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<p>Diffuse large B cell lymphoma (DLBCL) is the most common type of non-Hodgkin lymphoma (NHL), with approximately 30%–40% of cases occurring in extranodal organs. Primary gastrointestinal diffuse large B cell lymphoma (PGI-DLBCL) is a predominant subtype of extranodal lymphoma, accounting for 40%–50% of extranodal DLBCL cases. However, due to its location of onset in the gastrointestinal tract, it is different from cancer of epithelial or primary intranodal DLBCL or other extranodal DLBCL. The patients with PGI-DLBCL are often first diagnosed in the department of gastroenterology and gastrointestinal surgery. These patients are prone to develop bulky masses and lead to severe gastrointestinal complications. The heterogeneity of PGI-DLBCL is not only reflected in the differences in gene mutation profiles and tumor microenvironment but is also closely related to the diversity of clinical manifestations, varied treatment responses, and complex prognostic stratification. Currently, the clinicopathological characteristics and molecular genetics of these patients are not fully understood, which will lead to the clinical management difficulties and challenges. This review provides a relatively comprehensive analysis about the heterogeneity of PGI-DLBCL from four perspectives: aggressive growth features, pathological features, molecular genetic characteristics, and treatments.</p>

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Heterogeneity in primary gastrointestinal DLBCL: from clinical management to molecular mechanisms and treatment strategies

  • 期刊论文

  • 发表日期：

    2026年3月31日

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  • Xiaohong Liu,
  • Daoming Zhang,
  • Le Xu,
  • Dedong Cao,
  • Ximing Xu

摘要

Diffuse large B cell lymphoma (DLBCL) is the most common type of non-Hodgkin lymphoma (NHL), with approximately 30%–40% of cases occurring in extranodal organs. Primary gastrointestinal diffuse large B cell lymphoma (PGI-DLBCL) is a predominant subtype of extranodal lymphoma, accounting for 40%–50% of extranodal DLBCL cases. However, due to its location of onset in the gastrointestinal tract, it is different from cancer of epithelial or primary intranodal DLBCL or other extranodal DLBCL. The patients with PGI-DLBCL are often first diagnosed in the department of gastroenterology and gastrointestinal surgery. These patients are prone to develop bulky masses and lead to severe gastrointestinal complications. The heterogeneity of PGI-DLBCL is not only reflected in the differences in gene mutation profiles and tumor microenvironment but is also closely related to the diversity of clinical manifestations, varied treatment responses, and complex prognostic stratification. Currently, the clinicopathological characteristics and molecular genetics of these patients are not fully understood, which will lead to the clinical management difficulties and challenges. This review provides a relatively comprehensive analysis about the heterogeneity of PGI-DLBCL from four perspectives: aggressive growth features, pathological features, molecular genetic characteristics, and treatments.