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Br J Ophthalmol. 2019 Jun 8. pii: bjophthalmol-2019-314008. doi: 10.1136/bjophthalmol-2019-314008. [Epub ahead of print]

International multicentre retrospective cohort study of ocular adnexal marginal zone B-cell lymphoma.

Author information

Department of Ophthalmology, Rigshospitalet Glostrup, Glostrup, Denmark.
Orbital Oncology and Ophthalmic Plastic Surgery, Department of Plastic Surgery, University of Texas M.D. Anderson Cancer Center, Houston, Texas, USA.
Department of Pathology, Rigshospitalet, Copenhagen, Denmark.
Department of Clinical and Cancer Medicine, University of Liverpool, Liverpool, UK.
Ophthalmic Oncology, The New York Eye Cancer Center, New York City, New York, USA.
Section of Ocular Oncology, Emory University Eye Center, Atlanta, Georgia, USA.
Department of Ocular Oncology and Oculoplastics, LV Prasad Eye Institute, Hyderabad, India.
Orbital, Plastic and Lacrimal Unit, The Royal Victorian Eye and Ear Hospital, Melbourne, Victoria, Australia.
Department of Anatomical Pathology, St Vincent's Hospital, Melbourne, Victoria, Australia.
Ophthalmic Pathology, National Reporting Centre for Ophthalmic Pathology, Centre For Sight, Hyderabad, India.
Kallam Anji Reddy Campus, School of Medical Sciences, University of Hyderabad, Hyderabad, India.
Department of Ophthalmology, Rigshospitalet Glostrup, Glostrup, Denmark



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.


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.


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%).


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.


conjunctiva; epidemiology; eye lids; neoplasia; orbit

Conflict of interest statement

Competing interests: None declared.

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