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

Overview

Journal Front Oncol

Specialty Oncology

Date 2021 Apr 19

PMID 33869061

Citations 6

Authors

Huan Chen

Tao Pan

Yizi He

Ruolan Zeng

YaJun Li

Liming Yi

Hui Zang

Siwei Chen

Qintong Duan

Ling Xiao

Hui Zhou

Affiliations

Soon will be listed here.

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.

Citing Articles

Comprehensive genomic characterization of hematologic malignancies at a pediatric tertiary care center.

Kebede A, Garfinkle E, Mathew M, Varga E, Colace S, Wheeler G Front Oncol. 2024; 14:1498409.

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[Progress in treatment of primary mediastinal large B-cell lymphoma].

Chen L, Li J, Fan L Zhonghua Xue Ye Xue Za Zhi. 2024; 45(1):98-102.

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Molecular Characterization of Primary Mediastinal Large B-Cell Lymphomas.

Donzel M, Pesce F, Trecourt A, Groussel R, Bachy E, Ghesquieres H Cancers (Basel). 2023; 15(19).

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Efficacy of Residual Site Radiation Therapy (ISRT) in Patients with Primary Mediastinal Lymphoma with Deauville Score 4 Following R-CHT: Results of a Retrospective Mono Institutional Study.

Facondo G, Serio M, Vullo G, Bianchi M, Pelliccia S, Di Rocco A J Clin Med. 2023; 12(11).

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Primary mediastinal/thymic diffuse large B-cell lymphoma: a population-based study on incidence and survival.

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