Mucosa-Associated Lymphoid Tissue Lymphomas (MALTomas) Guidelines

Updated: Jan 21, 2022
  • Author: Sara J Grethlein, MD, MBA, FACP; Chief Editor: Emmanuel C Besa, MD  more...
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Guidelines

Guidelines Summary

Gastric MALT Lymphoma

National Comprehensive Cancer Network (NCCN) guidelines recommend the following studies to establish a diagnosis of gastric MALT lymphoma [18] :

  • Endoscopic biopsy; fine needle aspiration (FNA) is never acceptable
  • Immunohistochemistry panel: CD20, CD3, CD5, CD10, BCL2, kappa/lamda, CD21 or CD23, cyclin D1, BCL6
  • Cell surface marker analysis by flow cytometry: kappa/lambda, CD19, CD20, CD5, CD23, CD10
  • Helicobacter pylori stain; if positive, then polymerase chain reaction (PCR) or fluorescence in situ hybridization (FISH) for  t(11;18)

The following studies may be useful in select circumstances:

  • Molecular analysis for detection of antigen receptor gene rearrangements or if plasmacytic differentiation is present, MYD88 mutation status 
  • FISH or cytogenetics for detection of  t(1;14),  t(3;14),  t(11;14),  t(11;18)
  • FISH or PCR:  t(14;18)

The European Society for Medical Oncology (ESMO) recommends serology, urea breath test, and/or stool antigen test for H pylori in cases where immunohistochemistry findings are negative. [38]

The NCCN guidelines recommend antibiotic therapy for stages I and II in H pylori–positive patients, followed by endoscopy/biopsy for restaging at 3 months or sooner if symptomatic. Further treatments include second-line antibiotics, if H pylori testing remains positive, and involved-site radiation therapy (ISRT) if lymphoma positive. Patients who are t(11;18) positive can be treated with rituximab if ISRT is contraindicated. For patients who are H pylori negative, ISRT is the preferred treatment. Rituximab is the alternative if ISRT is contraindicated. [18]

Patients with advanced stage disease should be observed unless they have one of the following indications for treatment [18] :

  • Candidate for a clinical trial
  • Symptoms
  • GI bleeding
  • Threatened end-organ function
  • Bulky disease
  • Steady or rapid progression
  • Patient preference

For first-line therapy, the NCCN prefers the following regimens [18] :

  • Bendamustine + rituximab 
  • RCHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone)
  • RCVP (rituximab, cyclophosphamide, vincristine, prednisone)
  • Rituximab
  • Ibritumomab tiuxetan (category 2B)
  • Lenalidomide + rituximab (category 2B)

The ESMO guidelines are in general agreement with NCCN for the treatment of gastic MALT lymphoma. [38]

Nongastric MALT Lymphoma

The NCCN guidelines recommend the following studies to establish a diagnosis of nongastric MALT lymphoma [18] :

  • Immunohistochemistry panel: CD20, CD3, CD5, CD10, BCL2, kappa/lamda, CD21 or CD23, cyclin D1
  • Cell surface marker analysis by flow cytometry: kappa/lambda, CD19, CD20, CD5, CD23, CD10

The following studies may be useful in select circumstances:

  • Molecular analysis for detection of antigen receptor gene rearrangements or if plasmacytic differentiation is present,  MYD88 mutation status 
  • FISH or cytogenetics for detection of  t(3;14),  t(11;14),  t(11;18)
  • FISH or PCR:  t(14;18)

For stage I and II, the preferred treatment is ISRT. Surgery may be considered for certain sites (ie, thyroid, colon/small bowel and lumpectomy for breast). Rituximab and observation are options in select cases. [18]

For advanced stages, the treatment options are ISRT, observation in select cases or first-line therapy regimens as listed above for gastric MALT lymphoma. [18]