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

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.

Immunopathogenesis and environmental triggers in coeliac disease

“…This is further illustrated by the recent demonstration of constitutive mutations enhancing this pathway in patients with non-coeliac autoimmune enteropathies, who are also at risk of lymphomas 87. RCD2 and EATL also exemplify how the interplay between acquired somatic mutations and chronic inflammation driven by prolonged exposure to a luminal antigen can foster lymphomagenesis in the GI tract, a scenario already well established in mucosa-associated lymphoid tissue B lymphomas or in alpha chain immunoproliferative diseases 130…”

“… 87 RCD2 and EATL also exemplify how the interplay between acquired somatic mutations and chronic inflammation driven by prolonged exposure to a luminal antigen can foster lymphomagenesis in the GI tract, a scenario already well established in mucosa-associated lymphoid tissue B lymphomas or in alpha chain immunoproliferative diseases. 130 …”

“…87 RCD2 and EATL also exemplify how the interplay between acquired somatic mutations and chronic inflammation driven by prolonged exposure to a luminal antigen can foster lymphomagenesis in the GI tract, a scenario already well established in mucosa-associated lymphoid tissue B lymphomas or in alpha chain immunoproliferative diseases. 130 If to date the GFD proposed in the mid-1950s remains the only treatment for uncomplicated CD, mechanistic insight into CD pathogenesis has opened many new diagnosis and therapeutic avenues. The discovery that TG2 is the target of coeliac-specific autoantibodies has led to develop highly specific diagnostic tests.…”

Radiotherapeutic Outcomes for Localized Primary Rectal Mucosa-Associated Lymphoid Tissue Lymphoma: A Consecutive Case Series of Three Patients

“…Gut-associated lymphoid tissue (GALT) is known as the largest immunologic organ in the body and considerably contributes to the immune system of the whole body [ 9 ]. Intestinal MALToma is considered to develop from GALT, and the stomach is the most common site of intestinal lymphoma [ 10 ]. Excellent clinical outcomes have been achieved using EBRT in patients with gastric MALToma [ 11 ].…”

Rare primary rectal mucosa-associated lymphoid tissue lymphoma with curative resection by endoscopic submucosal dissection: A case report and review of literature

“…Lastly, a large case analysis of colorectal MALT lymphoma reported that chemotherapy is most commonly performed for advanced disease. Chemotherapy is not recommended for patients with localized MALT lymphomas as these tumors are less responsive to standard chemotherapy than aggressive lymphomas[ 32 ]. Chemotherapy and/or immunotherapy is given when patients fail to respond to localized therapy or develop metastasis.…”