity of the right eye markedly decreased to no light perception and enucleation was performed. Histopathological findings revealed infiltrates of malignant cells in the choroid, iris, ciliary body and trabecular meshwork. Immunohistochemistry confirmed the diagnosis of primary uveal EMZL.

Conclusions

This is the first case reporting primary EMZL diffusely involving the uvea with focal infiltration of the trabecular meshwork.
Literature
1.
Freeman C, Freeman LN, Berg JW, Cutler SJ. Occurrence and prognosis of extranodal lymphomas. Cancer. 1972;29:252–60.CrossRefPubMed
2.
Coupland SE, Damato B. Lymphomas involving the eye and the ocular adnexa. Curr Opin Ophthalmol. 2006;17:523–31.PubMed
3.
White WA, Ferry JA, Harris NL, Grove AS. Ocular adnexal lymphoma: a clinicopathologic study with identification of lymphomas of mucosa-associated lymphoid tissue type. Ophthalmology. 1995;102:1994–2006.CrossRefPubMed
4.
Whitcup SM, de Smet MD, Rubin BI, Palestine AG, Martin DF, Burnier Jr M, et al. Intraocular lymphoma: clinical and histopathologic diagnosis. Ophthalmology. 1993;100:1399–406.CrossRefPubMed
5.
Velez G, de Smet MD, Whitcup SM, Robinson M, Nussenblatt RB, Chan CC. Iris involvement in primary intraocular lymphoma: report of two cases and review of the literature. Surv Ophthalmol. 2000;44:518–26.CrossRefPubMed
6.
Yamada K, Hirata A, Kimura A, Tanihara H. A case of primary B-cell type non-Hodgkin lymphoma originating in the iris. Am J Ophthalmol. 2003;136:380–2.CrossRefPubMed
7.
Coupland SE, Damato B. Understanding intraocular lymphomas. Clin Experiment Ophthalmol. 2008;36:564–78.CrossRefPubMed
8.
Triebenstein O. Ein Beitrag zur Frage der aleukamischen Augenveranderungen. Kin Monatsbl Augenheilkd. 1920;64:825–36.
9.
Ryan SJ, Zimmerman LE, King FM. Reactive lymphoid hyperplasia. An unusual form of intraocular pseudotumor. Trans Am Acad Ophthalmol Otolaryngol. 1972;76:652–71.PubMed
10.
Cockerham GC, Hidayat AA, Bijwaard KE, Sheng ZM. Re-evaluation of ‘reactive lymphoid hyperplasia of the uvea’: an immunohistochemical and molecular analysis of 10 cases. Ophthalmology. 2000;107:151–8.CrossRefPubMed
11.
Harris NL, Jaffe ES, Stein H, Banks PM, Chan JK, Cleary ML, et al. A revised European–American classification of lymphoid neoplasms: a proposal from the International Lymphoma Study Group. Blood. 1994;84:1361–92.PubMed
12.
Coupland SE, Foss HD, Hidayat AA, Cockerham GC, Hummel M, Stein H. Extranodal marginal zone B cell lymphomas of the uvea: an analysis of 13 cases. J Pathol. 2002;197:333–40.CrossRefPubMed
13.
Coupland SE, Joussen A, Anastassiou G, Stein H. Diagnosis of a primary uveal extranodal marginal zone B-cell lymphoma by chorioretinal biopsy: case report. Graefes Arch Clin Exp Ophthalmol. 2005;243:482–6.CrossRefPubMed
14.
Fuller ML, Sweetenham J, Schoenfield L, Singh AD. Uveal lymphoma: a variant of ocular adnexal lymphoma. Leuk Lymphoma. 2008;49:2393–7.CrossRefPubMed
15.
García-Alvarez C, Saornil MA, Blanco G, Méndez MC, López-Lara F. Extranodal B-cell uveal lymphoma with extraocular involvement. Can J Ophthalmol. 2009;44:213–4.CrossRefPubMed
16.
