Skip to main content
SpringerLink
Log in

Orbital and Adnexal Lymphoma

  • Chapter
  • First Online:
Book cover Clinical Ophthalmic Oncology

  • 1070 Accesses

Abstract

Orbital and adnexal lymphoma (OAL) includes a heterogeneous group of lymphomas, the majority of which are low-grade, indolent, B cell, non-Hodgkin’s lymphomas (NHLs). By definition, OAL affects structures including the eyelids, conjunctiva, lacrimal apparatus, extraocular muscles, and the orbit. Disease may be limited to a single, localized tumor, or it may be multifocal. Overlap with ocular adnexal sites is common (10–20 % of cases), and coexisting uveal involvement has been observed. Moreover, OAL can affect regional, central, and peripheral lymph nodes as well as other distant extranodal sites. The 10-year, disease-specific mortality is approximately 5–10 %. Many of the advances in understanding OAL were initially demonstrated in systemic lymphoma.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 89.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 119.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 169.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Freeman C, Berg JW, Cutler SJ. Occurrence and prognosis of extranodal lymphomas. Cancer. 1972;29(1):252–60.

    Article  PubMed  CAS  Google Scholar 

  2. Fuller ML, Sweetenham J, Schoenfield L, Singh AD. Uveal lymphoma: a variant of ocular adnexal lymphoma. Leuk Lymphoma. 2008;49(12):2393–7.

    Article  PubMed  Google Scholar 

  3. Grossniklaus HE, Martin DF, Avery R, et al. Uveal lymphoid infiltration. Report of four cases and clinicopathologic review. Ophthalmology. 1998;105(7):1265–73.

    Article  PubMed  CAS  Google Scholar 

  4. Baryla J, Allen LH, Kwan K, et al. Choroidal lymphoma with orbital and optic nerve extension: case and review of literature. Can J Ophthalmol. 2012;47(1):79–81.

    Article  PubMed  Google Scholar 

  5. Sarraf D, Jain A, Dubovy S, et al. Mucosa-associated lymphoid tissue lymphoma with intraocular involvement. Retina. 2005;25(1):94–8.

    Article  PubMed  Google Scholar 

  6. 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. Jpn J Ophthalmol. 2011;55(5):585–7.

    Article  PubMed  Google Scholar 

  7. 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(2):130–45.

    Article  PubMed  Google Scholar 

  8. Cohen VM, Sweetenham J, Singh AD. Ocular adnexal lymphoma. What is the evidence for an infectious aetiology? Br J Ophthalmol. 2008;92(4):446–8.

    Article  PubMed  Google Scholar 

  9. Hatef E, Roberts D, McLaughlin P, et al. Prevalence and nature of systemic involvement and stage at initial examination in patients with orbital and ocular adnexal lymphoma. Arch Ophthalmol. 2007;125(12):1663–7.

    Article  PubMed  Google Scholar 

  10. Bayraktar S, Bayraktar UD, Stefanovic A, Lossos IS. Primary ocular adnexal mucosa-associated lymphoid tissue lymphoma (MALT): single institution experience in a large cohort of patients. Br J Haematol. 2011;152(1):72–80.

    Article  PubMed  Google Scholar 

  11. Shields CL, Shields JA, Carvalho C, et al. Conjunctival lymphoid tumors: clinical analysis of 117 cases and relationship to systemic lymphoma. Ophthalmology. 2001;108(5):979–84.

    Article  PubMed  CAS  Google Scholar 

  12. Fisher SG, Fisher RI. The epidemiology of non-Hodgkin’s lymphoma. Oncogene. 2004;23(38):6524–34.

    Article  PubMed  CAS  Google Scholar 

  13. Shields JA, Bakewell B, Augsburger JJ, Flanagan JC. Classification and incidence of space-occupying lesions of the orbit. A survey of 645 biopsies. Arch Ophthalmol. 1984;102(11):1606–11.

    Article  PubMed  CAS  Google Scholar 

  14. Char DH. Orbital lymphoid lesions. In: Char DH, editor. Clinical ocular oncology. Philadelphia: Lippincott-Raven; 1997.

