# Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma)

Source: https://onco.cc/cancers/malt-lymphoma/  
OnCo record `malt-lymphoma` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

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).

How 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).

How 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.

Treatment: 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).

## Fields

- Kind: Cancer
- Last checked: 2026-09-24
- Also known as: 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)
- Tags: subtype-page; wave4; haematologic; rare
- 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)

## Sections of this record

The page is a hub with ten sections in reading order; large sections have their own page. The same plan as JSON: https://onco.cc/api/v1/cancers/malt-lymphoma/sections.json

- Overview (on the hub): The TL;DR, the family this cancer belongs to, the organ, who gets it and what the state of the art is. https://onco.cc/cancers/malt-lymphoma/#overview [2 subtypes, 3 state-of-the-art points]
- Types and stages (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/malt-lymphoma/#what-it-is [8 subtypes]
- Symptoms and diagnosis (on the hub): How this cancer shows itself, how the diagnosis is confirmed, and the biomarkers clinicians test for. https://onco.cc/cancers/malt-lymphoma/#finding-it [4 biomarkers]
- Treatment (on the hub): The standard of care by setting, the medicines, surgery and radiotherapy named in it, and the regimens behind them. https://onco.cc/cancers/malt-lymphoma/#treating-it [5 settings, 2 decisions with options]
- Trials and papers (on the hub): Trials recruiting now, the landmark trials, the trials held by this cancer's subtypes, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/malt-lymphoma/#evidence [7 trials, 1 key paper]
- Biology and targets (on the hub): The molecular landscape: the targets and how often each appears, the pathways, the mechanics stages and the preclinical models. https://onco.cc/cancers/malt-lymphoma/#science [10 targets, 2 pathways]
- Countries and centres (own page): Cases by country, the UK and NHS pathway and other country lenses, the expert centres with trials on record, and the centres named on this cancer's subtypes. https://onco.cc/cancers/malt-lymphoma/where-you-are/ [3 centres in the subtypes, 25 UK centres]
- Decisions and support (on the hub): The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask. https://onco.cc/cancers/malt-lymphoma/#living-with-it [15 questions, 6 red cards]
- Pipeline and open problems (own page): Everything in development, the medicines held by this cancer's subtypes, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/malt-lymphoma/coming/ [7 medicines, 2 trials]
- Data (own page): Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked. https://onco.cc/cancers/malt-lymphoma/data/ [79 connected records]

## Standard of care

- Gastric, H. pylori-positive: H. pylori eradication first, with endoscopic follow-up; radiotherapy if the lymphoma persists. ([Marginal zone lymphoma](https://onco.cc/cancers/marginal-zone-lymphoma/))
- Localised at other sites: Involved-site radiotherapy (the FoRT trial tested the dose in follicular and marginal zone lymphoma). ([FoRT](https://onco.cc/trials/fort/), [Marginal zone lymphoma](https://onco.cc/cancers/marginal-zone-lymphoma/))
- Disseminated or relapsed: Rituximab alone or with chlorambucil or bendamustine; zanubrutinib or lenalidomide-rituximab later (AUGMENT; MAHOGANY). ([Rituximab](https://onco.cc/drugs/rituximab/), [Chlorambucil](https://onco.cc/drugs/chlorambucil/), [Bendamustine](https://onco.cc/drugs/bendamustine/), [Zanubrutinib](https://onco.cc/drugs/zanubrutinib/), [Lenalidomide](https://onco.cc/drugs/lenalidomide/), [AUGMENT](https://onco.cc/trials/augment/), [A Study of Zanubrutinib Plus Anti-CD20 Versus Lenalidomide Plus Rituximab in Participants With Relapsed/Refractory Follicular or Marginal Zone Lymphom](https://onco.cc/trials/nct05100862/))
- Gastric MALT lymphoma: a cancer cured by antibiotics: Helicobacter pylori eradication is the first treatment for every Helicobacter-positive gastric MALT lymphoma, whatever the stage, and it is a cure in most of them. The observation that made this possible was published in 1993, when eradication produced regression of the lymphoma in five of six patients, and it remains one of the very few instances in oncology in which a course of antibiotics is the definitive treatment of a malignancy.

