Marginal zone lymphoma
Marginal zone lymphoma is a slow B-cell lymphoma that often grows where the body has been fighting a chronic infection: the stomach with Helicobacter pylori, the eye, the skin or the spleen. Curing the infection cures many early cases; the rest are treated with rituximab, chemotherapy or BTK inhibitors.
Overview
Marginal zone lymphomas come in three forms. Extranodal (MALT) lymphoma arises in mucosal tissue chronically stimulated by infection or autoimmunity: gastric MALT lymphoma from Helicobacter pylori, ocular adnexal from Chlamydia in some regions, salivary gland in Sjogren's syndrome and thyroid in Hashimoto's disease; eradicating H. pylori puts most early gastric cases into remission, and localised disease elsewhere is cured with low-dose radiotherapy. Splenic marginal zone lymphoma presents with a large spleen and circulating villous lymphocytes and is linked to hepatitis C, whose treatment can induce remission; rituximab has replaced splenectomy. Nodal marginal zone lymphoma behaves like follicular lymphoma. Systemic treatment when needed is rituximab alone or with bendamustine or chlorambucil, lenalidomide-rituximab, and the BTK inhibitors ibrutinib and zanubrutinib for relapsed disease.
State of the art
- Gastric MALT lymphoma is the clearest example of a cancer cured by treating an infection.
- Very low-dose radiotherapy controls localised disease with almost no side effects.
- BTK inhibitors give durable responses in relapsed disease with zanubrutinib's approval in 2021.
Anatomy and lymph node drainage
Leukaemias, myeloma and MDS live in the marrow and blood; lymphomas grow in lymph nodes and spleen. The node stations are the disease map, not a route of spread, and staging counts them.
- Bone marrow (leukaemia, MDS, MPN, myeloma)
- Lymph node germinal centre (lymphomas)
- SpleenExtranodal marginal zone lymphoma of MALT (gastric, ocular adnexal, salivary, thyroid, lung, skin) · Splenic marginal zone lymphoma · Nodal marginal zone lymphoma · Paediatric nodal marginal zone lymphoma (indolent)
- Blood (leukaemic phase)
- Lytic bone lesions (myeloma)
- Skin and extranodal sitesExtranodal marginal zone lymphoma of MALT (gastric, ocular adnexal, salivary, thyroid, lung, skin)
- cervical
- axillary
- mediastinal
- para-aortic and mesenteric
- inguinal
In lymphoma the node stations are the disease itself; staging (Ann Arbor / Lugano) counts how many regions and sides of the diaphragm are involved.
Same organ: Cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome), Leukaemia (all types), Acute myeloid leukaemia, Acute lymphoblastic leukaemia, Chronic lymphocytic leukaemia, Chronic myeloid leukaemia (CML), Diffuse large B-cell lymphoma, Follicular lymphoma, Hodgkin lymphoma, Mantle cell lymphoma, Multiple myeloma, Non-Hodgkin lymphoma (all types), Myelodysplastic syndromes / neoplasms (MDS), Myeloproliferative neoplasms (PV, ET, myelofibrosis), Polycythaemia vera (PV), Essential thrombocythaemia (ET), Waldenström macroglobulinaemia, Hairy cell leukaemia, Peripheral T-cell lymphomas (including cutaneous T-cell lymphoma), Blastic plasmacytoid dendritic cell neoplasm (BPDCN), Burkitt lymphoma, HIV-associated (AIDS-related) lymphomas, Chronic myelomonocytic leukaemia and MDS/MPN overlap neoplasms, Systemic mastocytosis, Erdheim-Chester disease, Rosai-Dorfman disease and other histiocytic neoplasms, Langerhans cell histiocytosis (LCH), Post-transplant lymphoproliferative disorder (PTLD)
About one in ten non-Hodgkin lymphomas, the second commonest indolent type after follicular lymphoma; most patients live for many years and many are cured by treating the infection or inflammation that drove the disease.
- AI in radiologyEstablished
- cfDNA fragmentomicsEstablished
- Colorectal cancer screening (colonoscopy, FIT, stool DNA, blood)Standard of care
- DNA methylation profilingEstablished
- HCC surveillance in cirrhosis (ultrasound + AFP)Standard of care
- High-risk pancreatic surveillance (CAPS / PRECEDE)Established
- A 28-day national pathway for people with a positive multi-cancer blood test
- A breath test to rule out cancer in people with vague symptoms
- A cancer blood test for older people arriving at A&E with unexplained symptoms
- A legislated, publicly reported 28-day standard from urgent referral to diagnosis
- A live national dashboard of stage at diagnosis as the scorecard for early detection
- A single 'cancer check at 60' appointment bundling all screening tests
Background: Alpha-fetoprotein (AFP), Barrett's oesophagus, CA 19-9, Early detection, Faecal immunochemical test (FIT). Also on OnCo: Symptoms and red flags · Early detection roadmap.
Cases by country
No country-level case numbers. This cancer is not mapped to a GLOBOCAN site.
Eradication therapy and endoscopic follow-up; radiotherapy if the lymphoma persists or carries t(11;18).
