{"entity":{"id":"ocular-adnexal-malt-lymphoma","kind":"cancer","name":"Ocular adnexal MALT lymphoma","aka":["Ocular adnexal marginal zone lymphoma","OAMZL","Orbital lymphoma","Conjunctival lymphoma","Lacrimal gland lymphoma","Ocular adnexal lymphoma","Eyelid lymphoma","Salmon patch lymphoma"],"tldr":"A slow-growing MALT lymphoma of the tissues around the eye: the conjunctiva, the eye socket, the tear gland or the eyelid. It usually shows itself as a painless salmon-pink patch on the white of the eye or as an eye that has begun to bulge, and it is controlled in almost everybody, often by a short course of radiotherapy.","summary":"What it is. The tissues around the eye, the conjunctiva that lines the eyelids and covers the white of the eye, the orbit the eye sits in, the tear gland and the eyelids, are collectively the ocular adnexa. A marginal zone lymphoma arising there grows slowly and causes trouble by taking up space rather than by destroying anything. Three-quarters of lymphomas in this location are MALT lymphomas; the rest are follicular lymphoma, diffuse large B-cell lymphoma or others, which is why a biopsy is needed before anything is decided.\n\nHow it shows itself. A flat, salmon-pink patch under the conjunctiva that has been there for months; a painless swelling of the eyelid; an eye that has begun to bulge forward or to sit slightly out of line; double vision; a lump at the outer corner where the tear gland is. Pain, rapid growth and loss of vision are unusual and suggest a different, more aggressive lymphoma. Both sides are involved in a substantial minority, so the other eye is always examined.\n\nHow it differs from its parent and from its sibling in the stomach. WHO-HAEM5 sets out that marginal zone lymphomas differ genetically by the site they arise in, and that ocular adnexal disease has its own profile: mutation or deletion of TNFAIP3 is common, and gain of chromosome 6p with loss of 6q is recurrent here and not at other extranodal sites. The translocation that governs treatment in the stomach, t(11;18), is rare here.\n\nThe bacterium, and the honest version of the story. In Italian studies, the ocular adnexal lymphomas were associated with Chlamydia psittaci, the bacterium of psittacosis, and a course of doxycycline produced lymphoma regression in about two-thirds of patients in prospective trials. That association does not hold everywhere. A United States study tested 28 ocular adnexal lymphoma specimens with two different polymerase chain reaction assays and found no Chlamydia psittaci DNA in any of them, and noted that the association has been reported internationally with great variability. So whether an antibiotic is worth trying depends on where the patient lives and what the local series show, and a reader told that this lymphoma is caused by an infection should ask which population that finding came from.\n\nHow it is treated. For disease confined to the orbit, low-dose radiotherapy is the usual treatment and it is effective. In a series of 28 patients who were given doxycycline first and whose lymphoma relapsed or progressed, salvage radiotherapy at 30 to 36 Gy in 15 to 18 fractions produced a response in every patient and a complete response in 89 per cent, with 4-year progression-free survival of 74 per cent; the recorded toxicity was three cases of grade 2 cataract and three of grade 1 blepharitis, and all but one patient was alive at a median follow-up of 96 months from diagnosis. That paper also answers the question it was written to answer: delaying radiotherapy while an antibiotic is tried did not cost those patients their chance of cure. Very low doses of radiotherapy, around 4 Gy in two fractions, are used in some centres to spare the lens and the tear gland, with retreatment if needed. Disease that has spread beyond the orbit is treated systemically, like any other marginal zone lymphoma.","asOf":"2026-09-29","wikipedia":"https://en.wikipedia.org/wiki/MALT_lymphoma","links":[{"label":"WHO Classification of Haematolymphoid Tumours, 5th edition: lymphoid neoplasms (Alaggio, Leukemia 2022)","url":"https://doi.org/10.1038/s41375-022-01620-2"},{"label":"Salvage irradiation for ocular adnexal MALT lymphoma refractory to Chlamydia psittaci eradication, 28 patients (Advances in Radiation Oncology 2025)","url":"https://doi.org/10.1016/j.adro.2025.101822"},{"label":"Lack of an association between Chlamydia psittaci and ocular adnexal lymphoma, 28 United States specimens tested by two assays (Leukemia and Lymphoma 2007)","url":"https://doi.org/10.1080/10428190601132105"},{"label":"Orbital and ocular adnexal lymphoma, epidemiology and prognostic factors in 112 patients in Taiwan (Eye 2021)","url":"https://doi.org/10.1038/s41433-020-01198-y"},{"label":"Lymphoma incidence, survival and prevalence 2004 to 2014, subtype analyses from the UK Haematological Malignancy Research Network (Smith, Br J Cancer 2015)","url":"https://doi.org/10.1038/bjc.2015.94"}],"tags":["heme","lymphoma","subtype-page"],"related":["malt-lymphoma","marginal-zone-lymphoma","gastric-malt-lymphoma","non-hodgkin-lymphoma","primary-cutaneous-marginal-zone-lymphoma"],"cancers":[],"sections":[],"technologies":["histopathology-ihc","imrt-igrt"],"targets":[],"drugs":["rituximab"],"companies":[],"institutions":[],"pathways":[],"terms":["lymphoma-nodal-versus-extranodal","lymphoma-indolent-versus-aggressive","lymphoma-tx-radiotherapy","lymphoma-tx-watch-and-wait","lugano-classification"],"trials":[],"people":[],"bottlenecks":[],"keyPapers":["paper-esmo-marginal-zone-lymphoma-zucca-ann-oncol-2020"],"journals":[],"dependsOn":[],"notes":[],"group":"haematologic","burden":"No separate incidence figure for the ocular adnexa is published in the United Kingdom population series, which counts all extranodal marginal zone lymphomas together: 211 of 5,796 lymphomas, a European age-standardised rate of 0.60 per 100,000 a year and five-year relative survival of 87.9 per cent across all extranodal sites. In a Taiwanese series of 112 patients with lymphoma of the orbit and ocular adnexa, 67.9 per cent were MALT lymphomas, the mean age was 59 years, and disease-specific survival was 93.1 per cent at five years and 87.7 per cent at ten.","subtypes":["Conjunctival, the salmon-pink patch on the white of the eye","Orbital, an extranodal site behind the eye","Lacrimal gland","Eyelid","Bilateral, which occurs in a substantial minority"],"biomarkers":["A marginal zone phenotype: CD20 positive, CD5 negative, CD10 negative, often with plasmacytic differentiation","TNFAIP3 mutation or deletion, commoner here than at other marginal zone sites","Gain of chromosome 6p and loss of 6q, recurrent in ocular adnexal disease and not at other extranodal sites","Chlamydia psittaci, found in Italian series and absent from several United States series; the association varies by region","Staging of the orbit by magnetic resonance imaging, and of the rest of the body to exclude disease elsewhere"],"standardOfCare":[{"setting":"Diagnosis and staging","approach":"A biopsy of the lesion is needed, because about a third of lymphomas at this site are not marginal zone lymphomas and the treatment differs. Imaging of the orbit, usually by magnetic resonance, maps the extent and the relationship to the optic nerve and the muscles. Staging of the rest of the body follows, because apparently localised ocular disease is sometimes part of a systemic marginal zone lymphoma. Both orbits are examined; bilateral disease is common and does not by itself mean the lymphoma has spread.","refs":["histopathology-ihc","lugano-classification","fdg-pet","lymphoma-nodal-versus-extranodal"],"guideline":{"version":"WHO Classification of Haematolymphoid Tumours, 5th edition (2022), with the International Consensus Classification (2022) where they differ","url":"https://doi.org/10.1038/s41375-022-01620-2"}},{"setting":"Localised disease: radiotherapy, and an antibiotic where the evidence supports it","approach":"Radiotherapy to the orbit is the standard treatment for disease confined to the ocular adnexa and it controls it in almost everybody. In a series of 28 patients irradiated after a trial of doxycycline, 30 to 36 Gy in 15 to 18 fractions produced a response in all of them and a complete response in 89 per cent, with four-year progression-free survival of 74 per cent and little toxicity beyond three cases of grade 2 cataract and three of grade 1 blepharitis. Very low-dose schedules of about 4 Gy in two fractions are used to spare the lens and the tear gland, with retreatment if the lymphoma returns. Where local series have shown an association with Chlamydia psittaci, a course of doxycycline is a reasonable first step, and the same series shows that trying it first did not cost patients their response to radiotherapy afterwards.","refs":["imrt-igrt","lymphoma-tx-radiotherapy","palliative-radiotherapy"],"guideline":{"version":"Advances in Radiation Oncology 2025, salvage irradiation after Chlamydia psittaci eradication; NCI PDQ","url":"https://doi.org/10.1016/j.adro.2025.101822"}},{"setting":"Disease beyond the orbit","approach":"Treated as any other marginal zone lymphoma: watched where it is causing no trouble, and treated with an anti-CD20 antibody alone or with chemotherapy when it is. The regimens, the thresholds for starting and the evidence are on the marginal zone and MALT lymphoma pages and in the treatment layer of this family.","refs":["marginal-zone-lymphoma","malt-lymphoma","rituximab","bendamustine","lymphoma-tx-watch-and-wait","paper-esmo-marginal-zone-lymphoma-zucca-ann-oncol-2020"],"guideline":{"version":"NCI PDQ: adult non-Hodgkin lymphoma treatment","url":"https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq"}}],"stateOfArt":[],"history":[{"year":2004,"title":"Chlamydia psittaci reported in Italian series","note":"Italian investigators reported the bacterium of psittacosis in ocular adnexal marginal zone lymphomas and showed that doxycycline produced regression in a proportion of patients.","refs":[]},{"year":2007,"title":"The association not found in the United States","note":"A United States study tested 28 ocular adnexal lymphoma specimens with two polymerase chain reaction assays and found no Chlamydia psittaci DNA, and noted that the association has been reported internationally with great variability.","refs":[]},{"year":2025,"title":"Trying the antibiotic first does not cost the radiotherapy","note":"In 28 patients whose lymphoma relapsed or progressed after doxycycline, salvage radiotherapy produced a response in every one and a complete response in 89 per cent, with four-year progression-free survival of 74 per cent.","refs":["lymphoma-tx-radiotherapy"]}],"pipeline":[],"openProblems":["Whether Chlamydia psittaci causes this lymphoma depends on where the series was collected, and the question has not been settled in twenty years. A patient in Britain cannot be told with confidence whether an antibiotic is worth trying.","The right radiotherapy dose is unresolved: the conventional 24 to 36 Gy controls the lymphoma and causes cataract and dry eye, while very low doses of about 4 Gy spare both and have not been compared head to head here.","Bilateral disease is common and is sometimes counted as stage IV and sometimes as two localised sites, which changes the treatment a patient is offered."],"parent":"malt-lymphoma"},"route":"/cancers/ocular-adnexal-malt-lymphoma/","neighbours":{"cancer":[{"id":"malt-lymphoma","kind":"cancer","name":"Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma)","route":"/cancers/malt-lymphoma/"},{"id":"gastric-malt-lymphoma","kind":"cancer","name":"Gastric MALT lymphoma","route":"/cancers/gastric-malt-lymphoma/"},{"id":"marginal-zone-lymphoma","kind":"cancer","name":"Marginal zone lymphoma","route":"/cancers/marginal-zone-lymphoma/"},{"id":"non-hodgkin-lymphoma","kind":"cancer","name":"Non-Hodgkin lymphoma (all types)","route":"/cancers/non-hodgkin-lymphoma/"},{"id":"primary-cutaneous-marginal-zone-lymphoma","kind":"cancer","name":"Primary cutaneous marginal zone lymphoma","route":"/cancers/primary-cutaneous-marginal-zone-lymphoma/"}],"technology":[{"id":"fdg-pet","kind":"technology","name":"FDG PET","route":"/technologies/fdg-pet/"},{"id":"histopathology-ihc","kind":"technology","name":"Histopathology & immunohistochemistry","route":"/technologies/histopathology-ihc/"},{"id":"imrt-igrt","kind":"technology","name":"IMRT / IGRT (modern external beam)","route":"/technologies/imrt-igrt/"},{"id":"palliative-radiotherapy","kind":"technology","name":"Palliative radiotherapy","route":"/technologies/palliative-radiotherapy/"}],"drug":[{"id":"bendamustine","kind":"drug","name":"Bendamustine","route":"/drugs/bendamustine/"},{"id":"rituximab","kind":"drug","name":"Rituximab","route":"/drugs/rituximab/"}],"term":[{"id":"lymphoma-indolent-versus-aggressive","kind":"term","name":"Indolent and aggressive lymphoma","route":"/terms/lymphoma-indolent-versus-aggressive/"},{"id":"lugano-classification","kind":"term","name":"Lugano classification / Ann Arbor staging","route":"/terms/lugano-classification/"},{"id":"lymphoma-nodal-versus-extranodal","kind":"term","name":"Nodal and extranodal lymphoma","route":"/terms/lymphoma-nodal-versus-extranodal/"},{"id":"lymphoma-tx-radiotherapy","kind":"term","name":"Radiotherapy in lymphoma: involved-site fields, 24 Gy, 4 Gy and total skin electron therapy","route":"/terms/lymphoma-tx-radiotherapy/"},{"id":"lymphoma-tx-watch-and-wait","kind":"term","name":"Watch and wait in lymphoma: when the right treatment is none yet","route":"/terms/lymphoma-tx-watch-and-wait/"}],"paper":[{"id":"paper-esmo-marginal-zone-lymphoma-zucca-ann-oncol-2020","kind":"paper","name":"Marginal zone lymphomas: ESMO clinical practice guidelines","route":"/key-papers/paper-esmo-marginal-zone-lymphoma-zucca-ann-oncol-2020/"}]}}