    Google Scholar 

  15. Henderson JW. The individual tumors. In: Henderson JW, Farrow GM, editors. Orbital tumors. New York: Raven; 1994.

    Google Scholar 

  16. Spraul CW, Grossniklaus HE. Analysis of 24,444 surgical specimens accessioned over 55 years in an ophthalmic pathology laboratory. Int Ophthalmol. 1997;21(5):283–304.

    Article  PubMed  Google Scholar 

  17. Jenkins C, Rose GE, Bunce C, et al. Histological features of ocular adnexal lymphoma (REAL classification) and their association with patient morbidity and survival. Br J Ophthalmol. 2000;84(8):907–13.

    Article  PubMed  CAS  Google Scholar 

  18. Jenkins C, Rose GE, Bunce C, et al. Clinical features associated with survival of patients with lymphoma of the ocular adnexa. Eye (Lond). 2003;17(7):809–20.

    Article  CAS  Google Scholar 

  19. 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 

  20. Knowles DM, Jakobiec FA, McNally L, Burke JS. 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(9):959–73.

    Article  PubMed  CAS  Google Scholar 

  21. Stafford SL, Kozelsky TF, Garrity JA, et al. Orbital lymphoma: radiotherapy outcome and complications. Radiother Oncol. 2001;59(2):139–44.

    Article  PubMed  CAS  Google Scholar 

  22. Cockerham GC, Hidayat AA, Bijwaard KE, Sheng ZM. Re-evaluation of “reactive lymphoid hyperplasia of the uvea”: an immunohistochemical and molecular analysis of 10 cases. Ophthalmology. 2000;107(1):151–8.

    Article  PubMed  CAS  Google Scholar 

  23. LeBien TW, Tedder TF. B lymphocytes: how they develop and function. Blood. 2008;112(5):1570–80.

    Article  PubMed  CAS  Google Scholar 

  24. Ye H, Liu H, Attygalle A, et al. Variable frequencies of t(11;18)(q21;q21) in MALT lymphomas of different sites: significant association with CagA strains of H pylori in gastric MALT lymphoma. Blood. 2003;102(3):1012–8.

    Article  PubMed  CAS  Google Scholar 

  25. Raffeld M, Jaffe ES. bcl-1, t(11;14), and mantle cell-derived lymphomas. Blood. 1991;78(2):259–63.

    PubMed  CAS  Google Scholar 

  26. Takada S, Yoshino T, Taniwaki M, et al. Involvement of the chromosomal translocation t(11;18) in some mucosa-associated lymphoid tissue lymphomas and diffuse large B-cell lymphomas of the ocular adnexa: evidence from multiplex reverse transcriptase-polymerase chain reaction and fluorescence in situ hybridization on using formalin-fixed, paraffin-embedded specimens. Mod Pathol. 2003;16(5):445–52.

    Article  PubMed  Google Scholar 

  27. Remstein ED, James CD, Kurtin PJ. Incidence and subtype specificity of API2-MALT1 fusion translocations in extranodal, nodal, and splenic marginal zone lymphomas. Am J Pathol. 2000;156(4):1183–8.

    Article  PubMed  CAS  Google Scholar 

  28. Hoover RN. Lymphoma risks in populations with altered immunity – a search for mechanism. Cancer Res. 1992;52(19 Suppl):5477s–8.

    PubMed  CAS  Google Scholar 

  29. Ferreri AJ, Guidoboni M, Ponzoni M, et al. Evidence for an association between Chlamydia psittaci and ocular adnexal lymphomas. J Natl Cancer Inst. 2004;96(8):586–94.

    Article  PubMed  Google Scholar 

  30. Chan CC, Smith JA, Shen DF, et al. Helicobacter pylori (H. pylori) molecular signature in conjunctival mucosa-associated lymphoid tissue (MALT) lymphoma. Histol Histopathol. 2004;19(4):1219–26.

    PubMed  CAS  Google Scholar 

  31. Chanudet E, Zhou Y, Bacon CM, et al. Chlamydia psittaci is variably associated with ocular adnexal MALT lymphoma in different geographical regions. J Pathol. 2006;209(3):344–51.

