{"entity":{"id":"gastric-malt-lymphoma","kind":"cancer","name":"Gastric MALT lymphoma","aka":["Gastric mucosa-associated lymphoid tissue lymphoma","Gastric marginal zone lymphoma","Gastric MALToma","Extranodal marginal zone lymphoma of the stomach","Helicobacter pylori-associated gastric lymphoma","Primary gastric lymphoma"],"tldr":"A slow-growing MALT lymphoma that grows in the lining of the stomach, usually caused by a long-standing infection with the bacterium Helicobacter pylori. It is the one lymphoma that is often cured by a fortnight of antibiotics, and a single chromosome change predicts the minority in whom antibiotics will not work.","summary":"What it is. The stomach has no lymphoid tissue of its own. Chronic infection with Helicobacter pylori creates some, as part of the immune response to the bacterium, and over years a clone of B cells in that tissue can become a lymphoma. The lymphoma depends on the signals the infection provides, which is the reason for the treatment that follows.\n\nHow it differs from its parent. Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue arises at many sites and the sites differ genetically: WHO-HAEM5 sets out that ocular adnexal disease commonly carries mutation or deletion of TNFAIP3, salivary gland disease carries recurrent GPR34 mutation, and thyroid disease carries deleterious mutations of CD274, TNFRSF14 or TET2. What the stomach has, and only the stomach and the lung among the common sites, is the translocation t(11;18)(q21;q21), which fuses BIRC3 to MALT1. WHO-HAEM5 states its clinical application in one sentence: that fusion identifies the cases of gastric extranodal marginal zone lymphoma that will not respond to eradication of Helicobacter pylori.\n\nHow it presents and how it is found. Indigestion, pain, nausea, anaemia from slow bleeding, or nothing at all, found when an endoscopy is done for something else. The appearance at endoscopy is often unimpressive: thickened folds, redness, erosions, rather than an obvious tumour. Multiple biopsies are taken from the abnormal area and from each part of the normal-looking stomach, because the lymphoma is patchy. The depth of the lymphoma in the stomach wall is measured by endoscopic ultrasound, and the depth is part of the decision about antibiotics.\n\nThe treatment, and the numbers behind it. Eradicating Helicobacter pylori is the first treatment, and in early disease it is often the only one. In a prospective multicentre trial that followed 120 patients with stage I disease for a median of 75 months, 80 per cent (96 of 120) achieved a complete histological remission, 80 per cent of those remained in continuous remission, 3 per cent relapsed and were referred for other treatment, and five-year survival was 90 per cent. Seventeen per cent of those in remission showed residual lymphoma histologically at some point during follow-up; they were watched rather than treated and all entered a second remission, which is the evidence for not reacting to a single abnormal biopsy. Fifteen per cent of the lymphomas carried t(11;18), and both that translocation and a persisting monoclonal band were associated with a higher risk of not responding or of relapsing, although both were also found in patients who stayed in remission.\n\nWhat follows if antibiotics do not work. Radiotherapy to the stomach at a low dose, or an anti-CD20 antibody, or both, depending on the stage and on whether the translocation is present. Surgery has essentially no role, which is the main thing that separates this disease from gastric adenocarcinoma. Follow-up is by repeated endoscopy and biopsy, and a small number of people develop a gastric carcinoma later, so the endoscopies serve two purposes. The regimens sit in the treatment layer of this family and on the MALT lymphoma page.","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":"Long-term follow-up of gastric MALT lymphoma after Helicobacter pylori eradication, 120 patients with stage I disease (Fischbach, J Clin Oncol 2005)","url":"https://doi.org/10.1200/JCO.2005.02.3903"},{"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"},{"label":"NCI PDQ: adult non-Hodgkin lymphoma treatment (health professional