Low-dose involved-site radiotherapy (as little as 4 Gy in two fractions for some sites); surgery rarely.
Watch and wait if asymptomatic; antiviral therapy if hepatitis C-positive; rituximab alone or with chemotherapy; splenectomy now rare.
Rituximab with bendamustine or chlorambucil, lenalidomide-rituximab; zanubrutinib or ibrutinib for relapsed disease.
Subtypes & biomarkers
top- Extranodal marginal zone lymphoma of MALT (gastric, ocular adnexal, salivary, thyroid, lung, skin)
- Splenic marginal zone lymphoma
- Nodal marginal zone lymphoma
- Paediatric nodal marginal zone lymphoma (indolent)
- Helicobacter pylori status (gastric MALT)
- t (11;18) MALT1 translocation (predicts failure of eradication therapy)
- Hepatitis C serology (splenic)
- MYD88 wild-type (distinguishes from Waldenstrom)
- Plasmacytic differentiation and paraprotein
How often this target appears
- 1983Isaacson and Wright describe MALT lymphoma
- 1993H. pylori eradication induces regression of gastric MALT lymphoma (Wotherspoon)
- 2021Zanubrutinib approved for relapsed marginal zone lymphoma
What is in development for Marginal zone lymphoma, drawn from the whole corpus: 0 items. Drugs are grouped by the most advanced trial phase they have reached anywhere; approved treatments sit under standard of care. Technologies are the methods being tested for this cancer, trials are the studies recorded here, and ideas are proposals not yet in a trial.
Nothing recorded in development for this cancer yet.
Open problems and what is being done
Transformation to diffuse large B-cell lymphoma in a minority.
No standard sequence of therapies is proven by randomised trials.
Rare sites (lung, skin, dura) are managed by extrapolation.
and how the field plans to fix it →What is being done about thisRare cancers and small trialsAvailable now- AI trial matching & clinical decision supportEstablished
- Comprehensive genomic profilingStandard of care
- Dinutuximab (ch14.18) / dinutuximab betaApproved
- Eflornithine (DFMO)Approved
- LarotrectinibApproved
- Limb-salvage surgery and endoprosthetic reconstructionStandard of care
In trials- ACTIONRecruiting
- COG AALL1731Positive
- COG ANBL0032Positive
- DeFiPositive
- Euro Ewing 2012Positive
- Functional (ex vivo) drug testingEmerging
Ideas and roadmaps- A digital second-opinion network answering community oncologists within 72 hours
- A DRUP-style protocol for off-label generic targeted drugs in rare tumours
- A funded expert second opinion for every new high-stakes or rare cancer diagnosis
- A global first-in-human network for academic cancer trials with single ethics review
- A global open trials operating system any hospital can plug into
- A live 'seats available' feed for trial slots, like airline inventory
Background: Basket, umbrella, and platform trials, Centralisation and high-volume centres, FNCLCC grade (soft-tissue sarcoma), Histotype-tailored therapy, INRG staging and risk groups. Also on OnCo: Find a trial · Expert centres.
Trials
topTrials recruiting now
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Expert centres
topExpert centres
- Aarhus University HospitalAarhus, DKvia IMRT / IGRT (modern external beam)
- American Society for Radiation OncologyArlington, VA, USvia IMRT / IGRT (modern external beam)
- Centre Antoine LacassagneNice, FRvia IMRT / IGRT (modern external beam)
- Centre Oscar LambretLille, FRvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- Comprehensive Cancer Center Freiburg (CCCF)Freiburg im Breisgau, DEvia IMRT / IGRT (modern external beam)
- European Society for Radiotherapy and OncologyBrussels, BEvia IMRT / IGRT (modern external beam)
- Geneva University Hospitals (HUG)Geneva, CHvia IMRT / IGRT (modern external beam)
- Groote Schuur Hospital / University of Cape TownCape Town, ZAvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- Hacettepe University Cancer InstituteAnkara, TRvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- Ho Chi Minh City Oncology HospitalHo Chi Minh City, VNvia IMRT / IGRT (modern external beam)
- Hokkaido University HospitalSapporo, JPvia IMRT / IGRT (modern external beam)
- Hunan Cancer HospitalChangsha, CNvia IMRT / IGRT (modern external beam)
- Institut BergoniéBordeaux, FRvia IMRT / IGRT (modern external beam)
- Institut National d'Oncologie, RabatRabat, MAvia IMRT / IGRT (modern external beam)
- Institut Salah AzaïezTunis, TNvia IMRT / IGRT (modern external beam)
- Institute of Oncology LjubljanaLjubljana, SIvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- International Extranodal Lymphoma Study GroupBellinzona, CHvia IMRT / IGRT (modern external beam)
- IRCCS Humanitas Research HospitalRozzano (Milan), ITvia IMRT / IGRT (modern external beam)
- Istanbul University Institute of OncologyIstanbul, TRvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- Juravinski Cancer Centre / Escarpment Cancer Research InstituteHamilton, ON, CAvia IMRT / IGRT (modern external beam)
- Kenyatta National HospitalNairobi, KEvia IMRT / IGRT (modern external beam)
- Korle Bu Teaching HospitalAccra, GHvia IMRT / IGRT (modern external beam)
