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Am J Ophthalmol. 2018 Nov 9. pii: S0002-9394(18)30626-3. doi: 10.1016/j.ajo.2018.11.002. [Epub ahead of print]

Orbital Lymphoma - An International Multicenter Retrospective Study.

Author information

1
Department of Ophthalmology, Rigshospitalet, University of Copenhagen, Denmark.
2
Department of Pathology, Rigshospitalet, University of Copenhagen, Denmark.
3
Department of Ophthalmology, Rigshospitalet, University of Copenhagen, Denmark; Department of Pathology, Rigshospitalet, University of Copenhagen, Denmark.
4
Department of Clinical and Cancer Medicine, University of Liverpool, England.
5
Orbital Oncology and Ophthalmic Plastic Surgery, Department of Plastic Surgery, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA.
6
The New York Eye Cancer Center, New York, New York, USA.
7
Section of Ocular Oncology, Emory Eye Center, Atlanta, Georgia, USA.
8
Department of Ophthalmic and Facial Plastic Surgery, Orbit and Ocular Oncology, Centre for Sight, Hyderabad, India; Department of Ocular Oncology and Oculoplastics, LV Prasad Eye Institute, Hyderabad, India.
9
Orbital, Plastic and Lacrimal Unit, The Royal Victorian Eye and Ear Hospital, Melbourne, Australia.
10
National Reporting Centre for Ophthalmic Pathology, Centre for Sight, Hyderabad, India.
11
Visiting Faculty, Ophthalmic Pathology Services, LV Prasad Eye Institute, Hyderabad, India; Kallam Anji Reddy Campus, School of Medical Sciences, University of Hyderabad, Hyderabad, India.
12
Department of Ophthalmology, Rigshospitalet, University of Copenhagen, Denmark; Department of Pathology, Rigshospitalet, University of Copenhagen, Denmark. Electronic address: sthe@sund.ku.dk.

Abstract

PURPOSE:

To investigate and characterize the clinical features of subtype-specific orbital lymphoma.

DESIGN:

Retrospective, interventional case series.

METHODS:

The study included 7 international eye cancer centers. Patient data were collected from January 1, 1980 through December 31, 2017. A total of 797 patients with a histologically verified orbital lymphoma were included. The primary end points were overall survival, disease-specific survival and progression-free survival.

RESULTS:

The median age was 64 years, and 51% of patients were male. The majority of lymphomas were of B-cell origin (98%, n = 779). Extranodal marginal zone B-cell lymphoma (EMZL) was the most frequent subtype (57%, n = 452), followed by diffuse large B-cell lymphoma (DLBCL) (15%, n = 118), follicular lymphoma (FL) (11%, n = 91), and mantle cell lymphoma (MCL) (8%, n = 66). Localized Ann Arbor stage IE EMZL and FL were frequently treated with external beam radiation therapy. DLBCL, MCL and disseminated EMZL and FL were primarily treated with chemotherapy. EMZL and FL patients had a markedly better prognosis (10-year disease-specific survival of 92% and 71%, respectively) than DLBCL and MCL patients (10-year disease-specific survival of 41% and 32%, respectively).

CONCLUSIONS:

and Relevance: Four lymphoma subtypes were primarily found in patients with orbital lymphoma: EMZL, DLBCL, FL, and MCL. The histological subtype was found to be the main predictor for outcome, with EMZL and FL patients having a markedly better prognosis than DLBCL and MCL.

PMID:
30419193
DOI:
10.1016/j.ajo.2018.11.002

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