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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Further Reading
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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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