- Lagos University Teaching HospitalLagos, NGvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- National Institute of Oncology, HungaryBudapest, HUvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- Ocean Road Cancer InstituteDar es Salaam, TZvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- Rajiv Gandhi Cancer Institute and Research CentreNew Delhi, INvia IMRT / IGRT (modern external beam)
- Rambam Health Care CampusHaifa, ILvia IMRT / IGRT (modern external beam)
- Rigshospitalet, Copenhagen University HospitalCopenhagen, DKvia IMRT / IGRT (modern external beam)
- Royal Adelaide HospitalAdelaide, AUvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- Siriraj Hospital, Mahidol UniversityBangkok, THvia IMRT / IGRT (modern external beam)
- Tata Medical Center, KolkataKolkata, INvia IMRT / IGRT (modern external beam)
- Tawam HospitalAl Ain, AEvia IMRT / IGRT (modern external beam)
- Tel Aviv Sourasky Medical CenterTel Aviv, ILvia IMRT / IGRT (modern external beam)
- The Ohio State University Comprehensive Cancer Center, James Cancer Hospital and Solove Research InstituteColumbus, OH, USNCI comprehensivevia Ibrutinib
- via IMRT / IGRT (modern external beam)
- TROG Cancer ResearchNewcastle, NSW, AUvia IMRT / IGRT (modern external beam)
- UMC Utrecht Cancer CenterUtrecht, NLvia IMRT / IGRT (modern external beam)
- University of Malaya Medical CentreKuala Lumpur, MYvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- Velindre Cancer CentreCardiff, GBvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- Zhejiang Cancer HospitalHangzhou, CNvia IMRT / IGRT (modern external beam)
Questions to ask
topQuestions to ask your oncologist about Marginal zone lymphoma
Newly diagnosed
- What is my exact diagnosis, stage, and grade, and which tests established them?Why: Everything else follows from an accurate stage and subtype.
- Which biomarkers have been tested on my tumour (for example Helicobacter pylori status, tMALT1 translocation, Hepatitis C serology, MYD88 wild-type, Plasmacytic differentiation and paraprotein), and what were the results?Why: These results decide eligibility for targeted therapy, immunotherapy, and trials.
- Which subtype is my cancer, and does that change the recommended treatment?Why: Recognised subtypes for this cancer include Extranodal marginal zone lymphoma of MALT, Splenic marginal zone lymphoma, Nodal marginal zone lymphoma.
- Is germline (inherited) genetic testing recommended for me or my family?Why: Inherited variants can change treatment and matter for relatives.
Gastric MALT lymphoma, H. pylori-positive
- For my situation (gastric malt lymphoma, h. pylori-positive), which of the standard options do you recommend and why?Why: Guideline options include: Eradication therapy and endoscopic follow-up; radiotherapy if the lymphoma persists or carries t(11;18).
Localised extranodal disease
- For my situation (localised extranodal disease), which of the standard options do you recommend and why?Why: Guideline options include: Low-dose involved-site radiotherapy (as little as 4 Gy in two fractions for some sites); surgery rarely.
Splenic marginal zone lymphoma
- For my situation (splenic marginal zone lymphoma), which of the standard options do you recommend and why?Why: Guideline options include: Watch and wait if asymptomatic; antiviral therapy if hepatitis C-positive; rituximab alone or with chemotherapy; splenectomy now rare.
- Am I a candidate for Rituximab, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
Advanced or relapsed
- For my situation (advanced or relapsed), which of the standard options do you recommend and why?Why: Guideline options include: Rituximab with bendamustine or chlorambucil, lenalidomide-rituximab; zanubrutinib or ibrutinib for relapsed disease.
- Am I a candidate for Rituximab, Bendamustine, Chlorambucil or related drugs, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
Any stage
- Would a second opinion at a high-volume centre change anything, and can you help arrange it?Why: Rare or high-stakes decisions benefit from a centre that treats many similar patients.
- What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?Why: Supportive care improves quality of life and helps patients complete treatment.
- I read that “Transformation to diffuse large B-cell lymphoma in a minority”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
- I read that “No standard sequence of therapies is proven by randomised trials”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
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Direct links plus the targets, companies, and technologies of this cancer's products.
technologies
6targets
2drugs
6companies
7Latest papers
topQuery for this cancer: (TITLE:"Marginal zone lymphoma" OR ABSTRACT:"Marginal zone lymphoma" OR TITLE:"MZL" OR ABSTRACT:"MZL" OR TITLE:"MALT lymphoma" OR ABSTRACT:"MALT lymphoma" OR TITLE:"Extranodal marginal zone lymphoma" OR ABSTRACT:"Extranodal marginal zone lymphoma" OR TITLE:"Splenic marginal zone lymphoma" OR ABSTRACT:"Splenic marginal zone lymphoma" OR TITLE:"Nodal marginal zone lymphoma" OR ABSTRACT:"Nodal marginal zone lymphoma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Marginal zone lymphoma, not a curated reading list.
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