The recurrent positive dominant mutation L265P (~29% of ABC DLBCL cases) localized in the TIR domain of MyD88 (MyD88L265P) leads to the spontaneous oligomerization of MyD88 and drives the activation of NF-kB signaling without any additional stimuli, likely following the above-described phosphorylation and ubiquitination cascade for the formation of the natural Myddosome [4,19,20]. This evidence concerns the gene MYD88 and aneurysmal bone cyst.