Progress in diagnosis and treatment of primary mediastinal large B-cell lymphoma

doi:10.16138/j.1673-6087.2025.06.11

Abstract

Abstract:

Primary mediastinal large B-cell lymphoma (PMBCL) is an aggressive non-Hodgkin lymphoma originating from thymic B cells. It typically presents as a rapidly growing anterior mediastinal mass, often accompanied by local symptoms such as chest tightness and shortness of breath, as well as systemic symptoms like fever and night sweats. R-CHOP (rituximab + cyclophosphamide + doxorubicin + vincristine + prednisone) and R-EPOCH (etoposide + prednisone + vincristine + cyclophosphamide + doxorubicin + rituximab) are the commonly used first-line treatments. Formulating precise diagnostic and therapeutic strategies requires a comprehensive understanding of its unique clinical, pathological, and molecular characteristics. This review delivers a comprehensive overview of the clinical features, pathological characteristics, molecular mechanisms, differential diagnosis, and recent advances in treatment of PMBCL, aiming to provide a reference for accurate diagnosis, optimized patient management, and improved therapeutic outcomes.

Key words: Primary mediastinal large B-cell lymphoma, Lymphoma, Large B-cell lymphoma, Pathological diagnosis, CHOP

CLC Number:

R733

CAI Lu, YI Hongmei, WANG Chaofu. Progress in diagnosis and treatment of primary mediastinal large B-cell lymphoma[J]. Journal of Internal Medicine Concepts & Practice, 2026, 20(06): 490-494.

