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Published in: Journal of Medical Case Reports 1/2022

Open Access 01-12-2022 | Lymphoma | Case report

Management of primary choroidal lymphoma presenting as extensive serous retinal detachment and salmon patch of bulbar conjunctiva: a case report

Authors: Zahra Mahdizad, Elias Khalili Pour, Mohammadreza Mehrabi Bahar, Amirreza Esfandiari, Babak Masoomian, Hamid Riazi-Esfahani, Ahmad Mirshahi, Fariba Ghassemi

Published in: Journal of Medical Case Reports | Issue 1/2022

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Abstract

Background

We describe the outcome of ultra-low-dose radiotherapy plus intravitreal methotrexate and rituximab injections for a patient with primary choroidal lymphoma who presented with nodular conjunctival salmon patches and extensive serous retinal detachment.

Case presentation

A 34-year-old Iranian man presented with a nodular patch of bulbar conjunctiva in the right eye, and 1+ vitritis. A nearly complete shallow serous retinal detachment, retinal folds, and multifocal yellow choroidal infiltrates were seen during a fundus examination of the right eye. Enhanced depth imaging optical coherence tomography revealed macular retinal folds and an uneven, undulating, “seasick” appearance of the choroidal surface with choriocapillaris compression, intraretinal and subretinal fluid, and clusters of optically dense material at the outer retinal level. An incisional biopsy of the conjunctival lesion confirmed the diagnosis of primary choroidal lymphoma with epibulbar involvement. The patient was treated with ultra-low-dose “boom-boom” radiation (4 Gy delivered in two fractions over two consecutive days) as well as intravitreal methotrexate and rituximab injections. After a year, the lesions had completely disappeared, with no adverse effects or recurrence.

