# 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
- What it is (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 [6 subtypes]
- Finding it (on the hub): How it shows itself, how it is confirmed, what screening exists, and the biomarkers clinicians test for. https://onco.cc/cancers/malt-lymphoma/#finding-it [4 biomarkers]
- Treating it (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 [3 settings, 1 decision with options]
- Evidence (on the hub): Trials recruiting now, the landmark trials, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/malt-lymphoma/#evidence [5 trials]
- The science (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 [5 targets]
- Where you are (own page): Cases by country, the UK and NHS pathway and other country lenses, and the expert centres with trials on record. https://onco.cc/cancers/malt-lymphoma/where-you-are/
- Living with it (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 [12 questions, 6 red cards]
- What is coming (own page): Everything in development, 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]
- 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/ [33 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/))

## 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/), [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/), [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/)
- 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/)
- 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/), [ZUMA-5](https://onco.cc/trials/zuma-5/)

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