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Transformation to diffuse large B-cell lymphomas (DLBCL) occurs in <10% of extranodal marginal zone lymphomas (EMZL). Tumors that are negative for t(11;18) translocation and BIRC3-MALT1 fusion show a greater tendency to transform to DLBCL.
Progression from low-grade to high-grade lymphomas is promoted by loss of p16INK4A and TP53 gene function. Additional genetic alterations found in transformation to DLBCL include BCL6, CCND3, as well as MYC overexpression, and strong nuclear FOXP1 expression. Gastric EMZL progressing to DLBCL show additional changes, including upregulation of the chemokine receptors CXCR3 and CXCR7.
Image source: Schreuder MI et al. Novel developments in the pathogenesis and diagnosis of extranodal marginal zone lymphoma. J Hematop. 2017 Dec. 10(3-4): 91-107. Cropped from original image. Used under Creative Commons Attribution 4.0 International License.
Progression from low-grade to high-grade lymphomas is promoted by loss of p16INK4A and TP53 gene function. Additional genetic alterations found in transformation to DLBCL include BCL6, CCND3, as well as MYC overexpression, and strong nuclear FOXP1 expression. Gastric EMZL progressing to DLBCL show additional changes, including upregulation of the chemokine receptors CXCR3 and CXCR7.
Image source: Schreuder MI et al. Novel developments in the pathogenesis and diagnosis of extranodal marginal zone lymphoma. J Hematop. 2017 Dec. 10(3-4): 91-107. Cropped from original image. Used under Creative Commons Attribution 4.0 International License.