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Update on Diagnosis and Treatment of Primary Vitreoretinal Lymphoma

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Integrative Ophthalmology

Part of the book series: Advances in Visual Science and Eye Diseases ((AVSED,volume 3))

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Abstract

Primary vitreoretinal lymphoma (PVRL) is one manifestation of primary central nervous system lymphoma (PCNSL) and typically a systemic disease. It often masquerades as chronic uveitis, especially posterior and pan-uveitis, and is frequently misdiagnosed at first. The golden standard for diagnosis is malignant lymphoid cells from the eye. Cytology and immunohistochemistry could confirm the diagnosis of PVRL. Flow cytometry, gene rearrangement, and intraocular cytokine detection are important auxiliary examinations. New methods using microRNA, gene mutation, and SNP might be helpful in the diagnosis and treatment response monitoring. Treatments of PVRL include systemic and local therapy. Intravitreal methotrexate and ocular radiotherapy are used for PVRL patients without brain involvement. Intravitreal anti-CD20 agent is a relatively new therapy, and seems to be safe and effective.

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References

  1. Givner I. Malignant lymphoma with ocular involvement: clinicopathologic report. Bull N Y Acad Med. 1954;30(9):721–2.

    CAS  PubMed  Google Scholar 

  2. Chan CC, Fisson S, Bodaghi B. The future of primary intraocular lymphoma (retinal lymphoma). Ocul Immunol Inflamm. 2009;17(6):375–9.

    Article  PubMed  PubMed Central  Google Scholar 

  3. Chan CC, Rubenstein JL, Coupland SE, Davis JL, Harbour JW, Johnston PB, et al. Primary vitreoretinal lymphoma: a report from an International Primary Central Nervous System Lymphoma Collaborative Group symposium. Oncologist. 2011;16(11):1589–99.

    Article  PubMed  PubMed Central  Google Scholar 

  4. Chan CC, Sen HN. Current concepts in diagnosing and managing primary vitreoretinal (intraocular) lymphoma. Discov Med. 2013;15(81):93–100.

    PubMed  PubMed Central  Google Scholar 

  5. Sagoo MS, Mehta H, Swampillai AJ, Cohen VM, Amin SZ, Plowman PN, et al. Primary intraocular lymphoma. Surv Ophthalmol. 2014;59(5):503–16.

    Article  PubMed  Google Scholar 

  6. Grange LK, Kouchouk A, Dalal MD, Vitale S, Nussenblatt RB, Chan CC, et al. Neoplastic masquerade syndromes in patients with uveitis. Am J Ophthalmol. 2014;157(3):526–31.

    Article  PubMed  Google Scholar 

  7. Faia LJ, Chan CC. Primary intraocular lymphoma. Arch Pathol Lab Med. 2009;133(8):1228–32.

    CAS  PubMed  PubMed Central  Google Scholar 

  8. Rajagopal R, Harbour JW. Diagnostic testing and treatment choices in primary vitreoretinal lymphoma. Retina. 2011;31(3):435–40.

    Article  CAS  PubMed  Google Scholar 

  9. Mudhar HS, Sheard R. Diagnostic cellular yield is superior with full pars plana vitrectomy compared with core vitreous biopsy. Eye (Lond). 2013;27(1):50–5.

    Article  CAS  Google Scholar 

  10. Levasseur SD, Wittenberg LA, White VA. Vitreoretinal lymphoma: a 20-year review of incidence, clinical and cytologic features, treatment, and outcomes. JAMA Ophthalmol. 2013;131(1):50–5.

    Article  PubMed  Google Scholar 

  11. Kinoshita Y, Takasu K, Kobayashi TK, Yuri T, Tsubura A, Shikata N. Diagnosis of intraocular lesions using vitreous humor and intraocular perfusion fluid cytology: experience with 83 cases. Diagn Cytopathol. 2015;43(5):353–9.

    Article  PubMed  Google Scholar 

  12. Coupland SE, Bechrakis NE, Anastassiou G, Foerster AM, Heiligenhaus A, Pleyer U, et al. Evaluation of vitrectomy specimens and chorioretinal biopsies in the diagnosis of primary intraocular lymphoma in patients with Masquerade syndrome. Graefes Arch Clin Exp Ophthalmol. 2003;241(10):860–70.

    Article  PubMed  Google Scholar 

  13. Ranty ML, Laurent C, Aziza J, Mahieu L, Olle P, Quintyn JC, et al. Improving the cytological diagnosis of intraocular lymphoma from vitreous fluid. Histopathology. 2015;67(1):48–61.

    Article  PubMed  Google Scholar 

  14. Sen HN, Bodaghi B, Hoang PL, Nussenblatt R. Primary intraocular lymphoma: diagnosis and differential diagnosis. Ocul Immunol Inflamm. 2009;17(3):133–41.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  15. Coupland SE. Molecular pathology of lymphoma. Eye (Lond). 2013;27(2):180–9.

