Primary Mediastinal (Thymic) Large B-Cell Lymphoma: Experience in Treating 131 Patients at a National Medical Research Center in Russia

IZ Zavodnova, MYu Kichigina, EV Paramonova, YuE Ryabukhina, OA Kolomeitsev, OP Trofimova, NV Volkova, YuI Pryamikova, NV Kokosadze, GS Tumyan

NN Blokhin National Medical Cancer Research Center, 23 Kashirskoye sh., Moscow, Russian Federation, 115478

For correspondence: Prof. Gayane Sergeevna Tumyan, MD, PhD, 23 Kashirskoye sh., Moscow, Russian Federation, 115478; Tel.: +7(499)324-98-29; e-mail:

For citation: Zavodnova IZ, Kichigina MYu, Paramonova EV, et al. Primary Mediastinal (Thymic) Large B-Cell Lymphoma: Experience in Treating 131 Patients at a National Medical Research Center in Russia. Clinical oncohematology. 2019;12(1):59–67.

DOI: 10.21320/2500-2139-2019-12-1-59-67


Background. Primary mediastinal (thymic) large B-cell lymphoma (PMBCL) is one of the primary extranodal tumors and originates from B-cells of thymic medulla. This disease is characterized by specific immunomorphologic and genetic features which distinguish it from other malignant lymphoproliferative disorders with similar parameters. Standard PMBCL treatment consists of immunochemotherapy and subsequent radiotherapy of residual mediastinal tumor. The advantages of one immunochemotherapy regimens over the other in controlled studies have not been shown.

Aim. To study current approaches to chemoradiation in PMBCL patients with an attempt to individualize them focusing on various prognostic factors.

Materials & Methods. The data of 131 patients with newly diagnosed PMBCL were analyzed, all of them were treated at NN Blokhin National Medical Cancer Research Center in the period from 2000 to 2017. More than a half were women (58 %), median age was 30 years (range 16–70). At different historical periods PMBCL treatment was performed using different immunochemotherapy regimens: MACOP-B±R in 55 (42 %) patients, R-CHOP  in 40 (30.5  %) patients, and R-DA-EPOCH  in 36 (27.5 %) patients.

Results. In general, the treatment of all PMBCL patients (n = 131) appeared to be highly effective. The remission rate was 87 %, 3-year progression-free survival (PFS) and overall survival (OS) was 78 % and 88 %, respectively. With median follow-up of 37 months relapses and progression of the disease were detected in 17 (13 %) out of 131 patients within a period of 13 months after initiation of antitumor treatment. There was not a single case of late relapse. The treatment of relapsed patients was not effective: 12-month OS was not higher than 37 %. Intensive immunochemotherapy regimens (МАСОР-В±R, R-DA-EPOCH) do not differ in their effectiveness, but they have significant advantages over the standard R-CHOP regimen. The results of positron emission tomography (PET) considered to be an important prognostic factor in PMBCL treatment: 3-year PFS in the PET-negative group was 92 % vs. 26 % in the PET-positive group.

Conclusion. The optimal algorithm of PMBCL treatment was elaborated with consideration of clinical factors, immunochemotherapy programs, degrees of tumor regression, its metabolic activity, and radiotherapy method and irradiation area.

Keywords: primary mediastinal (thymic) large B-cell lymphoma, treatment algorithm, prognostic factors.

Received: October 19, 2018

Accepted: December 23, 2018

Read in PDF 


  1. Moller P, Lammler B, Herrmann B, et al. The primary mediastinal clear cell lymphoma of B-cell type has variable defects in MHC antigen expression. Immunology. 1986;59(3):411–7. doi: 10.1007/bf00705408.

  2. Hamlin PA, Portlock CS, Straus DJ, et al. Primary mediastinal large B-cell lymphoma: optimal therapy and prognostic factor analysis in 141 consecutive patients treated at Memorial Sloan Kettering from 1980 to 1999. Br J Haematol. 2005;130(5):691–9. doi: 10.1111/j.1365-2141.2005.05661.x.