version)","url":"https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq"}],"tags":["heme","lymphoma","subtype-page"],"related":["malt-lymphoma","marginal-zone-lymphoma","ocular-adnexal-malt-lymphoma","gastric","non-hodgkin-lymphoma"],"cancers":[],"sections":[],"technologies":["histopathology-ihc","endoscopic-ultrasound-systems"],"targets":[],"drugs":["rituximab","bendamustine"],"companies":[],"institutions":[],"pathways":[],"terms":["lymphoma-tx-h-pylori-eradication","lymphoma-lugano-gastrointestinal","lymphoma-nodal-versus-extranodal","lymphoma-indolent-versus-aggressive","lymphoma-tx-watch-and-wait","lymphoma-transformation"],"trials":[],"people":[],"bottlenecks":[],"keyPapers":["paper-wotherspoon-h-pylori-malt-lancet-1993","paper-esmo-marginal-zone-lymphoma-zucca-ann-oncol-2020"],"journals":[],"dependsOn":[],"notes":[],"group":"haematologic","burden":"No separate incidence figure for the stomach is published in the United Kingdom population series, which counts all extranodal marginal zone lymphomas together: 211 of 5,796 lymphomas (3.6 per cent), a European age-standardised rate of 0.60 per 100,000 a year, a median age at diagnosis of 68.8 years and five-year relative survival of 87.9 per cent across all extranodal sites. The stomach is the commonest of those sites. The corpus does not quote a gastric-only incidence figure because it could not verify one.","subtypes":["Helicobacter pylori-positive, the great majority","Helicobacter pylori-negative, in which a minority still respond to eradication therapy","With t(11;18)(q21;q21) producing BIRC3::MALT1, which predicts failure of eradication therapy"],"biomarkers":["Helicobacter pylori, by histology, urea breath test, stool antigen or serology; more than one test is used because treatment with acid suppression makes the biopsy unreliable","t(11;18)(q21;q21) giving BIRC3::MALT1, found in about 15 per cent, which predicts that eradication will not work","Depth of invasion of the stomach wall on endoscopic ultrasound, which predicts response to eradication","A monoclonal immunoglobulin heavy chain rearrangement, which often persists after histological remission and does not by itself mean treatment has failed","Trisomy 3 and trisomy 18, common across all marginal zone lymphomas","Large cells on the biopsy, which mean transformation to diffuse large B-cell lymphoma and change the treatment entirely"],"standardOfCare":[{"setting":"Diagnosis and staging","approach":"Endoscopy with multiple biopsies from the abnormal area and from every region of the stomach, because the lymphoma is patchy and a single biopsy can miss it. Helicobacter pylori is sought by more than one method. Endoscopic ultrasound measures how deep the lymphoma goes, which predicts whether antibiotics alone will work; in a prospective comparison against the resected stomach it judged the depth correctly in 91.5 per cent of cases. Fluorescence in situ hybridisation for t(11;18) is done where it will change the plan. Staging uses the gastrointestinal system that counts depth and node involvement rather than the ordinary node-region count.","refs":["endoscopy","endoscopic-ultrasound-systems","lymphoma-lugano-gastrointestinal","histopathology-ihc","paper-wotherspoon-h-pylori-malt-lancet-1993"],"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":"First treatment: eradicate the bacterium","approach":"A standard eradication regimen of a proton pump inhibitor with two antibiotics, chosen by local resistance patterns, is the first treatment for Helicobacter pylori-positive disease and is also offered in negative disease, where a minority still respond. Success is confirmed by a breath or stool test after treatment, and the lymphoma is then followed by repeated endoscopy and biopsy over months, because regression is slow. In the prospective series of 120 patients with stage I disease, 80 per cent achieved complete histological remission and 80 per cent of those remained in continuous remission at a median follow-up of 75 months.","refs":["lymphoma-tx-h-pylori-eradication","endoscopy","lymphoma-tx-watch-and-wait","lymphoma-lugano-gastrointestinal"],"guideline":{"version":"Fischbach, J Clin Oncol 2005; ESMO marginal zone lymphoma guideline; NCI PDQ","url":"https://doi.org/10.1200/JCO.2005.02.3903"}},{"setting":"Residual disease on a follow-up biopsy","approach":"Residual lymphoma on a biopsy after successful eradication is common and usually does not need treatment. In the same series, 17 per cent of those who had reached complete remission later showed histological residual disease; they were watched rather than treated, and all of them entered a second remission. A persisting monoclonal band in the immunoglobulin genes is also common and is not by itself a reason to treat. What does need action is growth, new symptoms, or large cells appearing on the