Ophthalmic lymphoma: epidemiology and pathogenesis

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.

CT and MRI features of Primary Orbital Lymphoma: review of 14 cases

“…7 Orbital lymphomas can occur at any age, but majority of orbital lymphomas occur around 60 years of age or older, with female predominance. [9][10][11] Clinical manifestations of the orbital lymphomas include swelling and prolapses of the eyelid, painless but palpable masses, and exophthalmia and oculomotor defects.…”

Nasal dissemination of a single-clone IgH-rearranged conjunctival MALT lymphoma through the nasolacrimal duct: A case report

“…Chromosomal abnormalities involving IgH gene translocation, including t(1;14)(p22;q32), t(14;18) (q32;q21), and t(3;14)(p14;q32), are frequently associated with gastric or pulmonary MALT lymphomas but seldom found in conjunctival MALT lymphomas (1,8). In contrast with IgH rearrangement, infectious agents associated with MALT lymphomas are more common including H. pylori, Chlamydia and hepatitis C virus.…”

“…Ophthalmic MALT lymphoma is a malignancy with a indolent course, and neither the clinical stage, ocular region localization, nor therapeutic strategy affects the 5-year survival or progression-free survival rate, with the exception of IgH translocation involvement (1,2). For an IgH-translocated ophthalmic MALT lymphoma, aggressive treatment is necessary due to the high-risk of tumor progression (13).…”

“…For an IgH-translocated ophthalmic MALT lymphoma, aggressive treatment is necessary due to the high-risk of tumor progression (13). MALT lymphomas are sensitive to external beam radiation, chemotherapy, cryotherapy and oral doxycycline for Chlamydia infection (1,9), and the systemic anti-CD20 antibody, rituximab, is reserved for refractory MALT lymphomas (14). In the present case, to avoid radiation-related ocular complications, a topical ciprofloxacin and steroid were chosen to treat the conjunctival MALT lymphoma, and radiotherapy was given covering bilateral neck, left nasal cavity, and left lacrimal sac.…”

“…Ophthalmic lymphomas account for 5-10% of extranodal lymphomas, and extranodal marginal zone B-cell lymphomas of mucosa-associated lymphoid tissue (MALT) are the leading subtype among ophthalmic lymphomas (1,2). However, only a few cases of sinonasal MALT lymphomas were reported in the past 20 years (3,4).…”