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Primary Uveal Non-Hodgkin Lymphoma

MedGen UID:
1786099
•Concept ID:
C5446532
•
Neoplastic Process
Synonym: Primary Uveal Lymphoma

Definition

An indolent, low-grade B-cell non-Hodgkin lymphoma that arises from the choroid, iris, or ciliary body. It is characterized by the presence of a diffuse infiltrate of small, round lymphocytes. Lymphoid follicles with germinal centers may be present. In the past these tumors were termed ''reactive lymphoid hyperplasia''. Now they are considered low-grade B-cell lymphomas, most commonly extranodal marginal zone lymphomas of mucosa-associated lymphoid tissue. [from NCI]

Professional guidelines

PubMed

Association of Disease Location and Treatment With Survival in Diffuse Large B-Cell Lymphoma of the Eye and Ocular Adnexal Region.
Ahmed AH, Foster CS, Shields CL
JAMA Ophthalmol 2017 Oct 1;135(10):1062-1068. doi: 10.1001/jamaophthalmol.2017.3286. PMID: 28880986Free PMC Article
Local treatment for lymphoid malignancies of the eye.
Giuliari GP, Hinkle DM, Foster CS
Anticancer Agents Med Chem 2009 Dec;9(10):1123-8. doi: 10.2174/187152009789735071. PMID: 19925396
Chronic non-infectious uveitis in the elderly: epidemiology, pathophysiology and management.
Gupta R, Murray PI
Drugs Aging 2006;23(7):535-58. doi: 10.2165/00002512-200623070-00001. PMID: 16930083

Recent clinical studies

Etiology

Multipurpose ultrasonographic characteristics of primary uveal MALT lymphoma.
Zhang L, Zhang T, Xu G, Zhang C, Qian J, Chang Q, Bi Y, Chen Q
Graefes Arch Clin Exp Ophthalmol 2023 Aug;261(8):2383-2394. Epub 2023 Mar 18 doi: 10.1007/s00417-022-05958-2. PMID: 36933009
Mucosal associated Lymphoid Tissue Lymphoma of the uvea: an analysis of 3 cases.
Wang Z, Liu Y, Mo J, Cao X, Xu X, Shen L, Wang H, Wei W
BMC Ophthalmol 2022 Sep 19;22(1):371. doi: 10.1186/s12886-022-02598-2. PMID: 36123589Free PMC Article
Familial associations of female breast cancer with other cancers.
Zheng G, Yu H, Hemminki A, Försti A, Sundquist K, Hemminki K
Int J Cancer 2017 Dec 1;141(11):2253-2259. Epub 2017 Aug 26 doi: 10.1002/ijc.30927. PMID: 28801919
Malignant uveitis masquerade syndromes.
Kubicka-Trzaska A, Romanowska-Dixon B
Klin Oczna 2008;110(4-6):199-202. PMID: 18655462
Chronic non-infectious uveitis in the elderly: epidemiology, pathophysiology and management.
Gupta R, Murray PI
Drugs Aging 2006;23(7):535-58. doi: 10.2165/00002512-200623070-00001. PMID: 16930083

Diagnosis

Multipurpose ultrasonographic characteristics of primary uveal MALT lymphoma.
Zhang L, Zhang T, Xu G, Zhang C, Qian J, Chang Q, Bi Y, Chen Q
Graefes Arch Clin Exp Ophthalmol 2023 Aug;261(8):2383-2394. Epub 2023 Mar 18 doi: 10.1007/s00417-022-05958-2. PMID: 36933009
Large B-cell lymphoma of the uvea: Histopathologic variants and clinicopathologic correlation.
Valenzuela J, Yeaney GA, Hsi ED, Azzato EM, Peereboom DM, Singh AD
Surv Ophthalmol 2020 May-Jun;65(3):361-370. Epub 2019 Oct 30 doi: 10.1016/j.survophthal.2019.10.008. PMID: 31676312
Case Report: Primary Conjunctival Non-Hodgkin Marginal Zone Lymphoma.
Bennett LW
Optom Vis Sci 2019 Feb;96(2):133-136. doi: 10.1097/OPX.0000000000001337. PMID: 30589762
Malignant uveitis masquerade syndromes.
Kubicka-Trzaska A, Romanowska-Dixon B
Klin Oczna 2008;110(4-6):199-202. PMID: 18655462
Primary intraocular lymphoma: a review of the clinical, histopathological and molecular biological features.
Coupland SE, Heimann H, Bechrakis NE
Graefes Arch Clin Exp Ophthalmol 2004 Nov;242(11):901-13. Epub 2004 Oct 29 doi: 10.1007/s00417-004-0973-0. PMID: 15565454

