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International multicentre retrospective cohort study of ocular adnexal marginal zone B-cell lymphoma
  1. Tine Gadegaard Hindsø1,
  2. Bita Esmaeli2,
  3. Frederik Holm3,
  4. Lauge Hjorth Mikkelsen1,3,
  5. Peter Kristian Rasmussen1,
  6. Sarah E Coupland4,
  7. Paul T Finger5,
  8. Gerardo F Graue5,
  9. Hans E Grossniklaus6,
  10. Santosh G Honavar7,
  11. Jwu Jin Khong8,
  12. Penelope A McKelvie9,
  13. Kaustubh Mulay10,
  14. Lene Dissing Sjö3,
  15. Geeta K Vemuganti11,
  16. Bradley A Thuro2,
  17. Steffen Heegaard1,3
  1. 1 Department of Ophthalmology, Rigshospitalet Glostrup, Glostrup, Denmark
  2. 2 Orbital Oncology and Ophthalmic Plastic Surgery, Department of Plastic Surgery, University of Texas M.D. Anderson Cancer Center, Houston, Texas, USA
  3. 3 Department of Pathology, Rigshospitalet, Copenhagen, Denmark
  4. 4 Department of Clinical and Cancer Medicine, University of Liverpool, Liverpool, UK
  5. 5 Ophthalmic Oncology, 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 Orbital, Plastic and Lacrimal Unit, The Royal Victorian Eye and Ear Hospital, Melbourne, Victoria, Australia
  9. 9 Department of Anatomical Pathology, St Vincent's Hospital, Melbourne, Victoria, Australia
  10. 10 Ophthalmic Pathology, National Reporting Centre for Ophthalmic Pathology, Centre For Sight, Hyderabad, India
  11. 11 Kallam Anji Reddy Campus, School of Medical Sciences, University of Hyderabad, Hyderabad, India
  1. Correspondence to Professor Steffen Heegaard, Department of Ophthalmology, Rigshospitalet Glostrup, Glostrup DK-2100, Denmark; sthe{at}


Backgrounds/aims To date, this is the largest cohort study on extranodal marginal zone B-cell lymphoma (EMZL) of the ocular adnexa (OA). The aim of the study was to characterise the clinical features of OA-EMZL.

Methods A retrospective multicentre study involving seven international eye cancer centres. Data were collected from 1 January 1980 through 31 December 2017. A total of 689 patients with OA-EMZL were included.

Results The median follow-up time was 42 months. The median age was 62 years (range, 8–100 years), and 55 % (378/689 patients) of patients were women. The majority of patients (82%, 558/680 patients) were diagnosed with primary OA-EMZL with Ann Arbor stage IE (90%, 485/541 patients) and American Joint Committee on Cancer stage T2 (61%, 340/557 patients) at the time of diagnosis. The orbit (66%, 452/689 patients) and the conjunctiva (37%, 255/689 patients) were the most frequently involved anatomical structures. The 5-year, 10-year and 20-year disease-specific survival (DSS) were 96%, 91% and 90%, respectively. Stage IE patients treated with external beam radiation therapy (EBRT) as monotherapy (10-year DSS, 95%) were found to have a better DSS than stage IE patients treated with chemotherapy (10-year DSS, 86%). Stage IIIE/IVE patients treated with chemotherapy and rituximab had a better DSS (10-year DSS, 96%) than stage IIIE/IVE patients treated with chemotherapy without rituximab (10-year DSS, 63%).

Conclusions and relevance EMZL is a slow-growing tumour with an excellent long-term survival. Low-dose EBRT as monotherapy should be considered in localised OA-EMZL. Rituximab-based chemotherapy should be chosen in those patients with disseminated disease.

  • orbit
  • neoplasia
  • epidemiology
  • conjunctiva
  • eye lids

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  • Contributors TGH, FH, PKR, SH had full access to all the data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis. Study concept and design: TGH, PKR, SEC, BE, SH. Acquisition, analysis, or interpretation of data: TGH, FH, LHM, PKR, SEC, BE, PTF, GG, HEG, SGH, JJK, PAM, KM, LDS, GV, BT, SH. Drafting of manuscript: TGH, FH, BE, SEC, SH. Critical revision of the manuscript for important intellectual content: FH, LHM, PKR, SEC, GG, PTF, HEG, SGH, JJK, PAM, KM, LDS, GV, BT, SH. Statistical analysis: TGH, FH. Obtained funding: TGH, SH, LHM. Administrative, technical, or material support: FH, LHM, SEC, BE, PTF, GG, HEG, JJK, KM, LDS, BT. Study supervision: FH, LHM, BE, SGH, GV, SH.

  • Funding This study was supported by grants from Synoptik-Fonden, Copenhagen, Denmark, and Fight for Sight Denmark, Copenhagen, Denmark. MD Lauge Hjorth Mikkelsen was supported by The Candy Foundation.

  • Disclaimer The funding sources played no role in the design of the study; collection, analysis, and interpretation of data; in writing of the report; or in the decision to submit the article for publication.

  • Competing interests None declared.

  • Patient consent for publication Obtained.

  • Ethics approval The study followed the tenets of the Declaration of Helsinki and the Health Insurance Portability and Accountability Act of 1996 in the USA. Institutional review board and health information privacy agency approval was obtained from the Danish Data Protection Agency and the Local Ethics Committee (J no. H-B-2009-054).

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

  • Data availability statement Data are available upon request.

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