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Clinical science
Specific location of ocular adnexal lymphoma and mortality: an international multicentre retrospective study
  1. Stine Dahl Vest1,2,
  2. Sarah E. Coupland3,
  3. Bita Esmaeli4,
  4. Paul T. Finger5,
  5. Gerardo F. Graue5,
  6. Hans E. Grossniklaus6,
  7. Tine Gadegaard Hindso2,
  8. Frederik Holm1,
  9. Santosh G. Honavar7,8,
  10. Jwu Jin Khong9,
  11. Marina Knudsen Kirkegaard1,
  12. Penelope A. McKelvie10,
  13. Lauge Hjorth Mikkelsen1,2,
  14. Kaustubh Mulay11,
  15. Peter Kristian Rasmussen2,
  16. Volkert Siersma12,
  17. Lene Dissing Sjö1,
  18. Matthew C. Sniegowski4,
  19. Bradley A. Thuro4,
  20. Geeta K. Vemuganti13,14,
  21. Steffen Heegaard1,2
  1. 1 Eye Pathology Section, Department of Pathology, Rigshospitalet, Copenhagen, Denmark
  2. 2 Department of Ophthalmology, Rigshospitalet, Copenhagen, Denmark
  3. 3 Department of Cellular and Molecular Pathology, University of Liverpool, Liverpool, UK
  4. 4 Orbital Oncology and Ophthalmic Plastic Surgery, Department of Plastic Surgery, University of Texas M.D. Anderson Cancer Center, Houston, Texas, USA
  5. 5 The New York Eye Cancer Center, New York City, New York, USA
  6. 6 Section of Ocular Oncology, Emory University Eye Center, Atlanta, Georgia, USA
  7. 7 Department of Ocular Oncology and Oculoplastics, LV Prasad Eye Institute, Hyderabad, India
  8. 8 Department of Ophthalmic and Facial Plastic Surgery, Orbit and Ocular Oncology, Centre for Sight, Hyderabad, India
  9. 9 Orbital, Plastic, and Lacrimal Unit, Royal Victorian Eye and Ear Hospital, East Melbourne, Victoria, Australia
  10. 10 Department of Anatomical Pathology, St Vincent's Hospital, Melbourne, Victoria, Australia
  11. 11 National Reporting Centre for Ophthalmic Pathology, Centre for Sight, Hyderabad, India
  12. 12 The Research Unit for General Practice and Section of General Practice, Department of Public Health, University of Copenhagen Faculty of Health Sciences, Copenhagen, Denmark
  13. 13 Kallam Anji Reddy Campus, School of Medical Sciences, University of Hyderabad, Hyderabad, India
  14. 14 Visiting Faculty, Ophthalmic Pathology Services, LV Prasad Eye Institute, Hyderabad, India
  1. Correspondence to Professor Steffen Heegaard, Eye Pathology Section, Department of Pathology, Rigshospitalet, Copenhagen, Denmark; sthe{at}sund.ku.dk

Abstract

Aims To examine whether the specific location of ocular adnexal lymphoma (OAL) and the American Joint Committee on Cancer (AJCC) TNM tumour stage are prognostic factors for mortality in the main OAL subtypes.

Methods Clinical and survival data were retrospectively collected from seven international eye cancer centres. All patients from 1980 to 2017 with histologically verified primary or secondary OAL were included. Cox regression was used to compare the ocular adnexal tumour locations on all-cause mortality and disease-specific mortality.

Results OAL was identified in 1168 patients. The most frequent lymphoma subtypes were extranodal marginal zone B-cell lymphoma (EMZL) (n=688, 59%); follicular lymphoma (FL) (n=150, 13%); diffuse large B-cell lymphoma (DLBCL) (n=131, 11%); and mantle cell lymphoma (MCL) (n=89, 8%). AJCC/TNM tumour-stage (T-stage) was significantly associated with disease-specific mortality in primary ocular adnexal EMZL and increased through T-categories from T1 to T3 disease. No associations between AJCC/TNM T-stage and mortality were found in primary ocular adnexal FL, DLBCL, or MCL. EMZL located in the eyelid had a significantly increased disease-specific mortality compared with orbital and conjunctival EMZL, in both primary EMZL and the full EMZL cohort. In DLBCL, eyelid location had a significantly higher disease-specific mortality compared with an orbital or lacrimal gland location.

Conclusion Disease-specific mortality is associated with AJCC/TNM T-stage in primary ocular adnexal EMZL patients. Lymphoma of the eyelid has the highest disease-specific mortality in primary EMZL, and in the full cohort of EMZL and DLBCL patients.

  • conjunctiva
  • eye lids
  • lacrimal gland
  • neoplasia
  • orbit

Data availability statement

Data are available on reasonable request.

https://doi.org/10.1136/bjophthalmol-2021-320466

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    Data availability statement

    Data are available on reasonable request.

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    Footnotes

    • Twitter @#paulfingermd, @jjkhong, @HjorthLauge

    • Contributors Study concept and design: SDV, PKR, SEC, BE and SH. Acquisition, analysis or interpretation of data: SDV, SEC, BE, PTF, GG, HEG, TGH, FH, SGH, JJK, MKK, PAM, LHM, KM, PKR, VDS, LDS, MCS, BT, GV and SH. Drafting of manuscript: SDV, SEC, LHM, VDS and SH. Critical revision of the manuscript for important intellectual content: SDV, SEC, BE, PTF, GG, HEG, TGH, FH, SGH, JJK, MKK, PAM, LHM, KM, PKR, VDS, LDS, MCS, BT, GV and SH. Statistical analysis: SDV, VDS. Obtained funding: SH. Administrative, technical or material support: SDV, SEC, BE, PTF, GG, HEG, TGH, FH, SGH, JJK, MKK, PAM, LHM, KM, PKR, VDS, LDS, MCS, BT, GV and SH. Study supervision: FH, LHHM, PKR, VDS and SH.

      SDV, SH had full access to the data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis.

    • Funding The Eye Cancer Foundation supported this study with an unrestricted fellowship grant for Gerardo Graue with grant number GG1.

    • Competing interests None declared.

    • Provenance and peer review Not commissioned; externally peer reviewed.

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