  3. Jacobson JO, Aisenberg AC, Lamarre L, et al. Mediastinal large cell lymphoma. An uncommon subset of adult lymphoma curable with combined modality therapy. Cancer. 1988;62(9):1893–8. doi: 10.1002/1097-0142(19881101)62:9<1893::AID-CNCR2820620904>3.0.CO;2-X.

  4. Zinzani PL, Martelli M, Magagnoli M, et al. Treatment and clinical management of primary mediastinal large B-cell lymphoma with sclerosis: MACOP-B regimen and mediastinal radiotherapy monitored by (67)Gallium scan in 50 patients. Blood. 1999;94(10):3289–93.

  5. Bishop PC, Wilson WH, Pearson D, et al. CNS involvement in primary mediastinal large B-cell lymphoma. J Clin Oncol. 1999;17(8):2479–85. doi: 1200/jco.1999.17.8.2479.

  6. Rosenwald A, Wright G, Leroy K, et al. Molecular diagnosis of primary mediastinal B cell lymphoma identifies a clinically favorable subgroup of diffuse large B cell lymphoma related to Hodgkin lymphoma. J Exp Med. 2003;198(6):851–62. doi: 10.1084/jem.20031074.

  7. Levitt LJ, Aisenberg AC, Harris NL, et al. Primary non-Hodgkin’s lymphoma of the mediastinum. Cancer. 1982;50(11):2486–92. doi: 10.1002/1097-0142(19821201)50:11<2486::AID-CNCR2820501138>3.0.CO;2-G.

  8. Zinzani PL, Stefoni V, Finolezzi E, et al. Rituximab combined with MACOP-B or VACOP-B and radiation therapy in primary mediastinal large B-cell lymphoma: a retrospective study. Clin Lymph Myel. 2009;9(5):381–5. doi: 10.3816/CLM.2009.n.074.

  9. Todeschini G, Ambrosetti A, Meneghini V, et al. Mediastinal large-B-cell lymphoma with sclerosis: a clinical study of 21 patients. J Clin Oncol. 1990;8(5):804–8. doi: 1200/jco.1990.8.5.804.

  10. Bertini M, Orsucci L, Vitolo U, et al. Stage II large B-cell lymphoma with sclerosis treated with MACOP-B. Ann Oncol. 1991;2(10):733–7. doi: 10.1093/oxfordjournals.annonc.a057853.

  11. Falini B, Venturi S, Martelli M, et al. Mediastinal large B-cell lymphoma: clinical and immunohistological findings in 18 patients treated with different third-generation regimens. Br J Haematol. 1995;89(4):780–9. doi: 10.1111/j.1365-2141.1995.tb08415.x.

  12. van Besien K, Kelta M, Bahaguna P. Primary mediastinal B-cell lymphoma: a review of pathology and management. J Clin Oncol. 2001;19(6):1855–64. doi: 10.1200/jco.2001.19.6.1855.

  13. Zinzani PL, Martelli M, Bendandi M, et al. Primary mediastinal large B-cell lymphoma with sclerosis: a clinical study of 89 patients treated with MACOP-B chemotherapy and radiation therapy. Haematologica. 2001;86(2):187–91.

  14. A predictive model for aggressive non-Hodgkin’s lymphoma. The international non-Hodgkin lymphoma prognostic factors project. NEJM. 1993;329(14):987–94. doi: 10.1056/nejm199309303291402.

  15. Barrington SF, Mikhaeel NG, Kostakoglu L, et al. Role of Imaging in the Staging and Response Assessment of Lymphoma: Consensus of the International Conference on Malignant Lymphomas Imaging Working Group. J Clin Oncol. 2014;32(27):3048–58. doi: 10.1200/jco.2013.53.5229.

  16. Cox JD, Stetz J, Pajak TF. Toxicity criteria of the Radiation Therapy Oncology Group (RTOG) and the European Organization for Research and Treatment of Cancer (EORTC). Int J Radiat Oncol Biol Phys. 1995;31(5):1341–6. doi: 10.1016/0360-3016(95)00060-c.