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Primary Ciliary Body Non-Hodgkin Lymphoma

MedGen UID:
1786572
•Concept ID:
C5446547
•
Neoplastic Process
Synonym: Primary Ciliary Body Lymphoma

Definition

An extremely rare low-grade B-cell non-Hodgkin lymphoma that arises from the ciliary body. It is characterized by the presence of a diffuse infiltrate of small, round lymphocytes. Most cases represent extranodal marginal zone lymphomas of mucosa-associated lymphoid tissue. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPrimary Ciliary Body Non-Hodgkin Lymphoma

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

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
Primary Ciliary Body Marginal Zone Lymphoma Presenting as Hemorrhagic Hypopyon.
Koç İ, Kiratli H, Kapucu Y, Gündoğdu F, Sağlam A
Ocul Immunol Inflamm 2021 Apr 3;29(3):496-499. Epub 2020 Oct 16 doi: 10.1080/09273948.2020.1815798. PMID: 33064042
Second primary malignancies of eye and ocular adnexa after a first primary elsewhere in the body.
Alfaar AS, Saad AM, KhalafAllah MT, Elsherif OE, Osman MH, Strauß O
Graefes Arch Clin Exp Ophthalmol 2021 Feb;259(2):515-526. Epub 2020 Sep 1 doi: 10.1007/s00417-020-04896-1. PMID: 32870371Free PMC Article
Choroidal lymphoma: clinical features and association with systemic lymphoma.
Mashayekhi A, Shukla SY, Shields JA, Shields CL
Ophthalmology 2014 Jan;121(1):342-351. Epub 2013 Aug 23 doi: 10.1016/j.ophtha.2013.06.046. PMID: 23978622

Diagnosis

Primary Ciliary Body Marginal Zone Lymphoma Presenting as Hemorrhagic Hypopyon.
Koç İ, Kiratli H, Kapucu Y, Gündoğdu F, Sağlam A
Ocul Immunol Inflamm 2021 Apr 3;29(3):496-499. Epub 2020 Oct 16 doi: 10.1080/09273948.2020.1815798. PMID: 33064042
Second primary malignancies of eye and ocular adnexa after a first primary elsewhere in the body.
Alfaar AS, Saad AM, KhalafAllah MT, Elsherif OE, Osman MH, Strauß O
Graefes Arch Clin Exp Ophthalmol 2021 Feb;259(2):515-526. Epub 2020 Sep 1 doi: 10.1007/s00417-020-04896-1. PMID: 32870371Free 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
Diagnostic dilemma of ocular lymphoma.
Batis V, Shuttleworth J, Shuttleworth G, Williams GS
BMJ Case Rep 2019 Jun 6;12(6) doi: 10.1136/bcr-2019-229513. PMID: 31175115Free PMC Article
Choroidal lymphoma: clinical features and association with systemic lymphoma.
Mashayekhi A, Shukla SY, Shields JA, Shields CL
Ophthalmology 2014 Jan;121(1):342-351. Epub 2013 Aug 23 doi: 10.1016/j.ophtha.2013.06.046. PMID: 23978622

Therapy

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
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
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.
Rasić DM, Stanković Z, Terzić T, Kovacević D, Koturović Z, Marković V
Med Oncol 2010 Sep;27(3):1010-6. Epub 2009 Oct 9 doi: 10.1007/s12032-009-9325-6. PMID: 19816817

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
Choroidal lymphoma: clinical features and association with systemic lymphoma.
Mashayekhi A, Shukla SY, Shields JA, Shields CL
Ophthalmology 2014 Jan;121(1):342-351. Epub 2013 Aug 23 doi: 10.1016/j.ophtha.2013.06.046. PMID: 23978622
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
Iris involvement in primary intraocular lymphoma: report of two cases and review of the literature.
Velez G, de Smet MD, Whitcup SM, Robinson M, Nussenblatt RB, Chan CC
Surv Ophthalmol 2000 May-Jun;44(6):518-26. doi: 10.1016/s0039-6257(00)00118-1. PMID: 10906383

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