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Review
. 2022 Jan 17;14(2):446.
doi: 10.3390/cancers14020446.

Mucosa-Associated Lymphoid Tissue (MALT) Lymphoma in the Gastrointestinal Tract in the Modern Era

Eri Ishikawa  1 , Masanao Nakamura  1 , Akira Satou  2 , Kazuyuki Shimada  3 , Shotaro Nakamura  4
Affiliations
Review

Mucosa-Associated Lymphoid Tissue (MALT) Lymphoma in the Gastrointestinal Tract in the Modern Era

Eri Ishikawa et al. Cancers (Basel). .

Abstract

Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) typically arises from sites such as the stomach, where there is no organized lymphoid tissue. Close associations between Helicobacter pylori and gastric MALT lymphoma or Campylobacter jejuni and immunoproliferative small intestinal disease (IPSID) have been established. A subset of tumors is associated with chromosomal rearrangement and/or genetic alterations. This disease often presents as localized disease, requiring diverse treatment approaches, from antibiotic therapy to radiotherapy and immunochemotherapy. Eradication therapy for H. pylori effectively cures gastric MALT lymphoma in most patients. However, treatment strategies for H. pylori-negative gastric MALT lymphoma are still challenging. In addition, the effectiveness of antibiotic therapy has been controversial in intestinal MALT lymphoma, except for IPSID. Endoscopic treatment has been noted to usually achieve complete remission in endoscopically resectable colorectal MALT lymphoma with localized disease. MALT lymphoma has been excluded from post-transplant lymphoproliferative disorders with the exception of Epstein-Barr virus (EBV)-positive marginal zone lymphoma (MZL). We also describe the expanding spectrum of EBV-negative MZL and a close association of the disease with the gastrointestinal tract.

Keywords: Helicobacter pylori; MALT lymphoma; extranodal marginal zone lymphoma; gastric lymphoma; gastrointestinal lymphoma; immunoproliferative small intestinal disease (IPSID); intestinal lymphoma.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Endoscopic images of small intestinal MALT lymphoma. (A) Irregularity of the duodenal mucosa. (B) A shallow ulcer with diffuse mucosal edema in the jejunum. (C,D) Multiple ulcerated lesions in the ileum.
Figure 2
Figure 2
Immunoproliferative small intestinal disease (IPSID). (A,B) Double-balloon endoscopy showing villous edema in the ileum. (C) A biopsy specimen revealing blunting of the villi. (D,E) A dense lymphoplasmacytic infiltrate detected. (F,G) The tumor cells are positive for CD138 (F) and IgA (G). Original magnification: ×25 (C), ×100 (D), and ×400 (E–G).
Figure 3
Figure 3
Rectal-mucosa-associated lymphoid tissue (MALT) lymphoma. (A,B) Endoscopic images showing a 10 mm solitary elevated lesion with erosion on the surface. (C) Narrow band imaging showing enlarged vessels on the surface of the lesion. (D) Specimen from endoscopic mucosal resection. (E,F) Monotonous proliferation of small- to intermediate-sized centrocyte-like cells observed. (G–I) The tumor cells are positive for CD20 (G) and negative for CD5 (H) and CD10 (I). Original magnification: ×100 (E) and ×400 (F–I).
See this image and copyright information in PMC

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