    Article  PubMed  CAS  Google Scholar 

  32. Husain A, Roberts D, Pro B, et al. Meta-analyses of the association between Chlamydia psittaci and ocular adnexal lymphoma and the response of ocular adnexal lymphoma to antibiotics. Cancer. 2007;110(4):809–15.

    Article  PubMed  Google Scholar 

  33. Amin S, Ramsay A. Orbital and lacrimal gland progressive transformation of germinal centres – an underdiagnosed entity? Br J Ophthalmol. 2012;96(9):1242–5.

    Article  PubMed  Google Scholar 

  34. Stacy RC, Jakobiec FA, Schoenfield L, Singh AD. Unifocal and multifocal reactive lymphoid hyperplasia vs follicular lymphoma of the ocular adnexa. Am J Ophthalmol. 2010;150(3):412–26.e1.

    Article  PubMed  Google Scholar 

  35. Harris NL, Jaffe ES, Stein H, et al. A revised European-American classification of lymphoid neoplasms: a proposal from the International Lymphoma Study Group. Blood. 1994;84(5):1361–92.

    PubMed  CAS  Google Scholar 

  36. Swerdlow SH, Campo E, Harris NL, et al., editors. World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues. Lyon: IARC Press; 2008.

    Google Scholar 

  37. 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(5):1382–91.

    Article  PubMed  Google Scholar 

  38. McKelvie PA, McNab A, Francis IC, et al. Ocular adnexal lymphoproliferative disease: a series of 73 cases. Clin Experiment Ophthalmol. 2001;29(6):387–93.

    Article  PubMed  CAS  Google Scholar 

  39. Mannami T, Yoshino T, Oshima K, et al. Clinical, histopathological, and immunogenetic analysis of ocular adnexal lymphoproliferative disorders: characterization of malt lymphoma and reactive lymphoid hyperplasia. Mod Pathol. 2001;14(7):641–9.

    Article  PubMed  CAS  Google Scholar 

  40. Bhatia S, Paulino AC, Buatti JM, et al. Curative radiotherapy for primary orbital lymphoma. Int J Radiat Oncol Biol Phys. 2002;54(3):818–23.

    Article  PubMed  Google Scholar 

  41. Sharara N, Holden JT, Wojno TH, et al. Ocular adnexal lymphoid proliferations: clinical, histologic, flow cytometric, and molecular analysis of forty-three cases. Ophthalmology. 2003;110(6):1245–54.

    Article  PubMed  Google Scholar 

  42. Nakata M, Matsuno Y, Katsumata N, et al. Histology according to the Revised European-American Lymphoma Classification significantly predicts the prognosis of ocular adnexal lymphoma. Leuk Lymphoma. 1999;32(5–6):533–43.

    PubMed  CAS  Google Scholar 

  43. White VA, Gascoyne RD, McNeil BK, et al. Histopathologic findings and frequency of clonality detected by the polymerase chain reaction in ocular adnexal lymphoproliferative lesions. Mod Pathol. 1996;9(11):1052–61.

    PubMed  CAS  Google Scholar 

  44. Cho EY, Han JJ, Ree HJ, et al. Clinicopathologic analysis of ocular adnexal lymphomas: extranodal marginal zone b-cell lymphoma constitutes the vast majority of ocular lymphomas among Koreans and affects younger patients. Am J Hematol. 2003;73(2):87–96.

    Article  PubMed  Google Scholar 

  45. Saatci AO, Arikan G, Ozcan MA, et al. Indocyanine green angiographic features of systemic non-Hodgkin’s lymphoma and bilateral choroidal involvement. Ophthalmic Surg Lasers Imaging. 2006;37(3):236–9.

    PubMed  Google Scholar 

  46. Coupland SE, Krause L, Delecluse HJ, et al. Lymphoproliferative lesions of the ocular adnexa. Analysis of 112 cases. Ophthalmology. 1998;105(8):1430–41.

    Article  PubMed  CAS  Google Scholar 

  47. Decaudin D, de Cremoux P, Vincent-Salomon A, et al. Ocular adnexal lymphoma: a review of clinicopathologic features and treatment options. Blood. 2006;108(5):1451–60.

    Article  PubMed  CAS  Google Scholar 

  48. Gardiner JA, White VA, Gascoyne RD, Rootman J. Histopathologic, immunophenotypic and genotypic analyses in ocular adnexal lymphoproliferative disorders. Aust N Z J Ophthalmol. 1992;20(3):247–51.

