Ophthalmic lymphoma: epidemiology and pathogenesis
- 23 January 2009
- journal article
- Published by Wiley in Acta Ophthalmologica
- Vol. 87 (thesis1), 1-20
- https://doi.org/10.1111/j.1755-3768.2008.01478.x
Abstract
With a lifetime risk of 1% and 700 new cases per year, Non-Hodgkin lymphoma (NHL) is the seventh most frequent type of cancer in Denmark. The incidence of NHL has increased considerably in Western countries over the last decades; consequently, NHL is an increasing clinical problem. Ophthalmic lymphoma, (lymphoma localized in the ocular region, i.e. eyelid, conjunctiva, lacrimal sac, lacrimal gland, orbit, or intraocularly) is relatively uncommon, accounting for 5%-10% of all extranodal lymphomas. It is, however, the most common orbital malignancy. The purpose of this thesis was to review specimens from all Danish patients with a diagnosis of ophthalmic lymphoma during the period 1980-2005, in order to determine the distribution of lymphoma subtypes, and the incidence- and time trends in incidence for ophthalmic lymphoma. Furthermore, an extended analysis of the most frequent subtype, extranodal marginal zone lymphoma (MALT lymphoma), was done to analyse clinical factors and cytogenetic changes with influence on prognosis. A total of 228 Danish patients with a biopsy-reviewed verified diagnosis of ocular adnexal-, orbital-, or intraocular lymphoma were identified. We found that more than 50% of orbital- and ocular adnexal lymphomas were of the MALT lymphoma subtype, whereas diffuse large B-cell lymphoma (DLBCL) predominated intraocularly (Sjo et al. 2008a). Furthermore, lymphoma arising in the lacrimal sac was surprisingly predominantly DLBCL (Sjo et al. 2006). Incidence rates were highly dependent on patient age. There was an increase in incidence rates for the whole population from 1980 to 2005, corresponding to an annual average increase of 3.4% (Sjo et al. 2008a). MALT lymphoma arising in the ocular region was found in 116 patients (Sjo et al. 2008b). One third of patients had a relapse or progression of disease after initial therapy and relapses were frequently found at extra-ocular sites. Overall survival, however, was not significantly poorer for patients with relapse. Furthermore, we found that the frequency of translocations involving the MALT1- and IGH-gene loci is low in ocular region MALT lymphoma (2 of 42, 5%), but may predict increased risk of relapse (Sjo et al. 2008b). In conclusion the incidence of ophthalmic lymphoma is increasing at a high rate in Denmark. Ophthalmic lymphoma consists primarily of MALT lymphoma. The molecular pathogenesis of MALT lymphoma arising in the ocular region rarely involves translocations in the MALT1- and IGH-gene loci.Keywords
This publication has 102 references indexed in Scilit:
- Lymphoma dissemination: the other face of lymphocyte homingBlood, 2007
- Clinical impact of genetic aberrations in gastric MALT lymphoma: a comprehensive analysis using interphase fluorescence in situ hybridisationGut, 2007
- MALT lymphoma in patients with autoimmune diseases: a comparative analysis of characteristics and clinical courseLeukemia, 2007
- The pathogenesis of MALT lymphomas: where do we stand?Leukemia, 2007
- Time trends in the registration of Hodgkin and non-Hodgkin lymphomas in EuropeEuropean Journal Of Cancer, 2007
- Expect the unexpectedBritish Journal of Ophthalmology, 2006
- Primary lymphoma of the lacrimal sac: an EORTC ophthalmic oncology task force studyBritish Journal of Ophthalmology, 2006
- Absence of Chlamydia psittaci in ocular adnexal lymphoma from Japanese patientsBritish Journal of Haematology, 2006
- T(3;14)(p14.1;q32) involving IGH and FOXP1 is a novel recurrent chromosomal aberration in MALT lymphomaLeukemia, 2005
- The Conjunctival Lymphoid Follicle in Mucosal ImmunologyDNA and Cell Biology, 2002