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Frederik Holm, Peter Ramussen, Lauge Hjorth Mikkelsen, Lene Dissing Sjö, Elisabeth Ralfkiaer, Steffen Heegaard; Ocular adnexal lymphoma in Denmark from 1980-2017 – a national study of 372 cases.. Invest. Ophthalmol. Vis. Sci. 2018;59(9):5587.
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© ARVO (1962-2015); The Authors (2016-present)
Ocular adnexal lymphoma(OAL) is rare and information on the subject is limited. In this retrospective observational study we reviewed data on all Danish OAL patients from January 1st 1980 to October 31st 2017 to determine the distribution of subtypes, the incidence of OAL in Denmark, and prognostic factors.
A total of 372 patients with a biopsy-proven OAL were included in this study. Both primary and secondary OAL were included. Cases without a conclusive diagnosis of OAL were excluded. The primary endpoints were overall survival, disease-specific survival(DSS), and progression-free survival.
The incidence of OAL in Denmark was 1.8/million/year and the incidence seems to increase. Most cases were B-cell lymphoma(98%). The major subtypes were extranodal marginal-zone lymphoma(EMZL)(55%), diffuse large B-cell lymphoma(DLBCL)(13%), mantle cell lymphoma(MCL)(11%), and follicular lymphoma(FL)(10%). The median age for OAL was 69(range, 5-96 years). Fifty-one percent were men, where EMZL had a female predilection(1.2:1) and MCL a male predominance(3.4:1). The ocular symptoms were first presenting symptom in 88% of the cases, and the most common symptom was tumor(50%). Dry eye, proptosis, diplopia and decreased vision were common in DLBCL and FL. Irritation/pain was common in DLBCL, FL, and MCL. Increased ocular resistance and epiphora were common in EMZL. DLBCL(98%), FL(92%), and EMZL(87%) presented unilaterally, whereas MCL presented bilateral in 45% of the cases. Sixty-six percent of EMZL were primary, whereas FL(60%) and MCL(90%) were secondary disease. The most common American Joint Committee on Cancer stage was T2(63%). In total 42% of the patients experienced relapse. Especially MCL(60%) and FL(54%) experienced relapse. The median follow-up for OAL was 50 months(range, 0-372 months) and the median time before progression or relapse was 23 months(range, 0-218 months). The 10-year DSS for OAL was 76%. The 10-year DSS was significantly better(p=>0.000) for EMZL(87%) and FL(64%) than DLBCL(47%) and MCL(27%). Factors predicting a poorer prognosis was male gender(p=0.001), secondary disease(p=>0.000), relapse of disease(p=>0.000), and Ann Arbor stage above IE(p=>0.000).
OAL is a rare disease with an incidence of 1.8/million/year, however, the incidence seems to increase. The major subtypes are EMZL, DLBCL, MCL, and FL. EMZL and FL had a favorable prognosis compared to DLBCL and MCL.
This is an abstract that was submitted for the 2018 ARVO Annual Meeting, held in Honolulu, Hawaii, April 29 - May 3, 2018.
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