Skip to main content
Log in

Comprehensive analysis of orbital lymphoma in a Turkish cohort: clinical characteristics, histological subtypes, treatment modalities, prognostic factors, and implications for management

  • Original Article
  • Published:
Annals of Hematology Aims and scope Submit manuscript

Summary

The study analysed the clinical characteristics, treatment approaches, and survival outcomes of 97 consecutive patients with orbital lymphoma (OL) over a 25-year period at. The median age of the patients was 57.6 years, and 59.8% (n = 58) were male. Marginal zone lymphoma constitutes the most prevalent subtype, accounting for 67% of cases, whereas other common subtypes include diffuse large B-cell lymphoma, follicular lymphoma, mantle cell lymphoma, Burkitt lymphoma, and T-cell lymphomas. Unilateral involvement was observed in the majority of cases (72.3%). Common clinical presentations included mass (30.9%), swelling (26.8%), and epiphora (11.3%). Of the patients, 7.2% received rituximab alone, 14.4% received radiotherapy alone, 48.5% received chemotherapy, 27.8% received radiotherapy plus rituximab, 22.7% received radiotherapy plus chemotherapy, and 5.2% underwent surgery as the first-line treatment. During a median follow-up of 4.3 years, 15.5% of patients experienced relapse or disease progression. The 5-year and 10-year progression-free survival rates were 84.1% and 79.1%, respectively. This study contributes to our understanding of OLs and provides a foundation for further investigations in this field. Male gender, presence of B symptoms, advanced stage, secondary orbital lymphoma, aggressive histological subtype, and elevated serum lactate dehydrogenase levels were associated with poorer (either inferior or worse) progression-free survival.

Graphical abstract

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

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6
Fig. 7
Fig. 8
Algorithm 1

Similar content being viewed by others

Data availability

The data that support the findings of this study are available from the corresponding author upon reasonable request.

References

  1. Olsen TG, Holm F, Mikkelsen LH, Rasmussen PK, Coupland SE, Esmaeli B, Finger PT, Graue GF, Grossniklaus HE, Honavar SG, Khong JJ, McKelvie PA, Mulay K, Sjö LD, Vemuganti GK, Thuro BA, Heegaard S (2019) Orbital lymphoma-an international multicenter retrospective study. Am J Ophthalmol 199:44–57

    Article  PubMed  Google Scholar 

  2. Hsu CR, Chen YY, Yao M, Wei YH, Hsieh YT, Liao SL (2021) Orbital and ocular adnexal lymphoma: a review of epidemiology and prognostic factors in Taiwan. Eye 35(7):1946–1953 (Lond)

    Article  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  4. Kalicińska E, Giza A, Zaucha JM, Giebel S, Zimowska-Curyło D, Andrasiak I, Spychałowicz W, Wojnar J, Balcerzak A, Romejko-Jarosińska J, Paszkiewicz-Kozik E, Knopińska-Posłuszny W, Rybka J, Jabłonowska P, Wróbel T (2023) A survey across orbital lymphoma in Poland: Multicenter retrospective study of polish lymphoma research group (PLRG). Cancer Med 12(3):3036–3045

    Article  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  6. Svendsen FH, Rasmussen PK, Coupland SE, Esmaeli B, Finger PT, Graue GF, Grossniklaus HE, Honavar SG, Khong JJ, McKelvie PA, Mulay K, Ralfkiaer E, Sjö LD, Vemuganti GK, Thuro BA, Curtin J, Heegaard S (2017) Lymphoma of the eyelid - an international multicenter retrospective study. Am J Ophthalmol 177:58–68

    Article  PubMed  Google Scholar 

  7. Tranfa F, Di Matteo G, Strianese D, Forte R, Bonavolontà G (2001) Primary orbital lymphoma. Orbit 20(2):119–124

    Article  PubMed  Google Scholar 

  8. Wierda WG, Byrd JC, Abramson JS, Bilgrami SF, Bociek G, Brander D, Brown J, Chanan-Khan AA, Chavez JC, Coutre SE, Davis RS, Fletcher CD, Hill B, Kahl BS, Kamdar M, Kaplan LD, Khan N, Kipps TJ, Ma S, Malek S, Mato A, Mosse C, Neppalli VT, Shadman M, Siddiqi T, Stephens D, Wagner N, Dwyer MA, Sundar H (2019) NCCN Guidelines Insights: Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma, Version 2.2019. J Natl Compr Canc Netw 17(1):12–20

