![]() ![]() 11, 12 Lastly, whether consolidative RT can be omitted using dose-intensified regimens or in low-risk patients treated with R-CHOP remains controversial. 10 Second, given the younger age and female predominance in PMBCL, significant concerns remain with respect to toxicity from mediastinal RT, particularly the increased risk of secondary breast cancer and cardiotoxicity. First, about 20%-30% of patients experience progression or relapses with poor outcomes. 5- 9ĭespite the good results with R-CHOP + RT, a number of issues remain. 2- 4 However, the addition of rituximab to CHOP has significantly improved the event free survival (EFS) and overall survival (OS) in PMBCL and a number of studies have suggested no benefit for dose-intensified regimens when compared with R-CHOP ± radiotherapy (RT) in the rituximab era. Prior to the introduction of rituximab, dose-dense and dose-intense second- and third-generation protocols such as VACOP-B (etoposide, doxorubicin, cyclophosphamide, vincristine, prednisone, and bleomycin) showed better patient outcomes over CHOP (cyclophosphamide, vincristine, doxorubicin, and prednisolone) chemotherapy. The optimal chemotherapy regimen and the role of radiotherapy in PMBCL remains an area of research. 1 This has led PMBCL to be recognized as a unique entity by the World Health Organization classification of lymphoid tumors since 2001. Although PMBCL was previously classified as a subtype of DLBCL, it has specific clinical, histological, and molecular features that distinguish it from DLBCL and overlap instead with nodular-sclerosis classical Hodgkin lymphoma. It typically occurs in young females, who present with bulky anterior mediastinal mass causing superior vena cava syndrome. Primary mediastinal large B-cell lymphoma (PMBCL) is an uncommon B-cell lymphoma accounting for 2%-4% of all non-Hodgkin lymphomas. In patients with bulky disease, the use of DA-EPOCH-R may be preferable as it allows omission of RT without reduction in efficacy. ![]() In conclusion, R-CHOP + RT and DA-EPOCH-R provide excellent outcomes in patients with PMBCL. In contrast, in patients without bulk (n = 42), there was no impact of treatment regimen on PFS ( P = 0.25). ![]() Patients who received R-CHOP + RT or DA-EPOCH-R had better PFS than those receiving R-CHOP alone, with 5-year PFS of 90% vs 88.5% vs 56%, respectively ( P = 0.02). The chemo-radiotherapy regimen, Japanese IPI and Ann-Arbor stage was significantly associated with PFS in univariate analysis, but only chemo-radiotherapy regimen remained significant ( P = 0.02) after multivariate analysis. The type of chemo-radiotherapy regimen, B symptoms and Ann-Arbor staging showed a significant association with OS on univariate analysis but only B symptoms remained prognostic ( P = 0.012) after multivariate analysis. With a median follow up of 45 months, the overall 5-year OS and PFS was 89.4% and 82.4%, respectively. 6% (n = 3) in the DA-EPOCH-R group received RT. We performed a multicenter retrospective review of 124 patients with newly diagnosed PMBCL between 20. Optimal treatment and role for radiotherapy is not fully defined. Primary mediastinal large B-cell lymphoma (PMBCL) is a distinct clinico-pathological subtype of diffuse large B-cell lymphoma with unclear prognostic factors and limited clinical data. ![]()
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