biopsy.","refs":["lymphoma-tx-watch-and-wait","endoscopy","histopathology-ihc"],"guideline":{"version":"Fischbach, J Clin Oncol 2005","url":"https://doi.org/10.1200/JCO.2005.02.3903"}},{"setting":"When antibiotics do not work, or cannot","approach":"Radiotherapy to the stomach at a low dose is the usual next step for disease that stays localised, and anti-CD20 antibody treatment, alone or with chemotherapy, for disease that has spread or that carries t(11;18) and is therefore unlikely to respond to eradication. Surgery has essentially no role at any stage, which is the main difference from gastric cancer. The regimens, the doses and the evidence are on the MALT lymphoma page and in the treatment layer of this family.","refs":["malt-lymphoma","rituximab","lymphoma-tx-radiotherapy","bendamustine","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":1993,"title":"Regression after antibiotics","note":"Wotherspoon and colleagues showed in the Lancet that eradicating Helicobacter pylori made gastric MALT lymphoma regress, the first time an antibiotic had been shown to treat a cancer.","refs":["paper-wotherspoon-h-pylori-malt-lancet-1993","lymphoma-tx-h-pylori-eradication"]},{"year":2005,"title":"Long-term follow-up confirms the remissions hold","note":"In 120 patients with stage I disease followed for a median of 75 months, 80 per cent reached complete histological remission, 80 per cent of those stayed in it, five-year survival was 90 per cent, and 15 per cent of lymphomas carried t(11;18), which predicted failure to respond.","refs":[]},{"year":2022,"title":"The translocation named as the clinical test","note":"WHO-HAEM5 sets out that BIRC3::MALT1 from t(11;18) is recurrent in gastric and pulmonary disease and rare at other sites, and that it identifies the gastric cases that will not respond to eradication.","refs":["lymphoma-classification-2022"]}],"pipeline":[],"openProblems":["Helicobacter pylori resistance to clarithromycin is rising in many countries, and the first treatment for this lymphoma is an antibiotic regimen that is becoming less reliable.","There is no agreed definition of how long to watch residual lymphoma on biopsy before treating it, and the evidence that watching is safe comes from one prospective series.","Nobody knows why a minority of Helicobacter pylori-negative gastric MALT lymphomas respond to eradication therapy anyway."],"parent":"malt-lymphoma"},"route":"/cancers/gastric-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","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":"non-hodgkin-lymphoma","kind":"cancer","name":"Non-Hodgkin lymphoma (all types)","route":"/cancers/non-hodgkin-lymphoma/"},{"id":"ocular-adnexal-malt-lymphoma","kind":"cancer","name":"Ocular adnexal MALT lymphoma","route":"/cancers/ocular-adnexal-malt-lymphoma/"}],"technology":[{"id":"endoscopic-ultrasound-systems","kind":"technology","name":"Endoscopic ultrasound and EBUS systems","route":"/technologies/endoscopic-ultrasound-systems/"},{"id":"histopathology-ihc","kind":"technology","name":"Histopathology & immunohistochemistry","route":"/technologies/histopathology-ihc/"}],"drug":[{"id":"bendamustine","kind":"drug","name":"Bendamustine","route":"/drugs/bendamustine/"},{"id":"rituximab","kind":"drug","name":"Rituximab","route":"/drugs/rituximab/"}],"term":[{"id":"endoscopy","kind":"term","name":"Endoscopy (EGD, EUS, ERCP)","route":"/terms/endoscopy/"},{"id":"lymphoma-tx-h-pylori-eradication","kind":"term","name":"Helicobacter pylori eradication as cancer treatment in gastric MALT lymphoma","route":"/terms/lymphoma-tx-h-pylori-eradication/"},{"id":"lymphoma-indolent-versus-aggressive","kind":"term","name":"Indolent and aggressive lymphoma","route":"/terms/lymphoma-indolent-versus-aggressive/"},{"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-lugano-gastrointestinal","kind":"term","name":"Staging a lymphoma of the stomach or bowel","route":"/terms/lymphoma-lugano-gastrointestinal/"},{"id":"lymphoma-classification-2022","kind":"term","name":"The two lymphoma classifications of 2022 (WHO-HAEM5 and ICC)","route":"/terms/lymphoma-classification-2022/"},{"id":"lymphoma-transformation","kind":"term","name":"Transformation of an indolent lymphoma","route":"/terms/lymphoma-transformation/"},{"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/"},{"id":"paper-wotherspoon-h-pylori-malt-lancet-1993","kind":"paper","name":"Regression of primary low-grade gastric MALT lymphoma after eradication of Helicobacter pylori","route":"/key-papers/paper-wotherspoon-h-pylori-malt-lancet-1993/"}]}}