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Below, week by week, is what OnCo's record of Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) says about the first two months: the order is typical, the timing is yours to ask about. 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. Sections appear only where the record has something to say. Orientation, not medical advice.
Staging tests establish exactly what and where the cancer is. Everything else follows from the answers.
Ask which of these were tested and what the results were; see the report reader for what each value means.
These specialties appear in the standard of care for this cancer. In most centres they meet weekly as a tumour board to agree each plan; you can ask when yours was discussed and what was decided.
A second opinion from a centre that treats many similar cases is normal, not rude; the standard of care tells you what to compare it against.
Each row is a setting from the standard of care, in the order it usually arises. Not all will apply to you; your stage and biomarkers decide which do. The guideline grade, where recorded, says how strong the evidence is.
Involved-site radiotherapy (the FoRT trial tested the dose in follicular and marginal zone lymphoma).
H. pylori eradication first, with endoscopic follow-up; radiotherapy if the lymphoma persists.
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.
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.
Rituximab alone or with chlorambucil or bendamustine; zanubrutinib or lenalidomide-rituximab later (AUGMENT; MAHOGANY).
Generated from this cancer's standard of care, biomarkers and open problems. Take the group that matches where you are. To tick, add your own and print, open the one-page appointment sheet.
Trials open now for this cancer in OnCo, largest phase first. Joining a trial is a decision like any other: ask what the comparison arm is, whether a placebo is used, and what happens if you leave. The cancer page searches ClinicalTrials.gov live for more.
Much of what helps in the first weeks is free if you know to ask: testing, helplines, rides and lodging, second opinions, trial travel.
Every term links to the glossary.