Rasic’ DM, Stankovic’ Z, Terzic’ T, Kovacevic’ D, Koturovic’ Z, Markovic’ V. Primary extranodal marginal zone lymphoma of the uvea associated with massive diffuse epibulbar extension and focal infiltration of the optic nerve and meninges, clinically presented as uveitis masquerade syndrome: a case report. Med Oncol. 2010;27:1010–6.CrossRef
17.
Tagami M, Nagai T, Sekimukai D, Hara R, Azumi A. Uveal extranodal marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue type with concomitant extraocular lesions in a Japanese man. Jpn J Ophthalmol. 2011;55:585–7.CrossRefPubMed
18.
Baryla J, Allen LH, Kwan K, Ong M, Sheidow T. Choroidal lymphoma with orbital and optic nerve extension: case and review of literature. Can J Ophthalmol. 2012;47:79–81.CrossRefPubMed
19.
Loriaut P, Charlotte F, Bodaghi B, Decaudin D, Leblond V, Fardeau C, et al. Choroidal and adnexal extranodal marginal zone B-cell lymphoma: presentation, imaging findings, and therapeutic management in a series of nine cases. Eye (Lond). 2013;27:828–35.CrossRef
20.
21.
Mashayekhi A, Shields CL, Shields JA. Iris involvement by lymphoma: a review of 13 cases. Clin Experiment Ophthalmol. 2013;41:19–26.CrossRefPubMed
22.
Aronow ME, Portell CA, Sweetenham JW, Singh AD. Uveal lymphoma: clinical features, diagnostic studies, treatment selection, and outcomes. Ophthalmology. 2014;121:334–41.CrossRefPubMed
23.
Mashayekhi A, Shukla SY, Shields JA, Shields CL. Choroidal lymphoma: clinical features and association with systemic lymphoma. Ophthalmology. 2014;121:342–51.CrossRefPubMed
24.
Sagoo MS, Mehta H, Swampillai AJ, Cohen VM, Amin SZ, Plowman PN, et al. Primary intraocular lymphoma. Surv Ophthalmol. 2014;59:503–16.CrossRefPubMed
25.
Brown SM, Jampol LM, Cantrill HL. Intraocular lymphoma presenting as retinal vasculitis. Surv Ophthalmol. 1994;39:133–40.CrossRefPubMed
26.
Gill MK, Jampol LM. Variations in the presentation of primary intraocular lymphoma: case reports and a review. Surv Ophthalmol. 2001;45:463–71.CrossRefPubMed
27.
Rothova A, Ooijman F, Kerkhoff F, Van Der Lelij A, Lokhorst HM. Uveitis masquerade syndromes. Ophthalmology. 2001;108:386–99.CrossRefPubMed
28.
29.
Coutinho AB, Muccioli C, Martins MC, Belfort Jr R, Sant’ Anna AE, Burnier Jr MN. Extranodal B-cell lymphoma of the uvea: a case report. Can J Ophthalmol. 2005;40:623–6.CrossRefPubMed
30.
Gündüz K, Shields JA, Shields CL, Eagle Jr RC, Diniz W, Mercado G, et al. Transscleral choroidal biopsy in the diagnosis of choroidal lymphoma. Surv Ophthalmol. 1999;43:551–5.CrossRefPubMed
31.
Qualman SJ, Mendelsohn G, Mann RB, Green WR. Intraocular lymphomas: natural history based on a clinicopathologic study of eight cases and review of the literature. Cancer. 1983;52:878–86.CrossRefPubMed
32.
Paik JS, Cho WK, Lee SE, Choi BO, Jung SE, Park GS, et al. Ophthalmologic outcomes after chemotherapy and/or radiotherapy in non-conjunctival ocular adnexal MALT lymphoma. Ann Hematol. 2012;91:1393–401.CrossRefPubMed
33.
Metadata
Title
Primary extranodal marginal zone B-cell lymphoma with diffuse uveal involvement and focal infiltration of the trabecular meshwork: a case report and review of literature
Authors
Xinxiao Gao
Bin Li
Qisheng You
Xiaoyan Peng
Publication date
01-12-2015
Publisher
BioMed Central
Published in
BMC Ophthalmology / Issue 1/2015
Electronic ISSN: 1471-2415
DOI
https://doi.org/10.1186/s12886-015-0038-7

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