    Article  PubMed  CAS  Google Scholar 

  49. Plaisier MB, Sie-Go DM, Berendschot TT, et al. Ocular adnexal lymphoma classified using the WHO classification: not only histology and stage, but also gender is a predictor of outcome. Orbit. 2007;26(2):83–8.

    Article  PubMed  Google Scholar 

  50. Johnson TE, Tse DT, Byrne Jr GE, et al. Ocular-adnexal lymphoid tumors: a clinicopathologic and molecular genetic study of 77 patients. Ophthal Plast Reconstr Surg. 1999;15(3):171–9.

    Article  PubMed  CAS  Google Scholar 

  51. Coupland SE, White VA, Rootman J, et al. A TNM-based clinical staging system of ocular adnexal lymphomas. Arch Pathol Lab Med. 2009;133(8):1262–7.

    PubMed  Google Scholar 

  52. Edge SE, Byrd DR, Compton CC, et al. AJCC cancer staging manual. 7th ed. New York: Springer; 2009. http://www.springerlink.com. Accessed 18 Jan 2013.

  53. Lee SE, Paik JS, Cho WK, et al. Feasibility of the TNM-based staging system of ocular adnexal extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma). Am J Hematol. 2011;86(3):262–6.

    Article  PubMed  Google Scholar 

  54. Liu H, Ye H, Dogan A, et al. T(11;18)(q21;q21) is associated with advanced mucosa-associated lymphoid tissue lymphoma that expresses nuclear BCL10. Blood. 2001;98(4):1182–7.

    Article  PubMed  CAS  Google Scholar 

  55. Moore AT, Pritchard J, Taylor DS. Histiocytosis X: an ophthalmological review. Br J Ophthalmol. 1985;69(1):7–14.

    Article  PubMed  CAS  Google Scholar 

  56. Herwig MC, Wojno T, Zhang Q, Grossniklaus HE. Langerhans cell histiocytosis of the orbit: five clinicopathologic cases and review of the literature. Surv Ophthalmol. 2013;58(4):330–40.

    Article  PubMed  Google Scholar 

  57. Thanos A, Vavvas D, Young LH, Foster CS. Choroidal neovascular membrane formation and retinochoroidopathy in a patient with systemic langerhans cell histiocytosis: a case report and review of the literature. Case Rep Ophthalmol. 2012;3(1):128–35.

    Article  PubMed  Google Scholar 

  58. Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy: a pseudolymphomatous benign disorder. Analysis of 34 cases. Cancer. 1972;30(5):1174–88.

    Article  PubMed  CAS  Google Scholar 

  59. Vemuganti GK, Naik MN, Honavar SG. Rosai Dorfman disease of the orbit. J Hematol Oncol. 2008;1:7.

    Article  PubMed  Google Scholar 

  60. Zimmerman LE, Hidayat AA, Grantham RL, et al. Atypical cases of sinus histiocytosis (Rosai-Dorfman disease) with ophthalmological manifestations. Trans Am Ophthalmol Soc. 1988;86:113–35.

    PubMed  CAS  Google Scholar 

  61. Coupland SE, Foss HD, Assaf C, et al. T-cell and T/natural killer-cell lymphomas involving ocular and ocular adnexal tissues: a clinicopathologic, immunohistochemical, and molecular study of seven cases. Ophthalmology. 1999;106(11):2109–20.

    Article  PubMed  CAS  Google Scholar 

  62. Mendenhall WM, Olivier KR, Lynch Jr JW, Mendenhall NP. Lethal midline granuloma-nasal natural killer/T-cell lymphoma. Am J Clin Oncol. 2006;29(2):202–6.

    Article  PubMed  Google Scholar 

  63. Ruf IK, Rhyne PW, Yang H, et al. Epstein-Barr virus regulates c-MYC, apoptosis, and tumorigenicity in Burkitt lymphoma. Mol Cell Biol. 1999;19(3):1651–60.

