NFKBIZ was originally shown to contribute to ABC-DLBCL pathogenesis by upregulating specific NF-κB target genes [108]. NFKBIZ mutations tend to cluster with BN2 -DLBCL, which also displays BCL10, PRKCB, and TRAF6 mutations, in addition to TNFAIP3 and TNIP1, potentially promoting the formation or function of the CBM protein complex. This evidence concerns the gene TNFAIP3 and aneurysmal bone cyst.