The clinical features of primary uveal lymphoma of four cases_Chinese Journal of Ocular Fundus Diseases

Authors：

Zhang Yongpeng ¹ , Li Zhihua ¹ , Yang Wenli ¹ , Tang Xin ¹ , Li Bin ² ,  Peng Xiaoyan ²

1. Beijing Tongren Eye Center, Beijing Key Laboratory of Ophthalmology and Visual Science, Beijing Tongren Hospital, Capital Medical University, Beijing 100730, China;
2. Beijing Institute of Ophthalmology, Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University; Beijing Key Laboratory of Ophthalmology and Visual Science, Beijing 100730, China;

Corresponding author：

Peng Xiaoyan, Email: drpengxy@163.com

Keywords：

Lymphoma, B-cell/diagnosis; Uveal neoplasms/diagnosis; Ultrasonography, Doppler, color

DOI：

10.3760/cma.j.cn511434-20190411-00196

Video：

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Objective To investigate the clinical, ophthalmological and pathological features of primary uveal lymphoma.Methods Retrospective clinical study. From 2012 to 2018 in Beijing Tongren Eye Cener, 4 cases and 4 eyes of patients with primary uveal lymphoma were included in the study. Among them, 3 cases were male and 1 case was female. The average age was (54 ± 13.58) years old. The average time from initial diagnosis to pathological diagnosis was (18.50 ± 9.29) months. 3 cases were enucleated and 1 case was biopsied. Extranodal marginal zone lymphoma (EMZL) of the mucosa associated lymphoid tissue (MALT) was confirmed by pathological examination. BCVA, fundus color photography, color Doppler ultrasound and orbital MRI were performed in all eyes. UBM, OCT, FFA and ICGA were performed in 2 eyes, 3 eyes, 3 eyes and 2 eyes respectively. The clinical, imaging and pathological changes were observed. Following up time was ≥ 6 months.Results At the initial diagnosis, BCVA was 0.6, 0.02 and 0.01 in 1, 2 and 1 eye respectively. Choroid, ciliary body and iris were involved in 3 eyes, choroid in 1 eye. The fundus of the eyes showed infiltration of choroid in yellow and white color, and the lesions were beyond the vascular arch to the equator and peripheral areas. Color Doppler ultrasonography showed that choroidal diffuse thickening and extrascleral extension (ESE) which was the corresponding hypoechoic areas behind the sclera. Among them, ESE showed crescent thickening in 1 eye and nodular thickening in 3 eyes. UBM showed that the echo of ciliary body was thicken and the internal echo was decreased with the iris involved. OCT showed that RPE was wavy and local retinal neuroepithelial layer detached. FFA showed that the early lesions were mottled with strong and weak fluorescence, and the late fluorescence leakage. The posterior wall of the eyeball was thickened and enhanced in MRI.Conclusion The clinical manifestations of uveal lymphoma are various, color Doppler ultrasound has characteristic manifestations and ESE of crescent or nodular thickening is valuable in diagnosis.

Citation： Zhang Yongpeng, Li Zhihua, Yang Wenli, Tang Xin, Li Bin, Peng Xiaoyan. The clinical features of primary uveal lymphoma of four cases. Chinese Journal of Ocular Fundus Diseases, 2020, 36(6): 447-452. doi: 10.3760/cma.j.cn511434-20190411-00196 Copy