    PubMed  CAS  Google Scholar 

  64. Burkitt D. A sarcoma involving the jaws in African children. Br J Surg. 1958;46(197):218–23.

    Article  PubMed  CAS  Google Scholar 

  65. Reifler DM, Warzynski MJ, Blount WR, et al. Orbital lymphoma associated with acquired immune deficiency syndrome (AIDS). Surv Ophthalmol. 1994;38(4):371–80.

    Article  PubMed  CAS  Google Scholar 

  66. Giuliari GP, Sadaka A, Cortez MA, Paniagua A. Orbital Burkitt’s lymphoma: an aggressive presentation. Case Rep Ophthalmol Med. 2012;2012:354043.

    PubMed  Google Scholar 

  67. Baker PS, Gold KG, Lane KA, et al. Orbital Burkitt lymphoma in immunocompetent patients: a report of 3 cases and a review of the literature. Ophthal Plast Reconstr Surg. 2009;25(6):464–8.

    Article  PubMed  Google Scholar 

  68. Le QT, Eulau SM, George TI, et al. Primary radiotherapy for localized orbital MALT lymphoma. Int J Radiat Oncol Biol Phys. 2002;52(3):657–63.

    Article  PubMed  Google Scholar 

  69. Uno T, Isobe K, Shikama N, et al. Radiotherapy for extranodal, marginal zone, B-cell lymphoma of mucosa-associated lymphoid tissue originating in the ocular adnexa: a multiinstitutional, retrospective review of 50 patients. Cancer. 2003;98(4):865–71.

    Article  PubMed  Google Scholar 

  70. Stacy RC, Jakobiec FA, Herwig MC, et al. Diffuse large B-cell lymphoma of the orbit: clinicopathologic, immunohistochemical, and prognostic features of 20 cases. Am J Ophthalmol. 2012;154(1):87–98.

    PubMed  Google Scholar 

  71. Blasi MA, Gherlinzoni F, Calvisi G, et al. Local chemotherapy with interferon-alpha for conjunctival mucosa-associated lymphoid tissue lymphoma: a preliminary report. Ophthalmology. 2001;108(3):559–62.

    Article  PubMed  CAS  Google Scholar 

  72. Sullivan TJ, Grimes D, Bunce I. Monoclonal antibody treatment of orbital lymphoma. Ophthal Plast Reconstr Surg. 2004;20(2):103–6.

    PubMed  Google Scholar 

  73. Abramson DH, Rollins I, Coleman M. Periocular mucosa-associated lymphoid/low grade lymphomas: treatment with antibiotics. Am J Ophthalmol. 2005;140(4):729–30.

    Article  PubMed  CAS  Google Scholar 

  74. Ferreri AJ, Govi S, Pasini E, et al. Chlamydophila psittaci eradication with doxycycline as first-line targeted therapy for ocular adnexae lymphoma: final results of an international phase II trial. J Clin Oncol. 2012;30(24):2988–94.

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Ophthalmic Oncology, Cole Eye Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH, 44195, USA

    Mary E. Aronow MD

  2. Taussig Cancer Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH, 44195, USA

    Brian T. Hill MD, PhD & Arun D. Singh MD

  3. Department of Ophthalmic Oncology, Cole Eye Institute, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH, 44195, USA

    Arun D. Singh MD

Authors
  1. Mary E. Aronow MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Brian T. Hill MD, PhD
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Arun D. Singh MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Arun D. Singh MD .

Editor information

Editors and Affiliations

  1. Division of Opthalmology, Cole Eye Institute Cleveland Clinic Foundation, Cleveland, Ohio, USA

    Julian D. Perry

  2. Department of Ophthalmic Oncology, Cole Eye Institute Cleveland Clinic Foundation, Cleveland, Ohio, USA

    Arun D. Singh

Rights and permissions

Reprints and permissions

Copyright information

© 2014 Springer-Verlag Berlin Heidelberg

About this chapter

Cite this chapter

Aronow, M.E., Hill, B.T., Singh, A.D. (2014). Orbital and Adnexal Lymphoma. In: Perry, J., Singh, A. (eds) Clinical Ophthalmic Oncology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-40492-4_12

Download citation

  • DOI: https://doi.org/10.1007/978-3-642-40492-4_12

  • Published:

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-642-40491-7

  • Online ISBN: 978-3-642-40492-4

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation