{"entity":{"id":"malt-lymphoma","kind":"cancer","name":"Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma)","aka":["MALT lymphoma","Mucosa-associated lymphoid tissue lymphoma","Extranodal marginal zone lymphoma","Extranodal marginal zone lymphoma of MALT","Gastric MALT lymphoma","Ocular adnexal MALT lymphoma","Orbital lymphoma (mostly MALT type)","Extranodal marginal zone lymphoma of MALT (gastric, ocular adnexal, salivary, thyroid, lung, skin)"],"tldr":"MALT lymphoma is a slow-growing lymphoma that starts in lymphoid tissue lining an organ, most often the stomach, where it is usually caused by long-standing Helicobacter pylori infection and can be cured with antibiotics alone. Other sites include the eye socket, salivary glands, thyroid, lung and skin; localised disease is treated with low-dose radiotherapy and widespread disease with rituximab.","summary":"WHO-HAEM5 keeps extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue as the extranodal member of the marginal zone lymphoma family, arising in tissue chronically inflamed by infection or autoimmunity: Helicobacter pylori in the stomach, Sjogren syndrome in the salivary glands, Hashimoto thyroiditis in the thyroid, and Chlamydia psittaci in some ocular adnexal cases (Alaggio 2022; Zucca 2020). The ESMO guideline recommends H. pylori eradication as first treatment for gastric MALT lymphoma whatever the stage, with the t(11;18) translocation predicting failure of antibiotics; even H. pylori-negative gastric disease can respond, with 12 of 13 patients alive and several in remission after antibiotics alone in one centre's series with a median follow-up of 95 months (Annals of Hematology 2015). Whether FDG-PET is useful for staging remains unsettled, with conflicting avidity across 32 studies (Clin Lymphoma Myeloma Leuk 2020).\n\nHow it differs from its parent: the parent page covers the three marginal zone lymphomas together; the extranodal type is the one with an infectious cause to remove, the one treated with involved-site radiotherapy at 24 Gy or less when localised, and the one with organ-specific presentations (gastritis, a salivary mass, an orbital swelling, lung nodules).\n\nHow common: the ESMO guideline gives marginal zone lymphomas as about 5 to 15 percent of non-Hodgkin lymphomas depending on region, with the extranodal type the largest share; no UK figure for the type alone is in the sources read.\n\nTreatment: H. pylori eradication for gastric disease; involved-site radiotherapy for localised disease at other sites; rituximab alone or with chlorambucil or bendamustine for disseminated or relapsed disease; BTK inhibitors (zanubrutinib) and lenalidomide-rituximab as later lines, as on the parent page (Zucca 2020).","asOf":"2026-09-24","wikipedia":"https://en.wikipedia.org/wiki/MALT_lymphoma","links":[{"label":"NCI PDQ: adult non-Hodgkin lymphoma treatment","url":"https://www.cancer.gov/types/lymphoma/patient/adult-nhl-treatment-pdq"},{"label":"Cancer Research UK: types and grades of non-Hodgkin lymphoma","url":"https://www.cancerresearchuk.org/about-cancer/non-hodgkin-lymphoma/types"},{"label":"Alaggio 2022, Leukemia: the 5th edition WHO classification of haematolymphoid tumours, lymphoid neoplasms","url":"https://doi.org/10.1038/s41375-022-01620-2"},{"label":"Zucca 2020, Annals of Oncology: marginal zone lymphomas, ESMO clinical practice guidelines","url":"https://doi.org/10.1016/j.annonc.2019.10.010"},{"label":"Annals of Hematology 2015: antibiotics as sole management of H. pylori-negative gastric MALT lymphoma","url":"https://doi.org/10.1007/s00277-014-2298-3"},{"label":"Clin Lymphoma Myeloma Leuk 2020: FDG PET in MALT lymphoma, critical review of 32 studies","url":"https://doi.org/10.1016/j.clml.2019.10.006"}],"tags":["subtype-page","wave4","haematologic","rare"],"related":["marginal-zone-lymphoma","splenic-marginal-zone-lymphoma","nodal-marginal-zone-lymphoma","primary-cutaneous-marginal-zone-lymphoma","gastric","dlbcl","myeloid-leukaemia-of-down-syndrome"],"cancers":[],"sections":[],"technologies":[],"targets":[],"drugs":["rituximab","chlorambucil","bendamustine","zanubrutinib","lenalidomide","orelabrutinib","umbralisib"],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":["fort","augment","zuma-5","nct05100862","nct06082102"],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"group":"haematologic","burden":"The