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Original Investigation
July 2014

Ocular Adnexal Follicular Lymphoma: A Multicenter International Study

Author Affiliations
  • 1Eye Pathology Institute, Department of Neuroscience and Pharmacology, University of Copenhagen, Copenhagen, Denmark
  • 2Department of Cellular and Molecular Pathology, University of Liverpool, Liverpool, United Kingdom
  • 3The New York Eye Cancer Center, New York
  • 4Section of Ocular Oncology, Emory Eye Center, Atlanta, Georgia
  • 5Kallam Anji Reddy Campus, L. V. Prasad Eye Institute, Hyderabad, India
  • 6The Orbital Plastic and Lacrimal Clinic, The Royal Victorian Eye and Ear Hospital, Melbourne, Australia
  • 7Department of Pathology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark
  • 8Department of Ophthalmology, Glostrup University Hospital, Glostrup, Denmark
JAMA Ophthalmol. 2014;132(7):851-858. doi:10.1001/jamaophthalmol.2014.376

Importance  The clinical features of the follicular subtype of ocular adnexal lymphoma (OAL) have not been previously evaluated in a large cohort.

Objective  To characterize the clinical features of follicular OAL.

Design, Setting, and Participants  We performed a retrospective multicenter study that involved 6 eye cancer centers from January 1, 1980, through December 31, 2010. A total of 105 patients with follicular OAL were identified, of which 7 patients were excluded because of missing clinical data. The median follow-up time was 52 months (range, 13-118 months).

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

Results  Ninety-eight eligible patients with follicular OAL were included; 60 (61%) were women. The median patient age was 63 years (range, 32–96 years). Sixty-nine patients (70%) had primary OAL, 19 (19%) had OAL in conjunction with a concurrent systemic lymphoma, and 10 (10%) presented with an ocular adnexal relapse. The lacrimal gland (28%), conjunctiva (28%), and orbit (28%) were the most frequently involved sites. Of the 69 patients with primary follicular lymphoma, 38 (55%) presented with Ann Arbor stage IE lymphoma, and 31 (45%) had stage IIE lymphoma. Patients with disseminated lymphoma had stage IIIE (9 of 19 [47%]) and stage IV (10 of 19 [53%]) disease, whereas patients with a relapse of systemic lymphoma presented with stage IE (8 of 10 [80%]), stage IIE (1 of 10 [10%]), and stage IIIE (1 of 10 [10%]) disease. Patients with primary follicular lymphoma (n = 69) and those with isolated ocular relapse (n = 9) were treated with external beam radiation therapy (EBRT) (35 of 78 [45%]) or EBRT plus chemotherapy (22 of 78 [28%]). Patients presenting with stage IIIE-IV follicular lymphoma (n = 20) most frequently received chemotherapy (9 of 20 [45%]) or EBRT plus chemotherapy (4 of 20 [20%]). The 10-year overall survival for the entire study cohort was 60%. Primary patients treated with EBRT had a better disease-specific survival compared with patients receiving ERBT plus chemotherapy (10-year disease-specific survival, 94% vs 40%; P = .02 by log-rank test).

Conclusions and Relevance  Follicular OAL was more commonly found in elderly female patients. These tumors were equally noted to involve the conjunctiva, lacrimal gland, and orbit. Patients with ocular adnexal follicular lymphoma primarily treated with EBRT had a more favorable long-term disease-specific survival.