What is given: a standard triple or quadruple eradication regimen chosen by local resistance patterns, typically a proton pump inhibitor with two antibiotics for 10 to 14 days. Eradication is then confirmed, by urea breath test or stool antigen at least four weeks after antibiotics and two weeks off the proton pump inhibitor, because failure of eradication is the commonest reason for failure of the lymphoma to respond.

What happens next: endoscopic and histological follow-up every three to six months. Regression is slow and can take twelve to eighteen months, so persistent histological disease in a patient whose Helicobacter has been eradicated and who has no symptoms is watched, not treated. The t(11;18) translocation predicts failure to respond to eradication and is worth testing where it is available. Helicobacter-negative gastric MALT lymphoma is still given eradication therapy in many centres, because some respond, but it is not relied on. ([Regression of primary low-grade gastric MALT lymphoma after eradication of Helicobacter pylori](https://onco.cc/key-papers/paper-wotherspoon-h-pylori-malt-lancet-1993/), [Helicobacter pylori eradication as cancer treatment in gastric MALT lymphoma](https://onco.cc/terms/lymphoma-tx-h-pylori-eradication/), [Watch and wait in lymphoma: when the right treatment is none yet](https://onco.cc/terms/lymphoma-tx-watch-and-wait/), [Endoscopy (EGD, EUS, ERCP)](https://onco.cc/terms/endoscopy/), [Marginal zone lymphomas: ESMO clinical practice guidelines](https://onco.cc/key-papers/paper-esmo-marginal-zone-lymphoma-zucca-ann-oncol-2020/))
- Gastric MALT lymphoma that does not respond to eradication, and MALT at other sites: Where the Helicobacter has been eradicated and the lymphoma persists and is causing symptoms or progressing, radiotherapy is the next treatment: about 24 Gy in 12 fractions to the stomach, which controls the disease in the great majority. Systemic treatment, usually rituximab alone or rituximab with chlorambucil or bendamustine, is used for disseminated disease or where radiotherapy is not possible. Surgery has no routine role.

MALT lymphoma at other sites, salivary gland, thyroid, lung, skin, orbit, breast, is treated on the same principle: a localised, symptomatic site gets 24 Gy, an asymptomatic one can be watched, and disseminated disease gets rituximab-based systemic treatment. Ocular adnexal MALT is sometimes given doxycycline first for Chlamydia psittaci, with response rates that vary widely by region. Salivary gland MALT in Sjogren syndrome and thyroid MALT in Hashimoto thyroiditis are managed jointly with the specialty that treats the underlying autoimmune disease.

The IELSG-19 trial is the randomised evidence for systemic treatment: five-year event-free survival was 68 per cent with chlorambucil and rituximab, 51 per cent with chlorambucil alone and 50 per cent with rituximab alone, with five-year overall survival of about 90 per cent in each arm. ([Radiotherapy in lymphoma: involved-site fields, 24 Gy, 4 Gy and total skin electron therapy](https://onco.cc/terms/lymphoma-tx-radiotherapy/), [Rituximab](https://onco.cc/drugs/rituximab/), [Chlorambucil](https://onco.cc/drugs/chlorambucil/), [Bendamustine](https://onco.cc/drugs/bendamustine/), [IMRT / IGRT (modern external beam)](https://onco.cc/technologies/imrt-igrt/), [Marginal zone lymphomas: ESMO clinical practice guidelines](https://onco.cc/key-papers/paper-esmo-marginal-zone-lymphoma-zucca-ann-oncol-2020/))

## State of the art

- Gastric MALT lymphoma begins as a lymphoma that still needs its antigen. Removing Helicobacter pylori removes the stimulus and most localised cases regress, which makes it the clearest example in oncology of curing a cancer with antibiotics.
- The t(11;18) translocation is what breaks that dependence. It fuses API2 to MALT1 and makes a protein that activates NF-kB on its own, so the lymphoma no longer needs the bacterium. Among 111 patients treated with eradication, 47 of the 48 who regressed completely were negative for the API2-MALT1 transcript, which is why a positive result means eradication alone is not enough whatever the stage.
- The related translocations do the same job by other routes, through BCL10 and MALT1, and inactivation of TNFAIP3 removes the brake on the same pathway. MYD88 L265P appears in 9% of MALT lymphomas, feeding NF-kB from the toll-like receptor side.

