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Ocular Adnexal Lymphoma: Clinical Presentation and Imaging Studies

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Ocular and Adnexal Lymphoma

Part of the book series: Essentials in Ophthalmology ((ESSENTIALS))

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Abstract

Ocular adnexal lymphoma (OAL) is a rare tumor arising from conjunctiva, orbital soft tissues including extraocular muscles and lacrimal gland, eyelids, and lacrimal sac. The overall incidence rate of primary OAL is 1–2% of all non-Hodgkin’s lymphomas (NHL) and 5–10% of all extranodal NHL with increasing trend noted in recent times. The most popularized classification and staging system for OAL is the American Joint Committee on Cancer (AJCC) TNM (tumor node metastasis) staging. Extranodal marginal zone B-cell lymphoma (EMZL) of mucosa-associated lymphoid tissue (MALT) variant is the most common type of OAL. The clinical presentation of OAL is insidious in onset, painless, slowly progressive, and associated with proptosis, dystopia, ptosis, “Salmon color conjunctival patch” depending on the location and extent of the tumor is characterized by a well-defined mass molding around the globe without any bony changes or globe indentation on imaging.

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References

  1. Bardenstein DS. Orbital and adnexal lymphoma. In: Singh A, et al., editors. Clinical ophthalmic oncology. Philadelphia, PA: Saunders-Elsevier; 2007. p. 565–70.

    Chapter  Google Scholar 

  2. Sjo LD. Ophthalmic lymphoma: epidemiology and pathogenesis. Acta Ophthalmol. 2009;87(1):1–20.

    Article  PubMed  Google Scholar 

  3. Sjo LD, Ralfkiaer E, Prause JU, et al. Increasing incidence of ophthalmic lymphoma in Denmark from 1980 to 2005. Invest Ophthalmol Vis Sci. 2008;49(8):3283–8.

    Article  PubMed  Google Scholar 

  4. Carbone PP, Kaplan HS, Musshoff K, et al. Report of the Committee on Hodgkin’s disease staging classification. Cancer Res. 1971;31:1860–1.

    CAS  PubMed  Google Scholar 

  5. Heegaard S, Chevez-Barrios P, White VA, Coupland SE, Finger PT. Ocular Adnexal lymphoma. In: Amin MB, Edge SB, Greene FL, Byrd DR, Brookland RK, Washington MK, et al., editors. AJCC cancer staging manual. 8th ed. Cham: Springer International Publishing; 2017. p. 849–54.

    Google Scholar 

  6. Coupland SE, White VA, Rootman JE, et al. A TNM-based clinical staging system of ocular adnexal lymphomas. Arch Pathol Lab Med. 2009;133:1262–7.

    Article  PubMed  Google Scholar 

  7. Cheson BD, Fisher RI, Barrington SF, et al. Recommendations for initial evaluation, staging, and response assessment of Hodgkin and non-Hodgkin lymphoma: the Lugano classification. J Clin Oncol. 2014;32(27):3059–68.

    Article  PubMed  PubMed Central  Google Scholar 

  8. Cheson BD, Ansell S, Schwartz L, et al. Refinement of the Lugano Classification lymphoma response criteria in the era of immunomodulatory therapy. Blood. 2016;128(21):2489–96.

    Article  CAS  PubMed  Google Scholar 

  9. Sjö LD. Ophthalmic lymphoma: epidemiology and pathogenesis. Acta Ophthalmol. 2009;87(1):1–20.

    Article  PubMed  Google Scholar 

  10. Li JJ, Chen W, Sadeghi N. Conjunctival lymphoma. BMJ Case Rep. 2019;12(7):e229599.

    Article  PubMed  PubMed Central  Google Scholar 

  11. Calvo R, Ribera JM, Vaquero M, et al. Low-grade, malt-type, primary B-cell lymphoma of the conjunctiva. Leuk Lymphoma. 1997;28(1–2):203–7.

    Article  CAS  PubMed  Google Scholar 

  12. Rubin PAD, Kent CJ, Jakobiec FA. Orbital and ocular adnexal lymphoid tumors: Albert & Jakobiec’s principles and practice of ophthalmology. 3rd ed. St Louis, MO: WB Saunders Company, Elsevier; 2018.

    Google Scholar 

  13. Ferry JA, Fung CY, Zukerberg L, et al. Lymphoma of the ocular adnexa: a study of 353 cases. Am J Surg Pathol. 2007;31(2):170–84.

