Abstract
Among the B-cell tumours of the ocular adnexa, mucosal-associated lymphoid tissue (MALT) lymphomas are considered to be the most common and constitute about 60%. Although these are slow-growing, low-grade tumours, about 50% develop systemic disease. They are also called extranodal marginal zone lymphomas (ENMZL). MALT lymphomas were originally described in lymphoid tissue in the submucosa of the gastrointestinal system, and later research revealed Helicobacter infection as an aetiological factor. In the conjunctiva and the lacrimal gland, there are lymphoid follicles that may be considered mucosal-associated, whereas the orbital soft tissues do not have any lymphoid tissue; and hence, the name ENMZL, and the diagnosis is based on histopathology, immunophenotype, and molecular features. Orbital ENMZL are often painless and can be unilateral or bilateral, solitary or multicentric, with potential to disseminate to lymph nodes or to distant sites.
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Collina F, De Chiara A, De Renzo A, et al. Chlamydia psittaci in ocular adnexa MALT lymphoma: a possible role in lymphomagenesis and a different geographical distribution. Infect Agent Cancer. 2012;7:8.
Ferreri AJ, Guidoboni M, Ponzoni M, et al. Evidence for an association between Chlamydia psittaci and ocular adnexal lymphomas. J Natl Cancer Inst. 2004;96:586–94.
Ferry JA, Fung CY, Zukerberg L, et al. Lymphoma of the ocular adnexa: a study of 353 cases. Am J Surg Pathol. 2007;31:170–84.
Guffey Johnson J, Terpak LA, Margo CE, Setoodeh R. Extranodal marginal zone B-cell lymphoma of the ocular adnexa. Cancer Control. 2016;23(2):140–9.
Nakayama R, Matsumoto Y, Horiike S, et al. Close pathogenetic relationship between ocular immunoglobulin G4-related disease (IgG4-RD) and ocular adnexal mucosa-associated lymphoid tissue (MALT) lymphoma. Leuk Lymphoma. 2014;55:1198–202.
Turbiner Geyer J, Knowles DM. Malignant lymphomas and lymphoid hyperplasias that occur in the ocular adnexa (orbit, conjunctiva and eyelids). In: Hematopathology N, editor. Knowles DM. Philadelphia: Lippincott Williams & Wilkins; 2013. p. 1304.
Uno T, Isobe K, Shikama N, et al. Radiotherapy for extranodal, marginal zone, B-cell lymphoma of mucosa-associated lymphoid tissue originating in the ocular adnexa: a multiinstitutional, retrospective review of 50 patients. Cancer. 2003;98:865–71.
Verdijk RM. An update of ocular adnexal lymphomas. Diagn Histopathol. 2015;21:26–33.
Verdijk RM. Lymphoproliferative tumors of the ocular adnexa. Asia Pac J Ophthalmol (Phila). 2017;6(2):132–42.
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Amrith, S., Young, S.M., Goh, P.S., Wu, B., Nga, M.E., Sundar, G. (2019). MALT Lymphoma. In: Amrith, S., Sundar, G., Young, S. (eds) Ocular Adnexal Lesions. Springer, Singapore. https://doi.org/10.1007/978-981-13-3798-7_35
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DOI: https://doi.org/10.1007/978-981-13-3798-7_35
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