    Article  CAS  PubMed  Google Scholar 

  9. Bessell EM, Henk JM, Wright JE, Whitelocke RA (1988) Orbital and conjunctival lymphoma treatment and prognosis. Radiother Oncol 13(4):237–244

    Article  CAS  PubMed  Google Scholar 

  10. Collina F, De Chiara A, De Renzo A, De Rosa G, Botti G, Franco R (2012) Chlamydia psittaci in ocular adnexa MALT lymphoma: a possible role in lymphomagenesis and a different geographical distribution. Infect Agent Cancer 7:8

    Article  PubMed  PubMed Central  Google Scholar 

  11. Chanudet E, Zhou Y, Bacon CM, Wotherspoon AC, Müller-Hermelink HK, Adam P, Dong HY, Jong D, Li Y, Wei R, Gong X, Wu Q, Ranaldi R, Goteri G, Pileri SA, Ye H, Hamoudi RA, Liu H, Radford J, Du MQ (2006) Chlamydia psittaci is variably associated with ocular adnexal MALT lymphoma in different geographical regions. J Pathol 209(3):344–351

    Article  CAS  PubMed  Google Scholar 

  12. Min GJ, Kim SE, Kim TY, Jeon YW, Hyun OJ, Choi BO, Park G, Yang SW, Cho SG (2022) Long-Term Clinical Outcomes in Treatment-Naïve Patients With Orbital Adnexal mucosa-associated lymphoid tissue lymphoma: A Single-center study. Front Oncol 12:769530

  13. Savino G, Midena G, Blasi MA, Battendieri R, Grimaldi G, Maceroni M, Tranfa F, Napolitano P, Lanni V, Iuliano A (2020) Orbital and eyelid B-cell lymphoma: a multicenter retrospective study. Cancers (Basel) 12(9):2538.

  14. Oktariana TP, Andriana A, Nugroho RS (2022) The outcome of radiation therapy as a primary treatment in orbital lymphoma: a systematic review. Rep Pract Oncol Radiother 27(4):724–733

    PubMed  PubMed Central  Google Scholar 

  15. Bischof M, Karagiozidis M, Krempien R, Treiber M, Neuhof D, Debus J, Zierhut D (2007) Radiotherapy for orbital lymphoma : outcome and late effects. Strahlenther Onkol 183(1):17–22

    Article  PubMed  Google Scholar 

  16. Alberti W (1997) Acute and late side effects of radiotherapy for ocular disease: an overview. Front Radiat Ther Oncol 30:281–286

    Article  CAS  PubMed  Google Scholar 

Download references

Acknowledgements

The authors thank all faculty members of the Department of Radiation Oncology, Hacettepe University Medical School, for their continuous support in the evaluation and treatment of cases with orbital lymphomas.

Funding

This research did not receive any specific grant from any funding agency in the public, commercial, or not-for-profit sector.

Author information

Authors and Affiliations

Authors

Contributions

Conceptualization, A.A. and H.K.; Formal analysis, A.A.; R.I.; and S.A.; Funding acquisition, H.K.; Investigation, A.A., N.R., M.T., and I.K.; Methodology, A.A., R.I. and S.A.; Project administration, A.A.; Resources, S.A. and H.K.; Supervision, H.K., and I.B; Validation, I.K. and S.A.; Visualization, R.I.; Writing-original draft, A.A.; Writing-review & editing, R.I., S.A., H.K., and I.B. All authors have read and agreed to the published version of the manuscript.

Corresponding author

Correspondence to Rashad Ismayilov.

Ethics declarations

Ethical standards

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Consent to publish

Patients signed informed consent regarding publishing their data and photographs.

Conflict of interest

The authors declare that they have no conflict of interest.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Akyildiz, A., Ismayilov, R., Rustamova, N. et al. Comprehensive analysis of orbital lymphoma in a Turkish cohort: clinical characteristics, histological subtypes, treatment modalities, prognostic factors, and implications for management. Ann Hematol 103, 905–915 (2024). https://doi.org/10.1007/s00277-023-05569-9

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00277-023-05569-9

Keywords

Navigation