    Article  PubMed  Google Scholar 

  14. Oh DE, Kim YD. Lymphoproliferative diseases of the ocular adnexa in Korea. Arch Ophthalmol. 2007;125(12):1668–73.

    Article  PubMed  Google Scholar 

  15. Ozkan MC, Palamar M, Tombuloglu M, et al. Ocular adnexal lymphomas: single-center experience. Clin Lymphoma Myeloma Leuk. 2015;15(Suppl):S158–60.

    Article  PubMed  Google Scholar 

  16. Streubel B, Lamprecht A, Dierlamm J, et al. T(14;18)(q32;q21) involving IGH and MALT1 is a frequent chromosomal aberration in MALT lymphoma. Blood. 2003;101:2335–9.

    Article  CAS  PubMed  Google Scholar 

  17. Streubel B, Vinatzer U, Lamprecht A, Raderer M, Chott A. T(3; 14)(p14.1;q32) involving IGH and FOXP1 is a novel recurrent chromosomal aberration in MALT lymphoma. Leukemia. 2005;19:652–8.

    Article  CAS  PubMed  Google Scholar 

  18. Verdijk RM. Lymphoproliferative tumors of the ocular adnexa. Asia Pac J Ophthalmol. 2017;6(2):132–42.

    Article  CAS  Google Scholar 

  19. Shields JA, Saunders WB. Lymphoid tumors and leukemias. Diagnosis and management of orbital tumors. 1st ed. Philadelphia, PA: J.B. Lippincott; 1989.

    Google Scholar 

  20. Watkins LM, Carter KD, Nerad JA. Ocular adnexal lymphoma of the extraocular muscles: case series from the University of Iowa and review of the literature. Ophthal Plast Reconstr Surg. 2011;27:471–6.

    Article  PubMed  Google Scholar 

  21. Gill MK, Jampol LM. Variations in the presentation of primary intraocular lymphoma: case reports and a review. Surv Ophthalmol. 2001;45:463–71.

    Article  CAS  PubMed  Google Scholar 

  22. Kuro M, Matsuyama K, Matsuoka M, et al. Case of primary intraocular lymphoma with extraocular extension. Ocul Oncol Pathol. 2015;2(2):66–70.

    Article  PubMed  PubMed Central  Google Scholar 

  23. Demirci H, Shields C, Shields J, et al. Orbital tumors in the older adult population. Ophthalmology. 2002;109:243–8.

    Article  PubMed  Google Scholar 

  24. Coupland SE, Damato B. Lymphomas involving the eye and the ocular adnexa. Curr Opin Ophthalmol. 2006;17:523–31.

    Article  PubMed  Google Scholar 

  25. Knowles DM, Jakobiec FA, McNally L, et al. Lymphoid hyperplasia and malignant lymphoma occurring in the ocular adnexa (orbit, conjunctiva, and eyelids): a prospective multiparametric analysis of 108 cases during 1977 to 1987. Hum Pathol. 1990;21:959–73.

    Article  CAS  PubMed  Google Scholar 

  26. Olsen TG, Heegaard S. Orbital lymphoma. Surv Ophthalmol. 2019;64(1):45–66.

    Article  PubMed  Google Scholar 

  27. Charton J, Witherspoon SR, Itani K, et al. Natural killer/T-cell lymphoma masquerading as orbital cellulitis. Ophthal Plast Reconstr Surg. 2008;24(2):143–5.

    Article  PubMed  Google Scholar 

  28. Cassidy DT, McKelvie P, Harris GJ, et al. Lacrimal gland orbital lobe cysts associated with MALT lymphoma and primary Sjogren’s syndrome. Orbit. 2005;24(4):257–63.

    Article  PubMed  Google Scholar 

  29. Coupland SE, Foss HD, Hummel M, et al. Extranodal marginal zone B-cell lymphoma of the lacrimal gland associated with crystal-storing histiocytosis. Ophthalmology. 2002;109(1):105–10.

    Article  PubMed  Google Scholar 

  30. Bertelmann E, Minko N. Safety of the preoperative evaluation of pleomorphic adenoma: a retrospective analysis. Klin Monatsbl Augenheilkd. 2012;229(3):231–5.

    Article  CAS  PubMed  Google Scholar 

  31. Coupland SE, Hellmich M, Auw-Haedrich C, et al. Prognostic value of cell-cycle markers in ocular adnexal lymphoma: an assessment of 230 cases. Graefes Arch Clin Exp Ophthalmol. 2004;242:130–45.

    Article  PubMed  Google Scholar 

  32. Coupland SE, Krause L, Delecluse HJ, et al. Lymphoproliferative lesions of the ocular adnexa. Analysis of 112 cases. Ophthalmology. 1998;105:1430–41.

    Article  CAS  PubMed  Google Scholar 

  33. Fung CY, Tarbell NJ, Lucarelli MJ, et al. Ocular adnexal lymphoma: clinical behavior of distinct World Health Organization classification subtypes. Int J Radiat Oncol Biol Phys. 2003;57:1382–91.

    Article  PubMed  Google Scholar 

  34. McKelvie PA, McNab A, Francis IC, et al. Ocular adnexal lymphoproliferative disease: a series of 73 cases. Clin Exp Ophthalmol. 2001;29:387–93.

    Article  CAS  PubMed  Google Scholar 

  35. Meunier J, Lumbroso-Le Rouic L, Vincent-Salomon A, et al. Ophthalmologic and intraocular non-Hodgkin’s lymphoma: a large single centre study of initial characteristics, natural history, and prognostic factors. Hematol Oncol. 2004;22:143–58.

    Article  PubMed  Google Scholar 

  36. Sullivan TJ, Whitehead K, Williamson R, et al. Lymphoproliferative disease of the ocular adnexa: a clinical and pathologic study with statistical analysis of 69 patients. Ophthal Plast Reconstr Surg. 2005;21:177–88.

    Article  PubMed  Google Scholar 

  37. Alkatan HM, Alaraj A, El-Khani A, et al. Ocular adnexal lymphoproliferative disorders in an ophthalmic referral center in Saudi Arabia. Saudi J Ophthalmol. 2013;27:227–30.

    Article  PubMed  PubMed Central  Google Scholar 

  38. Berry-Brincat A, Tomlins P, Hall A, et al. Primary extrasac orbital lymphoma presenting as nasolacrimal obstruction. Orbit. 2008;27(3):175–7.

    Article  CAS  PubMed  Google Scholar 

  39. Mashayekhi A, Shukla SY, Shields JA, et al. Choroidal lymphoma: clinical features and association with systemic lymphoma. Ophthalmology. 2014;121(1):342–51.

    Article  PubMed  Google Scholar 

  40. Shields CL, Shields JA, Carvalho C, Rundle P, Smith AF. Conjunctival lymphoid tumors: clinical analysis of 117 cases and relationship to systemic lymphoma. Ophthalmology. 2001;108(5):979–84.

    Article  CAS  PubMed  Google Scholar 

  41. Demirci H, Shields CL, Karatza EC, et al. Orbital lymphoproliferative tumors: analysis of clinical features and systemic involvement in 160 cases. Ophthalmology. 2008;115(9):1626–31.

    Article  PubMed  Google Scholar 

  42. Vempuluru VS, Jakati S, Godbole A, et al. Spectrum of AS-OCT features of ocular surface tumors and correlation of clinico-tomographic features with histopathology: a study of 70 lesions. Int Ophthalmol. 2021;41(11):3571–86.

    Article  PubMed  Google Scholar 

  43. Naik MN, Tourani KL, Sekhar GC, et al. Interpretation of computed tomography imaging of the eye and orbit: a systematic approach. Indian J Ophthalmol. 2002;50:339–53.

    PubMed  Google Scholar 

  44. Sullivan TJ, Valenzuela AA. Imaging features of ocular adnexal lymphoproliferative disease. Eye. 2006;20:1189–95.

    Article  CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Hima Bindu Yalamanchili Centre for Eye Cancer, Operation Eyesight Universal Institute for Eye Cancer, L V Prasad Eye Institute, Vijayawada, India

    Kavya Madhuri Bejjanki

  2. The Operation Eyesight Universal Institute for Eye Cancer, L V Prasad Eye Institute, Hyderabad, India

    Swathi Kaliki

Authors
  1. Kavya Madhuri Bejjanki
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  2. Swathi Kaliki
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Editor information

Editors and Affiliations

  1. Anant Bajaj Retina Institute, The Operation Eyesight Universal Institute for Eye Cancer, L V Prasad Eye Institute, Hyderabad, Telangana, India

    Vishal R. Raval

  2. Stanford Medicine, Palo Alto, CA, USA

    Prithvi Mruthyunjaya

  3. Cleveland Clinic, Cole Eye Institute, Cleveland, OH, USA

    Arun D. Singh

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Bejjanki, K.M., Kaliki, S. (2023). Ocular Adnexal Lymphoma: Clinical Presentation and Imaging Studies. In: Raval, V.R., Mruthyunjaya, P., Singh, A.D. (eds) Ocular and Adnexal Lymphoma. Essentials in Ophthalmology. Springer, Cham. https://doi.org/10.1007/978-3-031-24595-4_5

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  • DOI: https://doi.org/10.1007/978-3-031-24595-4_5

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-031-24594-7

  • Online ISBN: 978-3-031-24595-4

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