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/MALT_lymphoma
- NCI PDQ: adult non-Hodgkin lymphoma treatment: https://www.cancer.gov/types/lymphoma/patient/adult-nhl-treatment-pdq
- Cancer Research UK: types and grades of non-Hodgkin lymphoma: https://www.cancerresearchuk.org/about-cancer/non-hodgkin-lymphoma/types
- Alaggio 2022, Leukemia: the 5th edition WHO classification of haematolymphoid tumours, lymphoid neoplasms: https://doi.org/10.1038/s41375-022-01620-2
- Zucca 2020, Annals of Oncology: marginal zone lymphomas, ESMO clinical practice guidelines: https://doi.org/10.1016/j.annonc.2019.10.010
- Annals of Hematology 2015: antibiotics as sole management of H. pylori-negative gastric MALT lymphoma: https://doi.org/10.1007/s00277-014-2298-3
- Clin Lymphoma Myeloma Leuk 2020: FDG PET in MALT lymphoma, critical review of 32 studies: https://doi.org/10.1016/j.clml.2019.10.006

## Connected records

- cancers: [Diffuse large B-cell lymphoma](https://onco.cc/cancers/dlbcl/), [Gastric & gastro-oesophageal junction cancer](https://onco.cc/cancers/gastric/), [Gastric MALT lymphoma](https://onco.cc/cancers/gastric-malt-lymphoma/), [Marginal zone lymphoma](https://onco.cc/cancers/marginal-zone-lymphoma/), [Micronodular thymoma with lymphoid stroma](https://onco.cc/cancers/micronodular-thymoma/), [Myeloid leukaemia of Down syndrome](https://onco.cc/cancers/myeloid-leukaemia-of-down-syndrome/), [Nodal marginal zone lymphoma](https://onco.cc/cancers/nodal-marginal-zone-lymphoma/), [Ocular adnexal MALT lymphoma](https://onco.cc/cancers/ocular-adnexal-malt-lymphoma/), [Primary cutaneous marginal zone lymphoma](https://onco.cc/cancers/primary-cutaneous-marginal-zone-lymphoma/), [Splenic marginal zone lymphoma](https://onco.cc/cancers/splenic-marginal-zone-lymphoma/)
- terms: [Cancer-related fatigue (tiredness)](https://onco.cc/terms/cancer-related-fatigue/), [Central venous access (port, PICC line)](https://onco.cc/terms/central-venous-access/), [Endoscopy (EGD, EUS, ERCP)](https://onco.cc/terms/endoscopy/), [Febrile neutropenia](https://onco.cc/terms/febrile-neutropenia/), [Financial toxicity](https://onco.cc/terms/financial-toxicity/), [Helicobacter pylori eradication as cancer treatment in gastric MALT lymphoma](https://onco.cc/terms/lymphoma-tx-h-pylori-eradication/), [Hypogammaglobulinaemia and infection risk after B-cell therapies](https://onco.cc/terms/hypogammaglobulinaemia/), [Late effects and survivorship toxicity](https://onco.cc/terms/late-effects/), [Living with an indolent lymphoma rather than being cured of one](https://onco.cc/terms/lymphoma-living-indolent-lymphoma/), [Low antibodies and infection risk for years after anti-CD20 and bispecific antibodies](https://onco.cc/terms/lymphoma-living-infection-years-after/), [MYD88 L265P and CXCR4 mutations](https://onco.cc/terms/myd88-l265p/), [Neutropenia](https://onco.cc/terms/neutropenia/), [Nodal and extranodal lymphoma](https://onco.cc/terms/lymphoma-nodal-versus-extranodal/), [Radiotherapy in lymphoma: involved-site fields, 24 Gy, 4 Gy and total skin electron therapy](https://onco.cc/terms/lymphoma-tx-radiotherapy/), [Staging a lymphoma of the stomach or bowel](https://onco.cc/terms/lymphoma-lugano-gastrointestinal/), [The germinal centre: why lymphoma starts where antibodies are made](https://onco.cc/terms/lymphoma-bio-germinal-centre/), [Watch and wait in follicular lymphoma: being told you have cancer and that nobody will treat it](https://onco.cc/terms/lymphoma-decision-watch-and-wait/), [Watch and wait in lymphoma: when the right treatment is none yet](https://onco.cc/terms/lymphoma-tx-watch-and-wait/), [Watchful waiting, and how it differs from active surveillance](https://onco.cc/terms/watchful-waiting/)
