Abstract

Background: Primary mediastinal B-cell lymphoma (PMBCL) is a distinct clinicopathologic entity from diffuse large B-cell lymphoma. The optimal first-line therapy for PMBCL is subject of ongoing debate with no accepted standard of care. Patients and Methods: We searched retrospectively for adult patients with newly diagnosed PMBCL treated at our department between 2002 and 2014. Clinical, management and follow-up data were collected. Staging and response assessment of patients included PET and/or CT scan. Results: Twenty-nine patients with PMBCL (17 female and 12 male) were included. The median age at diagnosis was 36 years (18-79 years). Eighteen (62.1%) and 20 (69%) patients had limited-stage and bulky disease, respectively. All patients were treated with rituximab-based combination chemotherapy; 21 patients underwent consolidation radiotherapy. Seven patients (24.1%) were transplanted (six in first remission and the remaining in second remission). At the end of frontline therapy, 28 patients had responded (27 complete response and 1 partial response) and one patient showed progressive disease. Febrile neutropenia was the most frequent acute adverse event and three patients developed late toxicity. The median follow-up was 51,5 months. The 5-year overall progression-free survival was 83.8%. Four patients died, half of which died within the first year after diagnosis. Conclusion: Our study shows favorable prognosis of patients with PMBCL treated with rituximab-based chemotherapy and consolidation radiotherapy or autologous stem-cell transplant. While consolidation therapy continues to be commonly used, its role has become increasingly controversial.

Highlights

  • Primary mediastinal B-cell lymphoma (PMBCL) was first recognized in 1980, but only recently has caught attention after being classified as a distinct subtype of diffuse large B-cell lymphoma (DLBCL) [1,2]

  • All patients were treated with rituximab-based combination chemotherapy; 21 patients underwent consolidation radiotherapy

  • Our study shows favorable prognosis of patients with PMBCL treated with rituximab-based chemotherapy and consolidation radiotherapy or autologous stem-cell transplant

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Summary

Introduction

Primary mediastinal B-cell lymphoma (PMBCL) was first recognized in 1980, but only recently has caught attention after being classified as a distinct subtype of diffuse large B-cell lymphoma (DLBCL) [1,2]. It is an aggressive lymphoma accounting for less than 3% of all non-Hodgkin lymphoma and 10% of all DLBCL. It occurs more often during the third and fourth decades of life and shows a female propensity (male-to-female ratio of 1:2) [3,4]. The optimal first-line therapy for PMBCL is subject of ongoing debate with no accepted standard of care

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