Therapy

Diffuse Uveal Melanocytic Proliferation With Primary Vitreoretinal Lymphoma.
Leskov I, Lyon AT, Jampol LM
JAMA Ophthalmol 2019 Jul 1;137(7):834-837. doi: 10.1001/jamaophthalmol.2019.0905. PMID: 31046111Free PMC Article
Case Report: Primary Conjunctival Non-Hodgkin Marginal Zone Lymphoma.
Bennett LW
Optom Vis Sci 2019 Feb;96(2):133-136. doi: 10.1097/OPX.0000000000001337. PMID: 30589762
Mucosa-associated lymphoid tissue lymphoma with intraocular and orbital involvement: case presentation and review of the literature.
Freedman K, Shenoy S
Orbit 2018 Aug;37(4):243-247. Epub 2017 Oct 13 doi: 10.1080/01676830.2017.1383479. PMID: 29027822
Local treatment for lymphoid malignancies of the eye.
Giuliari GP, Hinkle DM, Foster CS
Anticancer Agents Med Chem 2009 Dec;9(10):1123-8. doi: 10.2174/187152009789735071. PMID: 19925396
Oculocerebral non-Hodgkin's lymphoma with uveal involvement: development of an epibulbar tumor after vitrectomy.
Cursiefen C, Holbach LM, Lafaut B, Heimann K, Kirchner T, Naumann GO
Arch Ophthalmol 2000 Oct;118(10):1437-40. doi: 10.1001/archopht.118.10.1437. PMID: 11030832

Prognosis

Mucosal associated Lymphoid Tissue Lymphoma of the uvea: an analysis of 3 cases.
Wang Z, Liu Y, Mo J, Cao X, Xu X, Shen L, Wang H, Wei W
BMC Ophthalmol 2022 Sep 19;22(1):371. doi: 10.1186/s12886-022-02598-2. PMID: 36123589Free PMC Article
Large B-cell lymphoma of the uvea: Histopathologic variants and clinicopathologic correlation.
Valenzuela J, Yeaney GA, Hsi ED, Azzato EM, Peereboom DM, Singh AD
Surv Ophthalmol 2020 May-Jun;65(3):361-370. Epub 2019 Oct 30 doi: 10.1016/j.survophthal.2019.10.008. PMID: 31676312
Malignant uveitis masquerade syndromes.
Kubicka-Trzaska A, Romanowska-Dixon B
Klin Oczna 2008;110(4-6):199-202. PMID: 18655462
Chronic non-infectious uveitis in the elderly: epidemiology, pathophysiology and management.
Gupta R, Murray PI
Drugs Aging 2006;23(7):535-58. doi: 10.2165/00002512-200623070-00001. PMID: 16930083
Primary intraocular lymphoma: a review of the clinical, histopathological and molecular biological features.
Coupland SE, Heimann H, Bechrakis NE
Graefes Arch Clin Exp Ophthalmol 2004 Nov;242(11):901-13. Epub 2004 Oct 29 doi: 10.1007/s00417-004-0973-0. PMID: 15565454

Clinical prediction guides

Mucosal associated Lymphoid Tissue Lymphoma of the uvea: an analysis of 3 cases.
Wang Z, Liu Y, Mo J, Cao X, Xu X, Shen L, Wang H, Wei W
BMC Ophthalmol 2022 Sep 19;22(1):371. doi: 10.1186/s12886-022-02598-2. PMID: 36123589Free PMC Article
Large B-cell lymphoma of the uvea: Histopathologic variants and clinicopathologic correlation.
Valenzuela J, Yeaney GA, Hsi ED, Azzato EM, Peereboom DM, Singh AD
Surv Ophthalmol 2020 May-Jun;65(3):361-370. Epub 2019 Oct 30 doi: 10.1016/j.survophthal.2019.10.008. PMID: 31676312
Diffuse Uveal Melanocytic Proliferation With Primary Vitreoretinal Lymphoma.
Leskov I, Lyon AT, Jampol LM
JAMA Ophthalmol 2019 Jul 1;137(7):834-837. doi: 10.1001/jamaophthalmol.2019.0905. PMID: 31046111Free PMC Article
Case Report: Primary Conjunctival Non-Hodgkin Marginal Zone Lymphoma.
Bennett LW
Optom Vis Sci 2019 Feb;96(2):133-136. doi: 10.1097/OPX.0000000000001337. PMID: 30589762
Intraocular collision tumour: case report and literature review.
Coupland SE, Dodson A, Liu H, Du MQ, Angi M, Damato BE
Graefes Arch Clin Exp Ophthalmol 2013 May;251(5):1383-8. Epub 2012 Dec 12 doi: 10.1007/s00417-012-2216-0. PMID: 23232651

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