Conclusion

Ultra-low-dose (boom-boom) radiotherapy combined with intravitreal chemotherapy and/or immunotherapy may be an effective treatment for primary choroidal lymphoma with anterior epibulbar extension and diffuse subretinal fluid with favorable response and minimal side effects.
Literature
1.
go back to reference Coupland SE, Foss H-D, 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 Coupland SE, Foss H-D, 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
2.
go back to reference Mashayekhi A, Shukla SY, Shields JA, Shields CL. Choroidal lymphoma: clinical features and association with systemic lymphoma. Ophthalmology. 2014;121:342–51.CrossRefPubMed Mashayekhi A, Shukla SY, Shields JA, Shields CL. Choroidal lymphoma: clinical features and association with systemic lymphoma. Ophthalmology. 2014;121:342–51.CrossRefPubMed
3.
go back to reference Yang X, Dalvin LA, Lim L-AS, Mashayekhi A, Shields JA, Shields CL. Ultra-low-dose (boom-boom) radiotherapy for choroidal lymphoma in three consecutive cases. Eur J Ophthalmol. 2021;31:NP91–6.CrossRefPubMed Yang X, Dalvin LA, Lim L-AS, Mashayekhi A, Shields JA, Shields CL. Ultra-low-dose (boom-boom) radiotherapy for choroidal lymphoma in three consecutive cases. Eur J Ophthalmol. 2021;31:NP91–6.CrossRefPubMed
4.
go back to reference Fasola CE, Jones JC, Huang DD, Le Q-T, Hoppe RT, Donaldson SS. Low-dose radiation therapy (2 Gy × 2) in the treatment of orbital lymphoma. Int J Radiat Oncol Biol Phys. 2013;86:930–5.CrossRefPubMedPubMedCentral Fasola CE, Jones JC, Huang DD, Le Q-T, Hoppe RT, Donaldson SS. Low-dose radiation therapy (2 Gy × 2) in the treatment of orbital lymphoma. Int J Radiat Oncol Biol Phys. 2013;86:930–5.CrossRefPubMedPubMedCentral
5.
go back to reference Dirani A, Allaire G, Callejo S, Belair ML, Biernacki K, Corriveau C, et al. Choroidal extranodal marginal zone lymphoma diagnosed by full-thickness retinochoroidal biopsy: case report and review of the literature. Int Med Case Rep J. 2017;10:153–8.CrossRefPubMedPubMedCentral Dirani A, Allaire G, Callejo S, Belair ML, Biernacki K, Corriveau C, et al. Choroidal extranodal marginal zone lymphoma diagnosed by full-thickness retinochoroidal biopsy: case report and review of the literature. Int Med Case Rep J. 2017;10:153–8.CrossRefPubMedPubMedCentral
6.
go back to reference Aronow ME, Portell CA, Sweetenham JW, Singh AD. Uveal lymphoma: clinical features, diagnostic studies, treatment selection, and outcomes. Ophthalmology. 2014;121:334–41.CrossRefPubMed Aronow ME, Portell CA, Sweetenham JW, Singh AD. Uveal lymphoma: clinical features, diagnostic studies, treatment selection, and outcomes. Ophthalmology. 2014;121:334–41.CrossRefPubMed
7.
go back to reference Rajagopal R, Harbour JW. Diagnostic testing and treatment choices in primary vitreoretinal lymphoma. Retina. 2011;31:435–40.CrossRefPubMed Rajagopal R, Harbour JW. Diagnostic testing and treatment choices in primary vitreoretinal lymphoma. Retina. 2011;31:435–40.CrossRefPubMed
8.
go back to reference Fend F, Ferreri AJM, Coupland SE. How we diagnose and treat vitreoretinal lymphoma. Br J Haematol. 2016;173:680–92.CrossRefPubMed Fend F, Ferreri AJM, Coupland SE. How we diagnose and treat vitreoretinal lymphoma. Br J Haematol. 2016;173:680–92.CrossRefPubMed
9.
go back to reference Araujo I, Coupland SE. Primary vitreoretinal lymphoma—a review. Asia-Pacific J Ophthalmol (Philadelphia, Pa). 2017;6:283–9. Araujo I, Coupland SE. Primary vitreoretinal lymphoma—a review. Asia-Pacific J Ophthalmol (Philadelphia, Pa). 2017;6:283–9.
10.
go back to reference Arias JD, Kumar N, Fulco EAM, Spaide R, Yannuzzi L, Shields JA, et al. The seasick choroid: a finding on enhanced depth imaging spectral-domain optical coherence tomography of choroidal lymphoma. Retin Cases Brief Rep. 2013;7:19–22.CrossRefPubMed Arias JD, Kumar N, Fulco EAM, Spaide R, Yannuzzi L, Shields JA, et al. The seasick choroid: a finding on enhanced depth imaging spectral-domain optical coherence tomography of choroidal lymphoma. Retin Cases Brief Rep. 2013;7:19–22.CrossRefPubMed
11.
go back to reference Shields CL, Arepalli S, Pellegrini M, Mashayekhi A, Shields JA. Choroidal lymphoma shows calm, rippled, or undulating topography on enhanced depth imaging optical coherence tomography in 14 eyes. Retina. 2014;34:1347–53.CrossRefPubMed Shields CL, Arepalli S, Pellegrini M, Mashayekhi A, Shields JA. Choroidal lymphoma shows calm, rippled, or undulating topography on enhanced depth imaging optical coherence tomography in 14 eyes. Retina. 2014;34:1347–53.CrossRefPubMed
12.
go back to reference Grossniklaus HE, Martin DF, Avery R, Shields JA, Shields CL, Kuo IC, et al. Uveal lymphoid infiltration. Report of four cases and clinicopathologic review. Ophthalmology. 1998;105:1265–73.CrossRefPubMed Grossniklaus HE, Martin DF, Avery R, Shields JA, Shields CL, Kuo IC, et al. Uveal lymphoid infiltration. Report of four cases and clinicopathologic review. Ophthalmology. 1998;105:1265–73.CrossRefPubMed
13.
go back to reference Ciulla TA, Bains RA, Jakobiec FA, Topping TM, Gragoudas ES. Uveal lymphoid neoplasia: a clinical–pathologic correlation and review of the early form. Surv Ophthalmol. 1997;41:467–76.CrossRefPubMed Ciulla TA, Bains RA, Jakobiec FA, Topping TM, Gragoudas ES. Uveal lymphoid neoplasia: a clinical–pathologic correlation and review of the early form. Surv Ophthalmol. 1997;41:467–76.CrossRefPubMed
14.
go back to reference Neudorfer M, Kessler A, Anteby I, Goldenberg D, Barak A. Co-existence of intraocular and orbital lymphoma. Acta Ophthalmol Scand. 2004;82:754–61.CrossRefPubMed Neudorfer M, Kessler A, Anteby I, Goldenberg D, Barak A. Co-existence of intraocular and orbital lymphoma. Acta Ophthalmol Scand. 2004;82:754–61.CrossRefPubMed
15.
go back to reference 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 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
16.
go back to reference Augsburger JJ, Greatrex KV. Intraocular lymphoma: clinical presentations, differential diagnosis and treatment. Trans Pa Acad Ophthalmol Otolaryngol. 1989;41:796–808.PubMed Augsburger JJ, Greatrex KV. Intraocular lymphoma: clinical presentations, differential diagnosis and treatment. Trans Pa Acad Ophthalmol Otolaryngol. 1989;41:796–808.PubMed
17.
go back to reference Mashayekhi A, Hasanreisoglu M, Shields CL, Shields JA. External beam radiation for choroidal lymphoma: efficacy and complications. Retina. 2016;36:2006–12.CrossRefPubMed Mashayekhi A, Hasanreisoglu M, Shields CL, Shields JA. External beam radiation for choroidal lymphoma: efficacy and complications. Retina. 2016;36:2006–12.CrossRefPubMed
18.
go back to reference König L, Stade R, Rieber J, Debus J, Herfarth K. Radiotherapy of indolent orbital lymphomas: two radiation concepts. Strahlenther Onkol. 2016;192:414–21.CrossRefPubMed König L, Stade R, Rieber J, Debus J, Herfarth K. Radiotherapy of indolent orbital lymphomas: two radiation concepts. Strahlenther Onkol. 2016;192:414–21.CrossRefPubMed
19.
go back to reference Shields JA, Dockery PW, Shields CL. Boom-boom radiotherapy for choroidal lymphoma—two days and done. Ophthalmol Retin. 2021;5:15.CrossRef Shields JA, Dockery PW, Shields CL. Boom-boom radiotherapy for choroidal lymphoma—two days and done. Ophthalmol Retin. 2021;5:15.CrossRef
Metadata
Title
Management of primary choroidal lymphoma presenting as extensive serous retinal detachment and salmon patch of bulbar conjunctiva: a case report
Authors
Zahra Mahdizad
Elias Khalili Pour
Mohammadreza Mehrabi Bahar
Amirreza Esfandiari
Babak Masoomian
Hamid Riazi-Esfahani
Ahmad Mirshahi
Fariba Ghassemi
Publication date
01-12-2022
Publisher
BioMed Central
Published in
Journal of Medical Case Reports / Issue 1/2022
Electronic ISSN: 1752-1947
DOI
https://doi.org/10.1186/s13256-022-03373-z

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