1.	Aronow ME, Portell CA, Sweetenham JW, et al. Uveal lymphoma: clinical features, diagnostic studies, treatment selection, and outcomes[J]. Ophthalmology, 2014, 121(1): 334-341. DOI: 10.1016/j.ophtha.2013.09.004.
2.	Mashayekhi A, Shukla SY, Shields JA, et al. Choroidal lymphoma: clinical features and association with systemic lymphoma[J]. Ophthalmology, 2014, 121(1): 342-351. DOI: 10.1016/j.ophtha.2013.06.046.
3.	Coupland SE, Damato B. Understanding intraocular lymphomas[J]. Clin Exp Ophthalmol, 2008, 36(6): 564-578. DOI: 10.1111/j.1442-9071.2008.01843.x.
4.	Gao X, Li B, You Q, et al. 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[J]. BMC Ophthalmol, 2015, 15(48): 1-8. DOI: 10.1186/s12886-015-0038-7.
5.	Loriaut P, Charlotte F, Bodaghi B, et al. Choroidal and adnexal extranodal marginal zone B cell lymphoma: presentation, imaging findings, and therapeutic management in a series of nine cases[J]. Eye (Lond), 2013, 27(7): 828-835. DOI: 10.1038/eye.2013.74.
6.	Isaacson P, Wright DH. Malignant lymphoma of mucosaassociated lymphoid tissue: a distinctive type of B-cell lymphoma[J]. Cancer, 1983, 52(8): 1410-1416. DOI: 10.1002/1097-0142(19831015)52:8<1410::aid-cncr2820520813>3.0.co;2-3.
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9.	Coupland SE, Foss HD, Hidayat AA, et al. Extranodal marginal zone B cell lymphomas of the uvea: an analysis of 13 cases[J]. J Pathol, 2002, 197(3): 333-340. DOI: 10.1002/path.1130.
10.	Holz FG, Boehmer HV, Mechtersheimer G, et al. Uveal non-Hodgkin's lymphoma with epibulbar extension simulating choroidal effusion syndrome[J]. Retina, 1999, 19(4): 343-346. DOI: 10.1097/00006982-199919040-00015.
11.	Saga T, Ohno S, Matsuda H, et al. Ocular involvement by a peripheral T-cell lymphoma[J]. Arch Ophthalmol, 1984, 102(3): 399-402. DOI: 10.1001/archopht.1984.01040030317027.
12.	Chang TS, Byrne SF, Gass JD, et al. Echographic findings in benign reactive lymphoid hyperplasia of the choroid[J]. Arch Ophthalmol, 1996, 114(6): 669-675. DOI: 10.1001/archopht.1996.01100130661005.
13.	Neudorfer M, Kessler A, Anteby I, et al. Co-existence of intraocular and orbital lymphoma[J]. Acta Ophthalmol Scand, 2004, 82(6): 754-761. DOI: 10.1111/j.1600-0420.2004.00354.x.
14.	Tagami M, Nagai T, Sekimukai D, et al. Uveal extranodal marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue type with concomitant extraocular lesions in a Japanese man[letter][J]. Jpn J Ophthalmol, 2011, 55(5): 585-587. DOI: 10.1007/s10384-011-0013-7.
15.	Theophanous C, Pekmezci M, Damato BE, et al. Choroidal lymphoma discovered on ultrasound in a patient with suspected corneal tumor[J]. Ocul Oncol Pathol, 2018, 4(5): 318-321. DOI: 10.1159/000486039.
16.	Sarraf D, Jain A, Dubovy S, Kreiger, et al. Mucosa-associated lymphoid tissue lymphoma with intraocular involvement[J]. Retina, 2005, 25(1): 94-98. DOI: 10.1097/00006982-200501000-00018.
17.	Arends JE, Bot FJ, Gisbertz IA, et al. Expression of CD10, CD75 and CD43 in MALT lymphoma and their usefulness in discriminating MALT lymphoma from follicular lymphoma and chronic gastritis[J]. Histopathology, 1999, 35(3): 209-215. DOI: 10.1046/j.1365-2559.1999.00694.x.