References 29

[1]	Alaggio R, Amador C, Anagnostopoulos I, et al. The 5^th edition of the World Health Organization classification of haematolymphoid tumours: lymphoid neoplasms[J]. Leukemia, 2022, 36(7):1720-1748. doi: 10.1038/s41375-022-01620-2
[2]	Swerdlow SH, Campo E, Pileri SA, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms[J]. Blood, 2016, 127(20):2375-2390. doi: 10.1182/blood-2016-01-643569
[3]	Steidl C, Gascoyne RD. The molecular pathogenesis of primary mediastinal large B-cell lymphoma[J]. Blood, 2011, 118(10):2659-2669. doi: 10.1182/blood-2011-05-326538
[4]	Noerenberg D, Briest F, Hennch C, et al. Genetic characterization of primary mediastinal B-cell lymphoma: pathogenesis and patient outcomes[J]. J Clin Oncol, 2024, 42(4):452-466. doi: 10.1200/JCO.23.01053
[5]	李倩玉, 奚豪, 韩冬艳, 等. 灰区淋巴瘤2例临床病理分析[J]. 诊断学理论与实践, 2012, 11(4):349-353. doi: 10.3969/j.issn.1671-2870.2012.04.07
	Li QY, Xi H, Han DY, et al. Grey zone lymphoma (GZL): report of 2 cases[J]. J Diagn Concepts Pract, 2012, 11(4):349-353. doi: 10.3969/j.issn.1671-2870.2012.04.07
[6]	叶振邦, 黄文亭. 弥漫大B细胞淋巴瘤与肿瘤纯度病理研究进展[J]. 临床与实验病理学杂志, 2024, 40(1):77-80. doi: 10.13315/j.cnki.cjcep.2024.01.017
	Ye ZB, Huang WT. Research progress on tumor purity in diffuse large B-cell lymphoma pathology[J]. J Clin Exp Pathol, 2024, 40(1):77-80. doi: 10.13315/j.cnki.cjcep.2024.01.017
[7]	El-Mallawany NK, Giulino-Roth L, Burke JM, et al. Mature B-cell lymphomas in adolescents and young adults[J]. EJHaem, 2023, 4(4):912-920.
[8]	Malysz J, Erdman P, Klapper J, et al. Clinical implications of CD30 expression in aggressive B-cell lymphomas[J]. Clin Lymphoma Myeloma Leuk, 2016, 16(8):429-433. doi: 10.1016/j.clml.2016.04.011
[9]	Marx A, Ströbel P, Badve SS, et al. ITMIG consensus statement on the use of the WHO histological classification of thymoma and thymic carcinoma: refined definitions, histological criteria, and reporting[J]. J Thorac Oncol, 2014, 9(5):596-611. doi: 10.1097/JTO.0000000000000154
[10]	李金航, 曹晨, 封琳, 等. 240例性腺外生殖细胞肿瘤患者的临床病理特点和免疫组化分析[J]. 解放军医学院学报, 2022, 43(9):932-936. doi: 10.3969/j.issn.2095-5227.2022.09.004
	Li JH, Cao C, Feng L, et al. Clinicopathological features and immunohistochemical analysis of extragonadal germ cell tumors: a series of 240 cases[J]. Acad J Chin PLA Med Sch, 2022, 43(9):932-936. doi: 10.3969/j.issn.2095-5227.2022.09.004
[11]	Bouttell J, Fraser H, Goodlad JR, et al. Adding a gene expression profile test to aid differential diagnosis and treatment in aggressive large B-cell lymphoma: an early exploratory economic evaluation[J]. Appl Health Econ Health Policy, 2024, 22(2):243-254. doi: 10.1007/s40258-023-00845-1
[12]	Kato K, Nakamura S, Wakana A, et al. Pembrolizumab in Japanese patients with primary mediastinal large B-cell lymphoma: results from the KEYNOTE-A33 study[J]. Int J Clin Oncol, 2024, 29(12):1977-1983. doi: 10.1007/s10147-024-02627-8
[13]	Lorenzi L, Lonardi S, Bonezzi M, et al. Immunoglobulin light chain transcript detection by ultrasensitive RNA in situ hybridization for B-cell lymphoma diagnosis[J]. Virchows Arch, 2024, 485(1):43-51. doi: 10.1007/s00428-023-03682-8
[14]	Quan L, He Z, Zuo X, et al. Treatment strategies and survival outcomes of primary mediastinal large B-cell lymphoma[J]. Hematology, 2023, 28(1):2217396. doi: 10.1080/16078454.2023.2217396
[15]	Gullickson C, Kersun L, Reilly A, et al. Isolated central nervous system relapse in two adolescents with primary mediastinal large B-cell lymphoma after treatment with R-DA-EPOCH[J]. Pediatr Blood Cancer, 2024, 71(8):e31065
[16]	Volzone F, Becchimanzi C, Crisci S, et al. Long-term complete remission in a patient with high-risk primary mediastinal B-cell lymphoma and iatrogenic symptomatic bradycardia after only two courses of DA-EPOCH-R followed by chemo-free treatment[J]. Ann Hematol, 2024, 103(11):4759-4764. doi: 10.1007/s00277-024-05994-4
[17]	Malenda A, Kołkowska-Leśniak A, Puła B, et al. Outcomes of treatment with dose-adjusted EPOCH-R or R-CHOP in primary mediastinal large B-cell lymphoma[J]. Eur J Haematol, 2020, 104(1):59-66.
[18]	Shah NN, Szabo A, Huntington SF, et al. R-CHOP versus dose-adjusted R-EPOCH in frontline management of primary mediastinal B-cell lymphoma: a multi-centre analysis[J]. Br J Haematol, 2018, 180(4):534-544.
[19]	Vassilakopoulos T, Ferhanoglu B, Horowitz NA, et al. P1229: Rituximab-dose-adjusted EPOCH (R-DA-EPOCH) in primary mediastinal large B-cell lymphoma (PMLBCL): real-life experience on 225 patients from 4 countries[J]. Hemasphere, 2022, 6(Suppl):1114-1115. doi: 10.1097/01.hs9.0000847780.61937.3d
[20]	Martelli M, Ceriani L, Ciccone G, et al. Omission of radiotherapy in primary mediastinal B-cell lymphoma: IELSG37 trial results[J]. J Clin Oncol, 2024, 42(34):4071-4083. doi: 10.1200/JCO-24-01373
[21]	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[J]. Medicine (Baltimore), 2024, 103(8):e37238. doi: 10.1097/md.0000000000037238
[22]	Cook MR, Williams LS, Dorris CS, et al. Improved survival for dose-intensive chemotherapy in primary mediastinal B-cell lymphoma: a systematic review and meta-analysis of 4, 068 patients[J]. Haematologica, 2024, 109(3):846-856. doi: 10.3324/haematol.2023.283446
[23]	Takaura K, Ando H, Ganoza ER. Pharmacological characteristics and clinical outcomes of Epcoritamab (recombinant) (Epkinly® subcutaneous injection) for malignant lymphoma[J]. Nihon Yakurigaku Zasshi, 2024, 159(1):61-68. doi: 10.1254/fpj.23076
[24]	Abramson JS, Palomba ML, Gordon LI, et al. Lisocabtagene maraleucel for patients with relapsed or refractory large B-cell lymphomas (TRANSCEND NHL 001): a multicentre seamless design study[J]. Lancet, 2020, 396(10254):839-852. doi: 10.1016/S0140-6736(20)31366-0
[25]	Lee D, Goyal A, Wang WL, et al. Lisocabtagene maraleucel for treatment of relapsed and refractory primary mediastinal large B-cell lymphoma in an adolescent patient[J]. EJHaem, 2024, 5(1):153-156. doi: 10.1002/jha2.859
[26]	Chiappella A, Casadei B, Chiusolo P, et al. Axicabtagene ciloleucel treatment is more effective in primary mediastinal large B-cell lymphomas than in diffuse large B-cell lymphomas: the Italian CART-SIE study[J]. Leukemia, 2024, 38(5):1107-1114. doi: 10.1038/s41375-024-02213-x
[27]	Zinzani PL, Thieblemont C, Melnichenko V, et al. Pembrolizumab in relapsed or refractory primary mediastinal large B-cell lymphoma: final analysis of KEYNOTE-170[J]. Blood, 2023, 142(2):141-145.
[28]	Yousefirizi F, Gowdy C, Klyuzhin IS, et al. Evaluating outcome prediction via baseline, end-of-treatment, and delta radiomics on PET-CT images of primary mediastinal large B-cell lymphoma[J]. Cancers (Basel), 2024, 16(6):1090. doi: 10.3390/cancers16061090
[29]	Tou C, Ma L. PD-1 inhibitor in the treatment of relapsed primary mediastinal large B-cell lymphoma follow up by ¹⁸F-FDG PET/CT: a case report and literature review[J]. Radiol Case Rep, 2024, 19(10):4497-4503. doi: 10.1016/j.radcr.2024.07.053