TY - JOUR

T1 - Ocular adnexal lymphoma in Denmark

T2 - a nationwide study of 387 cases from 1980 to 2017

AU - Holm, Frederik

AU - Mikkelsen, Lauge Hjorth

AU - Kamper, Peter

AU - Rasmussen, Peter Kristian

AU - Larsen, Thomas Stauffer

AU - Sjö, Lene Dissing

AU - Heegaard, Steffen

N1 - © Author(s) (or their employer(s)) 2020. No commercial re-use. See rights and permissions. Published by BMJ.

PY - 2021

Y1 - 2021

N2 - BACKGROUND: Nationwide studies of ocular adnexal lymphoma (OAL) are very rare in the literature, and knowledge on incidence, subtype distribution and long-term survival data is limited. This is the largest national study of OAL to date. This study sought to find information on incidence, changes in incidence, clinical findings, distribution of subtypes, survival and prognostic factors.METHODS: Patients diagnosed with OAL from January 1, 1980 to December 31, 2017 were identified in Danish registers, and clinical as well as survival data were collected. The data were analysed with Kaplan-Meier plots and log-rank test.RESULTS: 387 patients were included in the study. The major lymphoma subtypes were extranodal marginal-zone B cell lymphoma (EMZL) (55%), diffuse large B cell lymphoma (DLBCL) (13%), mantle cell lymphoma (MCL) (11%) and follicular lymphoma (FL) (10%). OAL is a disease of the elderly (median age 69 years). The incidence of lymphoma of the ocular adnexal region has increased significantly throughout the time period of the study (Pearson correlation coefficient, r=0.65; P<0.001). In the period 1980-1984, the incidence was 0.086 per 100 000, which increased to 0.307 per 100 000 in the period 2013-2017. Low-grade, low-stage primary lymphomas were treated with radiotherapy, whereas patients with high-stage, high-grade and/or relapsed disease were treated with chemotherapy. Low-grade subtypes EMZL (89%) and FL (56%) had better 10-year disease-specific survival than the high-grade lymphomas DLBCL (38%) and MCL (31%)(p<0.001).CONCLUSION: OAL is increasing in incidence in the Danish population for unknown reasons. However, the prognosis for most OAL is favourable, as highlighted in this national long-term study.

AB - BACKGROUND: Nationwide studies of ocular adnexal lymphoma (OAL) are very rare in the literature, and knowledge on incidence, subtype distribution and long-term survival data is limited. This is the largest national study of OAL to date. This study sought to find information on incidence, changes in incidence, clinical findings, distribution of subtypes, survival and prognostic factors.METHODS: Patients diagnosed with OAL from January 1, 1980 to December 31, 2017 were identified in Danish registers, and clinical as well as survival data were collected. The data were analysed with Kaplan-Meier plots and log-rank test.RESULTS: 387 patients were included in the study. The major lymphoma subtypes were extranodal marginal-zone B cell lymphoma (EMZL) (55%), diffuse large B cell lymphoma (DLBCL) (13%), mantle cell lymphoma (MCL) (11%) and follicular lymphoma (FL) (10%). OAL is a disease of the elderly (median age 69 years). The incidence of lymphoma of the ocular adnexal region has increased significantly throughout the time period of the study (Pearson correlation coefficient, r=0.65; P<0.001). In the period 1980-1984, the incidence was 0.086 per 100 000, which increased to 0.307 per 100 000 in the period 2013-2017. Low-grade, low-stage primary lymphomas were treated with radiotherapy, whereas patients with high-stage, high-grade and/or relapsed disease were treated with chemotherapy. Low-grade subtypes EMZL (89%) and FL (56%) had better 10-year disease-specific survival than the high-grade lymphomas DLBCL (38%) and MCL (31%)(p<0.001).CONCLUSION: OAL is increasing in incidence in the Danish population for unknown reasons. However, the prognosis for most OAL is favourable, as highlighted in this national long-term study.

U2 - 10.1136/bjophthalmol-2019-315637

DO - 10.1136/bjophthalmol-2019-315637

M3 - Journal article

C2 - 32732342

VL - 105

SP - 914

EP - 920

JO - British Journal of Ophthalmology

JF - British Journal of Ophthalmology

SN - 0007-1161

IS - 7

ER -