The prevalence of MYD88 mutations in DLBCL varies according to the lymphoma localization and is significantly higher (present in approximately 70% of cases) in immune-privileged (IP) site-associated DLBCLs, such as primary central nervous system lymphomas (PCNSLs) and primary testicular lymphomas (PTsL) [40,41], or other large B-cell lymphoma subtypes, as will be further addressed in this review. Here, MYD88 is linked to primary central nervous system lymphoma.