- biomarkers: [Immunoglobulin and T-cell receptor clonality](https://onco.cc/biomarkers/ig-tcr-clonality/)
- technologies: [Cytogenetics and FISH](https://onco.cc/technologies/cytogenetics-fish/), [Early integrated palliative care](https://onco.cc/technologies/palliative-care/), [Exercise during chemotherapy and radiotherapy](https://onco.cc/technologies/exercise-during-chemotherapy/), [Histopathology & immunohistochemistry](https://onco.cc/technologies/histopathology-ihc/), [Immunoglobulin and T-cell receptor clonality testing](https://onco.cc/technologies/clonality-testing/), [IMRT / IGRT (modern external beam)](https://onco.cc/technologies/imrt-igrt/), [Multidisciplinary tumour boards](https://onco.cc/technologies/multidisciplinary-tumour-board/), [Oncofertility and fertility preservation](https://onco.cc/technologies/fertility-preservation/), [Peer support and support groups](https://onco.cc/technologies/peer-support-groups/), [Prehabilitation before cancer surgery](https://onco.cc/technologies/prehabilitation/), [Psycho-oncology and distress screening](https://onco.cc/technologies/psycho-oncology/), [Survivorship care and late-effects surveillance](https://onco.cc/technologies/survivorship-care-plan/)
- targets: [BCL10](https://onco.cc/targets/bcl10/), [BIRC3](https://onco.cc/targets/birc3/), [CD20](https://onco.cc/targets/cd20/), [MALT1](https://onco.cc/targets/malt1/), [MYD88](https://onco.cc/targets/myd88/), [TNFAIP3](https://onco.cc/targets/tnfaip3/)
- drugs: [Bendamustine](https://onco.cc/drugs/bendamustine/), [Chlorambucil](https://onco.cc/drugs/chlorambucil/), [Lenalidomide](https://onco.cc/drugs/lenalidomide/), [Orelabrutinib](https://onco.cc/drugs/orelabrutinib/), [Rituximab](https://onco.cc/drugs/rituximab/), [Umbralisib](https://onco.cc/drugs/umbralisib/), [Zanubrutinib](https://onco.cc/drugs/zanubrutinib/)
- pathways: [Inflammation & NF-κB](https://onco.cc/pathways/inflammation-nfkb/), [Microbiome-tumour interactions](https://onco.cc/pathways/microbiome-tumour/)
- trials: [A Study of Zanubrutinib Plus Anti-CD20 Versus Lenalidomide Plus Rituximab in Participants With Relapsed/Refractory Follicular or Marginal Zone Lymphom](https://onco.cc/trials/nct05100862/), [AUGMENT](https://onco.cc/trials/augment/), [Efficacy and Safety of Orelabrutinib Combined With Rituximab Versus Lenalidomide Combined With Rituximab in Patients With Relapsed/Refractory Marginal Zone Lymphoma](https://onco.cc/trials/nct06082102/), [FoRT](https://onco.cc/trials/fort/), [IELSG-19](https://onco.cc/trials/ielsg-19/), [Mosunetuzumab with lenalidomide in relapsed marginal zone lymphoma](https://onco.cc/trials/mosun-len-mzl/), [ZUMA-5](https://onco.cc/trials/zuma-5/)
- key papers: [Final results of the IELSG-19 randomized trial of mucosa-associated lymphoid tissue lymphoma: improved event-free and progression-free survival with rituximab plus chlorambucil versus either chlorambucil or rituximab monotherapy](https://onco.cc/key-papers/paper-ielsg-19-chlorambucil-rituximab-malt-jco-2017/), [Marginal zone lymphomas: ESMO clinical practice guidelines](https://onco.cc/key-papers/paper-esmo-marginal-zone-lymphoma-zucca-ann-oncol-2020/), [Regression of primary low-grade gastric MALT lymphoma after eradication of Helicobacter pylori](https://onco.cc/key-papers/paper-wotherspoon-h-pylori-malt-lancet-1993/)
- roadmaps: [Lymphoma roadmap: from a jaw tumour in Uganda and the first human cancer virus to gene-expression subtypes, PET-adapted chemotherapy, CAR-T cells, bispecific antibodies and the genetics-directed trials now recruiting](https://onco.cc/roadmaps/lymphoma-roadmap/)

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JSON: https://onco.cc/api/v1/entities/malt-lymphoma.json