Primary Mediastinal B-Cell Lymphoma: Novel Precision Therapies and Future Directions

Primary mediastinal large B-cell lymphoma (PMBCL) is a distinct clinicopathologic disease from other types of diffuse large B-cell lymphoma (DLBCL) with unique prognostic factors and limited clinical data. The current standard treatment of newly diagnosed PMBCL has long been depending on a dosed intensive, dosed adjusted multi-agent chemotherapy regimen (DA-EPOCH-R). Recent randomized trials have provided evidence that R-CHOP followed by consolidation radiotherapy (RT) is a valuable alternative option to first-line treatment. For recurrent/refractory PMBCL (rrPMBCL), new drugs (such as Pembrolizumab and CAR T cell therapy) have proven to be effective in a few studies. PET is the preferred imaging modality of choice for the initial phase of lymphoma treatment and to assess response. In the future, baseline quantitative FDG-PET can be used to predict prognosis in PMBCL. This review focuses on the pathology of PMBCL, molecular basis, treatment, radiotherapy, targeted therapies, and the potential role of PET-CT to guide treatment choices in this disease.