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

Huan Chen; Tao Pan; Yizi He; Ruolan Zeng; Yajun Li; Liming Yi; Hui Zang; Siwei Chen; Qintong Duan; Ling Xiao; Hui Zhou

doi:10.3389/fonc.2021.654854

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

Front Oncol. 2021 Mar 22:11:654854. doi: 10.3389/fonc.2021.654854. eCollection 2021.

Authors

Huan Chen¹, Tao Pan^{1

2}, Yizi He¹, Ruolan Zeng¹, Yajun Li¹, Liming Yi³, Hui Zang⁴, Siwei Chen⁵, Qintong Duan⁵, Ling Xiao⁵, Hui Zhou¹

Affiliations

¹ Department of Lymphoma and Hematology, Hunan Cancer Hospital, The Affiliated Cancer Hospital of Xiangya School of Medicine, Central South University, Changsha, China.
² The Third Xiangya Hospital, Central South University, Changsha, China.
³ Department of Human Anatomy, Hunan University of Medicine, Huaihua, China.
⁴ Department of Basic Medicine, Yiyang Medical College, Yiyang, China.
⁵ Department of Histology and Embryology of School of Basic Medical Science, Central South University, Changsha, China.

Abstract

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 features and limited availability of clinical data. The current standard treatment for newly diagnosed PMBCL has long been dependent on a dose-intensive, dose-adjusted multi-agent chemotherapy regimen of rituximab plus etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin (DA-R-EPOCH). 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. Positron emission tomography-computed tomography (PET-CT) is the preferred imaging modality of choice for the initial phase of lymphoma treatment and to assess response to treatment. In the future, baseline quantitative PET-CT can be used to predict prognosis in PMBCL. This review focuses on the pathology of PMBCL, underlying molecular basis, treatment options, radiotherapy, targeted therapies, and the potential role of PET-CT to guide treatment choices in this disease.

Keywords: chemotherapy; mediastinal radiation; positron emission tomography-computed tomography; primary mediastinal large B-cell lymphoma; targeted therapy.

Publication types

Review