commonest marginal zone lymphoma; marginal zone lymphomas together are the third most common non-Hodgkin lymphoma group in the ESMO guideline, and the stomach is the most common MALT site (Zucca 2020; Clin Lymphoma Myeloma Leuk 2020). Cancer Research UK lists marginal zone lymphomas among the low-grade types.","subtypes":["Gastric MALT lymphoma (H. pylori-associated; t(11;18) predicts antibiotic failure)","Ocular adnexal (orbital and conjunctival) MALT lymphoma","Salivary gland MALT lymphoma (Sjogren syndrome)","Thyroid MALT lymphoma (Hashimoto thyroiditis)","Pulmonary MALT lymphoma","Extranodal MALT lymphoma with transformation to diffuse large B-cell lymphoma"],"biomarkers":["Helicobacter pylori status (gastric)","t(11;18) API2-MALT1 translocation","Immunophenotype: CD20 positive, CD5 and CD10 negative","FDG avidity (variable)"],"standardOfCare":[{"setting":"Gastric, H. pylori-positive","approach":"H. pylori eradication first, with endoscopic follow-up; radiotherapy if the lymphoma persists.","refs":["marginal-zone-lymphoma"]},{"setting":"Localised at other sites","approach":"Involved-site radiotherapy (the FoRT trial tested the dose in follicular and marginal zone lymphoma).","refs":["fort","marginal-zone-lymphoma"]},{"setting":"Disseminated or relapsed","approach":"Rituximab alone or with chlorambucil or bendamustine; zanubrutinib or lenalidomide-rituximab later (AUGMENT; MAHOGANY).","refs":["rituximab","chlorambucil","bendamustine","zanubrutinib","lenalidomide","augment","nct05100862"]}],"stateOfArt":[],"history":[],"pipeline":[],"openProblems":[],"parent":"marginal-zone-lymphoma"},"route":"/cancers/malt-lymphoma/","neighbours":{"cancer":[{"id":"dlbcl","kind":"cancer","name":"Diffuse large B-cell lymphoma","route":"/cancers/dlbcl/"},{"id":"gastric","kind":"cancer","name":"Gastric & gastro-oesophageal junction cancer","route":"/cancers/gastric/"},{"id":"marginal-zone-lymphoma","kind":"cancer","name":"Marginal zone lymphoma","route":"/cancers/marginal-zone-lymphoma/"},{"id":"micronodular-thymoma","kind":"cancer","name":"Micronodular thymoma with lymphoid stroma","route":"/cancers/micronodular-thymoma/"},{"id":"myeloid-leukaemia-of-down-syndrome","kind":"cancer","name":"Myeloid leukaemia of Down syndrome","route":"/cancers/myeloid-leukaemia-of-down-syndrome/"},{"id":"nodal-marginal-zone-lymphoma","kind":"cancer","name":"Nodal marginal zone lymphoma","route":"/cancers/nodal-marginal-zone-lymphoma/"},{"id":"primary-cutaneous-marginal-zone-lymphoma","kind":"cancer","name":"Primary cutaneous marginal zone lymphoma","route":"/cancers/primary-cutaneous-marginal-zone-lymphoma/"},{"id":"splenic-marginal-zone-lymphoma","kind":"cancer","name":"Splenic marginal zone lymphoma","route":"/cancers/splenic-marginal-zone-lymphoma/"}],"drug":[{"id":"bendamustine","kind":"drug","name":"Bendamustine","route":"/drugs/bendamustine/"},{"id":"chlorambucil","kind":"drug","name":"Chlorambucil","route":"/drugs/chlorambucil/"},{"id":"lenalidomide","kind":"drug","name":"Lenalidomide","route":"/drugs/lenalidomide/"},{"id":"orelabrutinib","kind":"drug","name":"Orelabrutinib","route":"/drugs/orelabrutinib/"},{"id":"rituximab","kind":"drug","name":"Rituximab","route":"/drugs/rituximab/"},{"id":"umbralisib","kind":"drug","name":"Umbralisib","route":"/drugs/umbralisib/"},{"id":"zanubrutinib","kind":"drug","name":"Zanubrutinib","route":"/drugs/zanubrutinib/"}],"trial":[{"id":"nct05100862","kind":"trial","name":"A Study of Zanubrutinib Plus Anti-CD20 Versus Lenalidomide Plus Rituximab in Participants With Relapsed/Refractory Follicular or Marginal Zone Lymphom","route":"/trials/nct05100862/"},{"id":"augment","kind":"trial","name":"AUGMENT","route":"/trials/augment/"},{"id":"nct06082102","kind":"trial","name":"Efficacy and Safety of Orelabrutinib Combined With Rituximab Versus Lenalidomide Combined With Rituximab in Patients With Relapsed/Refractory Marginal Zone Lymphoma","route":"/trials/nct06082102/"},{"id":"fort","kind":"trial","name":"FoRT","route":"/trials/fort/"},{"id":"zuma-5","kind":"trial","name":"ZUMA-5","route":"/trials/zuma-5/"}]}}