Journal of Internal Medicine Concepts & Practice >

2026 , Vol. 20 >Issue 06: 490 - 494

DOI: https://doi.org/10.16138/j.1673-6087.2025.06.11

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

  • CAI Lu ,
  • YI Hongmei ,
  • WANG Chaofu
Expand
  • Department of Pathology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200025, China

Received date: 2024-11-05

  Online published: 2026-01-30

Copyright

, 2025, Copyright reserved © 2025.
Fold

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

Cite this article

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 . DOI: 10.16138/j.1673-6087.2025.06.11

References

[1] Alaggio R, Amador C, Anagnostopoulos I, et al. The 5th edition of the World Health Organization classification of haematolymphoid tumours: lymphoid neoplasms[J]. Leukemia, 2022, 36(7):1720-1748.
[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.
[3] Steidl C, Gascoyne RD. The molecular pathogenesis of primary mediastinal large B-cell lymphoma[J]. Blood, 2011, 118(10):2659-2669.
[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.
[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.
  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.
[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.
  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.
[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.
[9] Marx A, Str?bel 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.
[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.
  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.
[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.
[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.
[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.
[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.
[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.
[17] Malenda A, Ko?kowska-Le?niak A, Pu?a 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.
[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.
[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.
[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.
[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.
[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.
[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.
[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.
[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.
[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.
Options
Outlines

/

〈 〉