Clinicopathological Features of Primary Mediastinal Large B-cell Lymphoma: A 5-Year Retrospective Study
DOI:
https://doi.org/10.33192/smj.v78i6.281789Keywords:
Primary mediastinal large B-cell lymphoma, clinicopathological features, treatment outcomes, ThailandAbstract
Objective: To describe the clinicopathological features and treatment outcomes of primary mediastinal large B-cell lymphoma (PMBCL) at Siriraj Hospital, Thailand.
Materials and Methods: This retrospective study included all patients diagnosed with PMBCL during 2020–2024. Clinical, pathological, treatment, and survival data were retrieved from medical records and pathology reports. Overall survival (OS) and progression-free survival (PFS) were estimated using the Kaplan–Meier method.
Results: PMBCL accounted for 37 of 74 mediastinal lymphoma cases (50%). Median age was 29 years (IQR: 22–32), with female predominance (62.2%). Presentations included cough (79.4%), dyspnea (74.3%), and SVC syndrome (51.4%). Pleural or pericardial effusion was found in 75.0%. Bulky disease (>10 cm) and elevated LDH were 75.7% and 91.4%, respectively. Histologically, sclerotic pattern and HRS-like cells were observed in 41.7% and 31.4%, respectively. All cases were CD20-positive; CD23 and CD30 were expressed in 65.5% and 80.6%, respectively. A non-GCB phenotype was identified in 64.5% by the Hans algorithm. DA-EPOCH-R was the most common first-line regimen (63.9%). At a median follow-up of 25 months, the 24-month OS and PFS rates were 80.1% and 66.8%, respectively.
Conclusion: This study provides the first characterization of PMBCL in a Thai cohort, showing frequent SVC syndrome and pleural or pericardial effusion at diagnosis. Given the overlapping features and complexity of differential diagnosis, comprehensive immunohistochemical workup was essential for accuracy. DA-EPOCH-R was the dominant first-line therapy, with 2-year outcomes comparable to the rituximab era. Prompt recognition, diagnostic expertise, and multidisciplinary management are critical for optimizing PMBCL outcomes.
References
Roden AC, Fang W, Shen Y, Carter BW, White DB, Jenkins SM, et al. Distribution of Mediastinal Lesions Across Multi-Institutional, International, Radiology Databases. J Thorac Oncol. 2020;15(4):568–79.
Zhang ML, Sohani AR. Lymphomas of the Mediastinum and Their Differential Diagnosis. Semin Diagn Pathol. 2020;37(4):156–65.
Owattanapanich W, Lamool R, Pongpruttipan T, Ruchutrakool T. Analysis of 332 Mediastinal Masses in Thailand: Histopathology and Predictive Factors for Lymphoma. J Med Assoc Thai. 2017;100(6):630–7.
Zhou H, Liu Q, Lu S, Zou L. Primary mediastinal/thymic diffuse large B-cell lymphoma: a population-based study on incidence and survival. Ann Hematol. 2023;102(7):1879–86.
Traverse-Glehen AR, Rosenwald A, Fromm JR, Sarkozy C, Klapper W, Siebert R, et al. Primary mediastinal large B-cell lymphoma. In: WHO Classification of Tumours Editorial Board. Haematolymphoid tumours. 5th ed. Lyon (France): International Agency for Research on Cancer; 2024. p. 523–6.
Ahmed Z, Afridi SS, Shahid Z, Zamani Z, Rehman S, Aiman W, et al. Primary Mediastinal B-Cell Lymphoma: A 2021 Update on Genetics, Diagnosis, and Novel Therapeutics. Clin Lymphoma Myeloma Leuk. 2021;21(11):e865–e75.
Giulino-Roth L. How I treat primary mediastinal B-cell lymphoma. Blood. 2018;132(8):782–90.
Zhou J, Wang H-Y. Primary Mediastinal Large B-cell Lymphoma: Diagnostic Challenges and Recent Advances. Journal of Clinical and Translational Pathology. 2021;1(1):21–7.
Donzelli L, Rocco AD, Petrucci L, Martelli M. Primary mediastinal large B-cell Lymphoma: Biological features, clinical characteristics and current treatment strategies. Cancer Treat Rev. 2025;134:102898.
Intragumtornchai T, Bunworasate U, Wudhikarn K, Lekhakula A, Julamanee J, Chansung K, et al. Non-Hodgkin lymphoma in South East Asia: An analysis of the histopathology, clinical features, and survival from Thailand. Hematol Oncol. 2018;36(1):28–36.
Wang B, Chen R, Wang H, Qiu T, Liang Z, Cao L, et al. Real world outcomes and prognostic factors in Chinese patients with primary mediastinal B cell lymphoma: A single center experience and the Surveillance, Epidemiology, and End Results validation. Ann Hematol. 2026;105(4).
Küçükyurt S, Koca O, Terzi Demirsoy E, Akın S, Doğan A, Gören D, et al. Real-world outcomes and prognostic factors in primary mediastinal B-cell lymphoma: a multicenter study of 157 patients. Ann Hematol. 2025;104(9):4679–90.
Aoki T, Izutsu K, Suzuki R, Nakaseko C, Arima H, Shimada K, et al. Prognostic significance of pleural or pericardial effusion and the implication of optimal treatment in primary mediastinal large B-cell lymphoma: a multicenter retrospective study in Japan. Haematologica. 2014;99(12):1817–25.