    Article  CAS  Google Scholar 

  16. Zaldivar RA, Martin DF, Holden JT, Grossniklaus HE. Primary intraocular lymphoma: clinical, cytologic, and flow cytometric analysis. Ophthalmology. 2004;111(9):1762–7.

    PubMed  Google Scholar 

  17. Chan CC. Molecular pathology of primary intraocular lymphoma. Trans Am Ophthalmol Soc. 2003;101:275–92.

    PubMed  PubMed Central  Google Scholar 

  18. Wang Y, Shen D, Wang VM, Sen HN, Chan CC. Molecular biomarkers for the diagnosis of primary vitreoretinal lymphoma. Int J Mol Sci. 2011;12(9):5684–97.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  19. Chan CC, Whitcup SM, Solomon D, Nussenblatt RB. Interleukin-10 in the vitreous of primary intraocular lymphoma. Am J Ophthalmol. 1995;120(5):671–3.

    Article  CAS  PubMed  Google Scholar 

  20. Kimura K, Usui Y, Goto H, Japanese Intraocular Lymphoma Study Group. Clinical features and diagnostic significance of the intraocular fluid of 217 patients with intraocular lymphoma. Jpn J Ophthalmol. 2012;56(4):383–9.

    Article  PubMed  Google Scholar 

  21. Fisson S, Ouakrim H, Touitou V, Baudet S, Ben Abdelwahed R, Donnou S, et al. Cytokine profile in human eyes: contribution of a new cytokine combination for differential diagnosis between intraocular lymphoma or uveitis. PLoS One. 2013;8(2):e52385.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  22. Usui Y, Wakabayashi Y, Okunuki Y, Kimura K, Tajima K, Matsuda R, et al. Immune mediators in vitreous fluids from patients with vitreoretinal B-cell lymphoma. Invest Ophthalmol Vis Sci. 2012;53(9):5395–402.

    Article  CAS  PubMed  Google Scholar 

  23. Ecker S, Hines JC, Pfahler SM, Glaser BM. Aqueous cytokine and growth factor levels do not reliably reflect those levels found in the vitreous. Mol Vis. 2011;17:2856–63.

    CAS  PubMed  PubMed Central  Google Scholar 

  24. Cassoux N, Giron A, Bodaghi B, Tran TH, Baudet S, Davy F, et al. IL-10 measurement in aqueous humor for screening patients with suspicion of primary intraocular lymphoma. Invest Ophthalmol Vis Sci. 2007;48(7):3253–9.

    Article  PubMed  PubMed Central  Google Scholar 

  25. Saleh M, Nikolitch K, Bourcier T, Speeg C, Gaucher D. Repeated IL-10 measurement in aqueous humor and OCT imaging are valuable tools to monitor intraocular lymphoma treated with intravitreal injections of methotrexate. Graefes Arch Clin Exp Ophthalmol. 2012;250(5):761–4.

    Article  PubMed  Google Scholar 

  26. Tuo J, Shen D, Yang HH, Chan CC. Distinct microRNA-155 expression in the vitreous of patients with primary vitreoretinal lymphoma and uveitis. Am J Ophthalmol. 2014;157(3):728–34.

    Article  CAS  PubMed  Google Scholar 

  27. Pulido JS, Salomao DR, Frederick LA, Viswanatha DS. MyD-88 L265P mutations are present in some cases of vitreoretinal lymphoma. Retina. 2015;35(4):624–7.

    Article  CAS  PubMed  Google Scholar 

  28. Bonzheim I, Giese S, Deuter C, Süsskind D, Zierhut M, Waizel M, et al. High frequency of MYD88 mutations in vitreoretinal B-cell lymphoma: a valuable tool to improve diagnostic yield of vitreous aspirates. Blood. 2015;126(1):76–9.

    Article  CAS  PubMed  Google Scholar 

  29. Ramkumar HL, Shen de F, Tuo J, Braziel RM, Coupland SE, Smith JR, et al. IL-10-1082 SNP and IL-10 in primary CNS and vitreoretinal lymphomas. Graefes Arch Clin Exp Ophthalmol. 2012;250(10):1541–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  30. Hoang-Xuan K, Bessell E, Bromberg J, Hottinger AF, Preusser M, Rudà R, et al., European Association for Neuro-Oncology Task Force on Primary CNS Lymphoma. Diagnosis and treatment of primary CNS lymphoma in immunocompetent patients: guidelines from the European Association for Neuro-Oncology. Lancet Oncol. 2015;16(7):e322–32.

