Clinicopathological Features of Ocular Adnexal Mantle-Cell Lymphoma in an International Multicenter Cohort | Allergy and Clinical Immunology | JAMA Ophthalmology | JAMA Network
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Original Investigation
December 2017

Clinicopathological Features of Ocular Adnexal Mantle-Cell Lymphoma in an International Multicenter Cohort

Author Affiliations
  • 1Department of Pathology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark
  • 2Department of Cellular and Molecular Pathology, University of Liverpool, Liverpool, England
  • 3Orbital Oncology and Ophthalmic Plastic Surgery, Department of Plastic Surgery, University of Texas MD Anderson Cancer Center, Houston
  • 4The New York Eye Cancer Center, New York
  • 5Section of Ocular Oncology, Emory Eye Center, Atlanta, Georgia
  • 6Orbital, Plastic, and Lacrimal Unit, The Royal Victorian Eye and Ear Hospital, Melbourne, Australia
  • 7National Reporting Centre for Ophthalmic Pathology, Centre for Sight, and Ocular Pathology, LV Prasad Eye Institute, Hyderabad, India
  • 8Visiting Faculty, Ophthalmic Pathology Services, LV Prasad Eye Institute, Hyderabad, India
  • 9School of Medical Sciences, University of Hyderabad, Hyderabad, India
  • 10Department of Ophthalmology, Rigshospitalet-Glostrup, University of Copenhagen, Copenhagen, Denmark
JAMA Ophthalmol. 2017;135(12):1367-1374. doi:10.1001/jamaophthalmol.2017.4810
Key Points

Question  What are the clinical features of ocular adnexal mantle-cell lymphoma (OA-MCL)?

Findings  This retrospective multicenter study included 55 patients, largely elderly and male, with stage IVE OA-MCL involving bilateral tumor masses (frequently affecting the orbit). Five-year disease-specific survival was 38%, which can potentially be improved through the addition of rituximab to the chemotherapy treatment.

Meaning  Ocular adnexal MCL tends to manifest as bilateral tumor masses of the orbit, most often in men; it has an aggressive clinical course with poor prognosis.


Importance  To our knowledge, the clinical features of ocular adnexal mantle-cell lymphoma (OA-MCL) have not previously been evaluated in a large multicenter cohort.

Objective  To characterize the clinical features of OA-MCL.

Design, Setting, and Participants  This retrospective multicenter study included patient data collected from January 1, 1980, through December 31, 2015, at 6 eye cancer centers in 4 countries. Medical records of 55 patients with OA-MCL were reviewed; the median length of follow-up was 33 months.

Main Outcomes and Measures  Overall survival, disease-specific survival, and progression-free survival were the primary end points.

Results  Fifty-five patients were included; ocular adnexal MCL was found to be most common in older individuals (mean age, 70 years) and men (n = 42 of 55; 76%). Patients with OA-MCL frequently presented with disseminated lymphoma (n = 34 of 55; 62%), and were likely to experience stage IVE disease (n = 35 of 55; 64%), with bilateral involvement (n = 27 of 55; 47%), tumor masses (n = 27 of 36; 75%), and involvement of the orbit (n = 32 of 55; 58%). Chemotherapy with or without external beam radiation therapy was the most frequently used treatment. Overall survival rates for the entire cohort were 65% at 3 years (95% CI, 52%-78%) and 34% at 5 years (95% CI, 21%-47%). Disease-specific survival after 5 years was 38% for the entire cohort (95% CI, 25%-51%); the disease-specific survival adjusted by eye cancer center was better in patients who had received rituximab in addition to the chemotherapy regimen (hazard ratio, 3.3; 95% CI, 1.0-14.7; P = .06). The median progression-free survival was 2.3 years (95% CI, 1.8-2.7 years) in patients who experienced recurrence after primary treatment, and 4.1 years (95% CI, 3.9-4.3 years) in patients who presented with a relapse of systemic lymphoma in the ocular adnexal region.

Conclusions and Relevance  These results suggest that the distinctive features of OA-MCL are its appearance in older male individuals, advanced stage and bilateral manifestation at the time of diagnosis, and aggressive course. The prognosis of patients with OA-MCL might be improved by addition of rituximab to chemotherapy treatment.