![]() ![]() Although initially classified as a subgroup of diffuse large B-cell lymphoma (DLBCL), it is now well established that PMBL presents some features that overlap with those of classical Hodgkin lymphoma (cHL), particularly nodular sclerosis cHL (NS-cHL). Nearly all studies have shown very good prognosis for PMBL, with a survival rate exceeding 80% at 5 years ( 3- 5) this is a singular aspect of PMBL. Accounting for nearly 2–4% of newly diagnosed non-Hodgkin lymphomas (NHL), it mostly affects young female patients (median age 37 years) ( 2) and often manifests as a large and compressive anterior mediastinal mass. Many specific clinical and biological characteristics distinguish this disease from other lymphomas ( 1). Primary mediastinal B-cell lymphoma (PMBL) is a rare entity that is characterized in the WHO classification as a mature aggressive large B-cell lymphoma (LBCL) of presumed thymic B-cell origin that mostly invades the mediastinum. Received: 26 July 2022 Accepted: 21 October 2022 Published: 30 December 2022. Keywords: Primary mediastinal B-cell lymphoma (PMBL) diagnosis and treatment relapse refractory disease We also provide future directions for PMBL management and a rationale for the use of ctDNA monitoring in PMBL patients. In the present review, we will discuss pathobiology, diagnostic spectrum, genomics, clinical issues in the treatment and prognosis of PMBL to establish unmet medical needs related to this rare condition and to help the community by describing innovative support and research. Emerging data suggest that, in addition to PET, circulating tumor DNA (ctDNA) might be an interesting tool for the assessment of minimal residual disease (MRD) in PMBL. These false PET-positive residual uptakes may prompt physicians to perform intensive consolidation and/or CRT, resulting in high rates of unnecessary ASCT and CRT in PMBL patients. Interpreting PET images of PMBL patients is very difficult due to the presence of inflammatory phenomena in the mediastinum under chemotherapy and after the end of the treatment regimen. The place of positron emission tomography (PET)-driven treatment and autologous stem cell transplantation (ASCT) consolidation in the frontline setting also remain a subject of debate. Therapeutic options include standard rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone (R-CHOP), dose-dense immunochemotherapy with rituximab plus doxorubicin, cyclophosphamide, vindesine, bleomycin, prednisone (R-ACVBP) or dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin and rituximab (DA-EPOCH-R), with or without consolidation radiotherapy (CRT). However, the therapeutic standard of care in the frontline and relapsed/refractory settings remains debated. The remarkable prognosis of patients treated with curative intention is a particular feature of PMBL, with a survival rate greater than 80% at 5 years in most reports. Several teams have highlighted the biological complexity of PMBL and contributed to building the concept that PMBL tumors possess a singular immune escape profile. It typically affects young females (median age 37 years) with onset of a large and compressive anterior mediastinal mass. Policy of Dealing with Allegations of Research MisconductĪbstract: Primary mediastinal B-cell lymphoma (PMBL) is an uncommon entity of aggressive large B-cell lymphoma (LBCL), representing approximately 2–4% of newly diagnosed non-Hodgkin lymphomas (NHL) and having many biological and clinical features that separate PMBL from diffuse large B-cell lymphoma (DLBCL).Policy of Screening for Plagiarism Process. ![]()
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