However, application of a targeted therapeutic approach involving the CARD11–BCL10-MALT1 (CBM) signaling pathway or drugs like lenalidomide which are used for diffuse large B cell lymphomas (DLBCL) and MALT1 protease inhibitors may help in the better management of BENTA patients in the near future. This evidence concerns the gene MALT1 and diffuse large B-cell lymphoma.