1. Aronow ME, Portell CA, Sweetenham JW, et al. Uveal lymphoma: clinical features, diagnostic studies, treatment selection, and outcomes[J]. Ophthalmology, 2014, 121(1): 334-341. DOI: 10.1016/j.ophtha.2013.09.004.
2. Mashayekhi A, Shukla SY, Shields JA, et al. Choroidal lymphoma: clinical features and association with systemic lymphoma[J]. Ophthalmology, 2014, 121(1): 342-351. DOI: 10.1016/j.ophtha.2013.06.046.
3. Coupland SE, Damato B. Understanding intraocular lymphomas[J]. Clin Exp Ophthalmol, 2008, 36(6): 564-578. DOI: 10.1111/j.1442-9071.2008.01843.x.
4. Gao X, Li B, You Q, et al. 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[J]. BMC Ophthalmol, 2015, 15(48): 1-8. DOI: 10.1186/s12886-015-0038-7.
5. Loriaut P, Charlotte F, Bodaghi B, et al. Choroidal and adnexal extranodal marginal zone B cell lymphoma: presentation, imaging findings, and therapeutic management in a series of nine cases[J]. Eye (Lond), 2013, 27(7): 828-835. DOI: 10.1038/eye.2013.74.
6. Isaacson P, Wright DH. Malignant lymphoma of mucosaassociated lymphoid tissue: a distinctive type of B-cell lymphoma[J]. Cancer, 1983, 52(8): 1410-1416. DOI: 10.1002/1097-0142(19831015)52:8<1410::aid-cncr2820520813>3.0.co;2-3.
7. Burke JS. Are there site-specific differences among the MALT lymphomas: morphologic, clinical?[J]. Am J Clin Pathol, 1999, 111(1 Suppl 1): S13-143.
8. Harris NL, Jaffe ES, Stein H, et al. A revised European–American classification of lymphoid neoplasms: a proposal from the International Lymphoma Study Group[J]. Blood, 1994, 84(5): 1361-1392. DOI: 10.1182/blood.V84.5.1361.1361.
9. Coupland SE, Foss HD, Hidayat AA, et al. Extranodal marginal zone B cell lymphomas of the uvea: an analysis of 13 cases[J]. J Pathol, 2002, 197(3): 333-340. DOI: 10.1002/path.1130.
10. Holz FG, Boehmer HV, Mechtersheimer G, et al. Uveal non-Hodgkin's lymphoma with epibulbar extension simulating choroidal effusion syndrome[J]. Retina, 1999, 19(4): 343-346. DOI: 10.1097/00006982-199919040-00015.
11. Saga T, Ohno S, Matsuda H, et al. Ocular involvement by a peripheral T-cell lymphoma[J]. Arch Ophthalmol, 1984, 102(3): 399-402. DOI: 10.1001/archopht.1984.01040030317027.
12. Chang TS, Byrne SF, Gass JD, et al. Echographic findings in benign reactive lymphoid hyperplasia of the choroid[J]. Arch Ophthalmol, 1996, 114(6): 669-675. DOI: 10.1001/archopht.1996.01100130661005.
13. Neudorfer M, Kessler A, Anteby I, et al. Co-existence of intraocular and orbital lymphoma[J]. Acta Ophthalmol Scand, 2004, 82(6): 754-761. DOI: 10.1111/j.1600-0420.2004.00354.x.
14. Tagami M, Nagai T, Sekimukai D, et al. Uveal extranodal marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue type with concomitant extraocular lesions in a Japanese man[letter][J]. Jpn J Ophthalmol, 2011, 55(5): 585-587. DOI: 10.1007/s10384-011-0013-7.
15. Theophanous C, Pekmezci M, Damato BE, et al. Choroidal lymphoma discovered on ultrasound in a patient with suspected corneal tumor[J]. Ocul Oncol Pathol, 2018, 4(5): 318-321. DOI: 10.1159/000486039.
16. Sarraf D, Jain A, Dubovy S, Kreiger, et al. Mucosa-associated lymphoid tissue lymphoma with intraocular involvement[J]. Retina, 2005, 25(1): 94-98. DOI: 10.1097/00006982-200501000-00018.
17. Arends JE, Bot FJ, Gisbertz IA, et al. Expression of CD10, CD75 and CD43 in MALT lymphoma and their usefulness in discriminating MALT lymphoma from follicular lymphoma and chronic gastritis[J]. Histopathology, 1999, 35(3): 209-215. DOI: 10.1046/j.1365-2559.1999.00694.x.

Chinese Journal of Ocular Fundus Diseases

The clinical features of primary uveal lymphoma of four cases

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