    Article  PubMed  Google Scholar 

  31. Bessell EM, Hoang-Xuan K, Ferreri AJ, Reni M. Primary central nervous system lymphoma: biological aspects and controversies in management. Eur J Cancer. 2007;43(7):1141–52.

    Article  PubMed  Google Scholar 

  32. Frenkel S, Hendler K, Siegal T, Shalom E, Pe’er J. Intravitreal methotrexate for treating vitreoretinal lymphoma: 10 years of experience. Br J Ophthalmol. 2008;92(3):383–8.

    Article  CAS  PubMed  Google Scholar 

  33. STuraka K, Bryan JS, De Souza S, Gordon AJ, Kwong HM Jr, Ziemianski MC, et al. Vitreoretinal lymphoma: changing trends in diagnosis and local treatment modalities at a single institution. Clin Lymphoma Myeloma Leuk. 2012;12(6):412–7.

    Article  Google Scholar 

  34. Chu LC, Eberhart CG, Grossman SA, Herman JG. Epigenetic silencing of multiple genes in primary CNS lymphoma. Int J Cancer. 2006;119(10):2487–91.

    Article  CAS  PubMed  Google Scholar 

  35. Gorovoy I, Prechanond T, Abia M, Afshar AR, Stewart JM. Toxic corneal epitheliopathy after intravitreal methotrexate and its treatment with oral folic acid. Cornea. 2013;32(8):1171–3.

    Article  PubMed  Google Scholar 

  36. Isobe K, Ejima Y, Tokumaru S, Shikama N, Suzuki G, Takemoto M, et al. Treatment of primary intraocular lymphoma with radiation therapy: a multi-institutional survey in Japan. Leuk Lymphoma. 2006;47(9):1800–5.

    Article  PubMed  Google Scholar 

  37. Mikami R, Nakayama H, Goto H, Kimura K, Usui Y, Nogi S, et al. Preliminary results of radiotherapy for primary intraocular non-Hodgkin lymphoma. Leuk Lymphoma. 2013;54(10):2181–4.

    Article  PubMed  Google Scholar 

  38. Manna S, Banerjee RK, Augsburger JJ, Al-Rjoub MF, Donnell A, Correa ZM. Biodegradable chitosan and polylactic acid-based intraocular micro-implant for sustained release of methotrexate into vitreous: analysis of pharmacokinetics and toxicity in rabbit eyes. Graefes Arch Clin Exp Ophthalmol. 2015;253(8):1297–305.

    Article  CAS  PubMed  Google Scholar 

  39. Siegal T. Primary central nervous system lymphoma: current state of anti-CD20 therapy and appraisal of reported response criteria. J Clin Neurosci. 2014;21(5):709–15.

    Article  CAS  PubMed  Google Scholar 

  40. Kitzmann AS, Pulido JS, Mohney BG, Baratz KH, Grube T, Marler RJ, et al. Intraocular use of rituximab. Eye (Lond). 2007;21(12):1524–7.

    Article  CAS  Google Scholar 

  41. Mineo JF, Scheffer A, Karkoutly C, Nouvel L, Kerdraon O, Trauet J, et al. Using human CD20-transfected murine lymphomatous B cells to evaluate the efficacy of intravitreal and intracerebral rituximab injections in mice. Invest Ophthalmol Vis Sci. 2008;49(11):4738–45.

    Article  PubMed  Google Scholar 

  42. Larkin KL, Saboo US, Comer GM, Forooghian F, Mackensen F, Merrill P, et al. Use of intravitreal rituximab for treatment of vitreoretinal lymphoma. Br J Ophthalmol. 2014;98(1):99–103.

    Article  PubMed  Google Scholar 

  43. Ben Abdelwahed R, Donnou S, Ouakrim H, Crozet L, Cosette J, Jacquet A, et al. Preclinical study of Ublituximab, a Glycoengineered anti-human CD20 antibody, in murine models of primary cerebral and intraocular B-cell lymphomas. Invest Ophthalmol Vis Sci. 2013;54(5):3657–65.

    Article  PubMed  Google Scholar 

  44. Galand C, Donnou S, Crozet L, Brunet S, Touitou V, Ouakrim H, et al. Th17 cells are involved in the local control of tumor progression in primary intraocular lymphoma. PLoS One. 2011;6(9):e24622.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

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Zhang, X., Zhang, M. (2020). Update on Diagnosis and Treatment of Primary Vitreoretinal Lymphoma. In: Wang, N. (eds) Integrative Ophthalmology. Advances in Visual Science and Eye Diseases, vol 3. Springer, Singapore. https://doi.org/10.1007/978-981-13-7896-6_19

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  • DOI: https://doi.org/10.1007/978-981-13-7896-6_19

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  • Publisher Name: Springer, Singapore

  • Print ISBN: 978-981-13-7895-9

  • Online ISBN: 978-981-13-7896-6

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