Tai WM, Quah D, Peng Yap S, Huey Tan S, Tang T, Tay KW, et al. Primary mediastinal large B-cell lymphoma: optimal therapy and prognostic factors in 41 consecutive Asian patients. Leuk Lymphoma. 2011;52(4):604–12.
Noel R, Oca CMd, Sfumato P, Calleja A, Collignon A, Villetard F, et al. Long-Term Outcomes and Management Strategies With DA-R-EPOCH in Primary Mediastinal B-Cell Lymphoma: Insights From a Single-Center Experience. Hematol Oncol. 2025;43(3):e70060.
Renaud L, Donzel M, Decroocq J, Decazes P, Galtier J, Burroni B, et al. Primary mediastinal B-cell lymphoma (PMBCL): The LYSA pragmatic guidelines. Eur J Cancer. 2025;220:115369.
Zhou H, Xu-Monette ZY, Xiao L, Strati P, Hagemeister FB, He Y, et al. Prognostic factors, therapeutic approaches, and distinct immunobiologic features in patients with primary mediastinal large B-cell lymphoma on long-term follow-up. Blood Cancer J. 2020;10(5):49.
Khalid S, Khalid A, Daw H, Maroo P. Management of Primary Mediastinal B-Cell Lymphoma in Pregnancy. Cureus. 2018;10(2):e2215.
Hsieh T-C, Shah A, Olayinka O, Tissot A, Chronakas J, Pathan N, et al. Unexpected cause of postpartum cardiac arrest: primary mediastinal large B-cell lymphoma. Chest. 2021;160(4 Suppl):A1292.
Marino M, Ascani S. An overview on the differential diagnostics of tumors of the anterior-superior mediastinum: the pathologist's perspective. Mediastinum. 2019;3:6.
Moretti L, Medeiros LJ, Kunkalla K, Williams MD, Singh RR, Vega F. N-terminal PAX8 polyclonal antibody shows cross-reactivity with N-terminal region of PAX5 and is responsible for reports of PAX8 positivity in malignant lymphomas. Mod Pathol. 2012;25(2):231–6.
Sukpanichnant S. Malignancy of the Lymph Node: How General Practitioners and Pathologists can achieve a Definitive Diagnosis. Siriraj Med J. 2022;74(9):604–17.
Fairchild A, McCall CM, Oyekunle T, Niedzwiecki D, Champ C, McKinney M, et al. Primary Mediastinal (Thymic) Large B-Cell Lymphoma: Fidelity of Diagnosis Using WHO Criteria. Clin Lymphoma Myeloma Leuk. 2021;21(5):e464–e9.
Bledsoe JR, Redd RA, Hasserjian RP, Soumerai JD, Nishino HT, Boyer DF, et al. The immunophenotypic spectrum of primary mediastinal large B-cell lymphoma reveals prognostic biomarkers associated with outcome. Am J Hematol. 2016;91(10):E436–41.
Aggarwal R, Rao S, Dhawan S, Bhalla S, Kumar A, Chopra P. Primary mediastinal lymphomas, their morphological features and comparative evaluation. Lung India. 2017;34(1):19–24.
Salama ME, Rajan Mariappan M, Inamdar K, Tripp SR, Perkins SL. The value of CD23 expression as an additional marker in distinguishing mediastinal (thymic) large B-cell lymphoma from Hodgkin lymphoma. Int J Surg Pathol. 2010;18(2):121–8.
Thiebaut P-A, Isnard P, Couronné L, Kaltenbach S, Lepine C, Sibon D, et al. Multimodal integration of clinic, pathology, and genomics for a rare diagnosis of EBV-positive primary mediastinal large B-cell lymphoma. Virchows Archiv. 2024;485(5):931–6.
Gibson SE, Dojcinov S, Dotlic S, Hartmann S, Hsi ED, Klimkowska M, et al. Mediastinal large B cell lymphoma and surrounding gray areas: a report of the lymphoma workshop of the 20th meeting of the European Association for Haematopathology. Virchows Archiv. 2023;483(6):733–49.
Aussedat G, Traverse-Glehen A, Stamatoullas A, Molina T, Safar V, Laurent C, et al. Composite and sequential lymphoma between classical Hodgkin lymphoma and primary mediastinal lymphoma/diffuse large B-cell lymphoma, a clinico-pathological series of 25 cases. Br J Haematol. 2020;189(2):244–56.
Cook MR, Williams LS, Dorris CS, Luo Y, Makambi K, Dunleavy K. Improved survival for dose-intensive chemotherapy in primary mediastinal B-cell lymphoma: a systematic review and meta-analysis of 4,068 patients. Haematologica. 2024;109(3):846–56.
Hang H, Zhou H, Ma L. Prognostic factors and clinical survival outcome in patients with primary mediastinal diffuse large B-cell lymphoma in rituximab era: A population-based study. Medicine (Baltimore). 2024;103(8):e37238.
Zinzani PL, Santoro A, Gritti G, Brice P, Barr PM, Kuruvilla J, et al. Nivolumab combined with brentuximab vedotin for R/R primary mediastinal large B-cell lymphoma: a 3-year follow-up. Blood Adv. 2023;7(18):5272–80.
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