Primary breast lymphoma (PBL) is a rare and unique extranodal lymphoma, the most common clinical manifestation of which is the painless progressive breast tumor. The clinical progress of PBL is fast and the prognosis of patients is extremely poor. Rituximab cannot improve the survival of PBL patients, so new treatment strategies are needed. PIM1, MYD88, CD79B, CARD11, and TNFIAP3 are high-frequency mutant genes of PBL, which are screened by next generation sequencing (NGS) technology in recent years. The nuclear factor-κB signaling pathway is used as the main target signal.This article summarizes the overview of PBL, the effort of nuclear factor-κB signaling pathway in PBL, and the relationship between high-frequency mutant genes and occurrence of PBL, in order to provide a theoretical basis for exploring the ideal treatment plan of PBL and improving the cure rate of patients.
