A relatively large subset of primary OA-DLBCL-NOS patients (36%) exhibited genetic features according to the LymphGen MCD subtype with pathogenic variants in several genes that are involved in NF-κB signaling, and which are recurrently mutated in other primary extranodal DLBCLs of non-GCB origin, including those of immune privileged sites. Here, NFKB1 is linked to diffuse large B-cell lymphoma.