HEALTH PROFESSIONAL · SOURCE READING
Treatment options for marginal zone lymphoma (including MALT lymphoma)
Source: Childhood Non-Hodgkin Lymphoma Treatment (PDQ®)–Health Professional Version, National Cancer Institute.
Source updated: April 17, 2025 · Captured 2026-09-09.
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Treatment options for marginal zone lymphoma (including MALT lymphoma) include the following:
Surgery only.
Radiation therapy.
Rituximab with or without chemotherapy.
Antibiotic therapy, for MALT lymphoma.
Most pediatric patients with marginal zone lymphomas require no more than local therapy involving curative surgery and/or radiation therapy.[22,24] Treatment of patients with MALT lymphoma of the gastric mucosa may also include antibiotic therapy, which is considered standard treatment in adults. A series of five patients with H. pylori-associated MALT lymphoma received triple therapy with amoxicillin, nitroimidazole or a macrolide, and a proton pump inhibitor for 14 days. All patients achieved H. pylori eradication and had complete resolution of histological findings at the last follow-up.[25]
Evidence (treatment of marginal zone lymphoma):
In the largest retrospective study of pediatric patients (aged 18 years or younger) with marginal zone lymphoma (N = 66), the following was reported:[26][Level of evidence C1]
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In the largest retrospective study of pediatric patients (aged 18 years or younger) with marginal zone lymphoma (N = 66), the following was reported:[26][Level of evidence C1]
The overall 5-year EFS rate was 70%.
In the largest retrospective study of pediatric patients (aged 18 years or younger) with marginal zone lymphoma (N = 66), the following was reported:[26][Level of evidence C1]
The OS rate was 98%.
In the largest retrospective study of pediatric patients (aged 18 years or younger) with marginal zone lymphoma (N = 66), the following was reported:[26][Level of evidence C1]
Patients primarily fell into the following two WHO-defined groups:
In the largest retrospective study of pediatric patients (aged 18 years or younger) with marginal zone lymphoma (N = 66), the following was reported:[26][Level of evidence C1] › Patients primarily fell into the following two WHO-defined groups:
Nodal (32%): Nearly all patients were male, with localized primary tumors in the head and neck. The treatment for all patients was resection (complete or incomplete) followed by observation. The EFS rate was 94%, and the OS rate was 100%.
In the largest retrospective study of pediatric patients (aged 18 years or younger) with marginal zone lymphoma (N = 66), the following was reported:[26][Level of evidence C1] › Patients primarily fell into the following two WHO-defined groups:
Extranodal (67%): 57% of patients were male, and 27% of patients had a preexisting condition, which was immune compromising in most patients. The treatment options included chemotherapy, radiation, rituximab, resection, and observation. The EFS rate was 64%, and the OS rate was 97%. The only two deaths resulted from treatment-related complications of HSCT. Both patients had an underlying immunodeficiency. Of note, 9 of 12 patients with extranodal marginal zone lymphoma who were managed with resection only remained in a first continuous complete remission with no further therapy. The other 3 patients who relapsed had their disease successfully salvaged.
Although the number of pediatric patients with MALT lymphoma is too small to perform meaningful clinical trials, studies of adult patients support the use of rituximab with or without chemotherapy. For more information, see the Marginal Zone Lymphoma section in Indolent B-Cell Non-Hodgkin Lymphoma Treatment.
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Intralesional interferon-alpha for conjunctival MALT lymphoma has been studied in trials.[27]
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Publication references
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