Abstract: Primary mediastinal large B-cell lymphoma (PMBL) has unique clinical clinicopathological and biological characteristics. Studies have found that certain molecular features are similar to nodular sclerosis Hodgkin lymphoma(NSHL). Recent researches have focused not only on the common biological characteristics of these two tumors, but also on the identification of new key pathways for lymphoma formation, as well as their different mutant genes. Although the current standard of treatment for PMBL is chemotherapy and radiation therapy, methods are also proposed to eliminate radiation and thereby eliminate its long-term toxic effects. The role and impact of new targeted drug have been evaluated in PMBL, paving the way for testing drugs such as small molecule inhibitors and immunological checkpoint inhibitors, and indicating that future treatments should focus on combining effective new drugs with chemotherapy.