International Journal of Hematology

, Volume 105, Issue 4, pp 440–444 | Cite as

Primary mediastinal large B-cell lymphoma in Japanese children and adolescents

  • Tomoo OsumiEmail author
  • Fumiko Tanaka
  • Tetsuya Mori
  • Reiji Fukano
  • Masahito Tsurusawa
  • Koichi Oshima
  • Atsuko Nakazawa
  • Ryoji Kobayashi
Original Article


This is the first case series to describe primary mediastinal large B-cell lymphoma (PMLBL) patients in children and adolescents in Asia. We retrospectively identified 17 PMLBL patients diagnosed between 1991 and 2014; in seven of these cases, the diagnosis was confirmed by central review, representing 1.0% of all NHL and 2.2% of all B-NHL cases registered. All patients were teenagers, including seven adolescents, with a median age of 14 years (range 12–18 years). Ten patients were male, and seven were female. The 5-year EFS and OS rates were 81.9 and 84.4%, respectively. All seven recent cases remain alive, of which three received rituximab combination therapy. Incidence, characteristics, and outcome varied considerably from those of Western populations. Further studies, including molecular analysis, are warranted.


Primary mediastinal large B-cell lymphoma PMLBL Children Adolescents Non-Hodgkin lymphoma 



Primary mediastinal large B-cell lymphoma


Non-Hodgkin lymphoma


Diffuse large B-cell lymphoma


B-Cell NHL


Overall survival


Event-free survival


Complete remission


Stem-cell transplantation


Hodgkin lymphoma



We would like to thank Dr. Daisuke Tomizawa of Children’s Cancer Center and Dr. Julian Tang of the Department of Education for Clinical Research, National Center for Child Health and Development, for proofreading and editing this manuscript. This research is supported by the Practical Research for Innovative Cancer Control from the Japan Agency for Medical Research and Development, AMED.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.


  1. 1.
    Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW (eds). WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, Fourth Edition, World Health Organization, 2008 pp 251–252.Google Scholar
  2. 2.
    Burkhardt B, Oschlies I, Klapper W, Zimmermann M, Woessmann W, Meinhardt A, et al. Non-Hodgkin’s lymphoma in adolescents: experiences in 378 adolescent NHL patients treated according to pediatric NHL-BFM protocols. Leukemia. 2011;25:153–60.CrossRefPubMedGoogle Scholar
  3. 3.
    Gerrard M, Waxman IM, Sposto R, Auperin A, Perkins SL, Goldman S, et al. Outcome and pathologic classification of children and adolescents with mediastinal large B-cell lymphoma treated with FAB/LMB96 mature B-NHL therapy. Blood. 2013;121:278–85.CrossRefPubMedPubMedCentralGoogle Scholar
  4. 4.
    Cairo MS, Sposto R, Gerrard M, Auperin A, Goldman SC, Harrison L, et al. Advanced stage, increased lactate dehydrogenase, and primary site, but not adolescent age (≥15 years), are associated with an increased risk of treatment failure in children and adolescents with mature B-cell non-Hodgkin’s lymphoma: results of the FAB LMB 96 study. J Clin Oncol. 2012;30:387–93.CrossRefPubMedPubMedCentralGoogle Scholar
  5. 5.
    Woessmann W, Seidemann K, Mann G, Zimmermann M, Burkhardt B, Oschlies I, et al. The impact of the methotrexate administration schedule and dose in the treatment of children and adolescents with B-cell neoplasms: a report of the BFM Group Study NHL-BFM95. Blood. 2005;105:948–58.CrossRefPubMedGoogle Scholar
  6. 6.
    Patte C, Auperin A, Gerrard M, Michon J, Pinkerton R, Sposto R, et al. Results of the randomized international FAB/LMB96 trial for intermediate risk B-cell non-Hodgkin lymphoma in children and adolescents: it is possible to reduce treatment for the early responding patients. Blood. 2007;109:2773–80.PubMedPubMedCentralGoogle Scholar
  7. 7.
    Cairo MS, Gerrard M, Sposto R, Auperin A, Pinkerton CR, Michon J, et al. Results of a randomized international study of high-risk central nervous system B non-Hodgkin lymphoma and B acute lymphoblastic leukemia in children and adolescents. Blood. 2007;109:2736–43.PubMedPubMedCentralGoogle Scholar
  8. 8.
    Tsurusawa M, Katano N, Hirota T, Ito M, Yanase T, Asami K. et al [Studies of childhood non-Hodgkin’s lymphoma–treatment results with the CCLSG NHL 960 protocol. Children’s Cancer and Leukemia Study Group (CCLSG)]. Rinsho Ketsueki. 1998;39:1092–8.PubMedGoogle Scholar
  9. 9.
    Kikuchi A, Mori T, Fujimoto J, Kumagai M, Sunami S, Okimoto Y, et al. Outcome of childhood B-cell non-Hodgkin lymphoma and B-cell acute lymphoblastic leukemia treated with the Tokyo Children’s Cancer Study Group NHL B9604 protocol. Leuk Lymphoma. 2008;49:757–62.CrossRefPubMedGoogle Scholar
  10. 10.
    Fujita N, Kobayashi R, Takimoto T, Nakagawa A, Ueda K, Horibe K. Results of the Japan Association of Childhood Leukemia Study (JACLS) NHL-98 protocol for the treatment of B-cell non-Hodgkin lymphoma and mature B-cell acute lymphoblastic leukemia in childhood. Leuk Lymphoma. 2011;52:223–9.CrossRefPubMedGoogle Scholar
  11. 11.
    Tsurusawa M, Mori T, Kikuchi A, Mitsui T, Sunami S, Kobayashi R, et al. Improved treatment results of children with B-cell non-Hodgkin lymphoma: a report from the Japanese Pediatric Leukemia/Lymphoma Study Group B-NHL03 study. Pediatr Blood Cancer. 2014;61:1215–21.CrossRefPubMedGoogle Scholar
  12. 12.
    Lones MA, Perkins SL, Sposto R, Kadin ME, Kjeldsberg CR, Wilson JF, et al. Large-cell lymphoma arising in the mediastinum in children and adolescents is associated with an excellent outcome: a Children’s Cancer Group report. J Clin Oncol. 2000;18:3845–53.CrossRefPubMedGoogle Scholar
  13. 13.
    Horibe K, Saito AM, Takimoto T, Tsuchida M, Manabe A, Shima M, et al. Incidence and survival rates of hematological malignancies in Japanese children and adolescents (2006-2010): based on registry data from the Japanese Society of Pediatric Hematology. Int J Hematol. 2013;98:74–88.CrossRefPubMedGoogle Scholar
  14. 14.
    Koga Y, Kumagai M, Takimoto T, Mimaya J, Nakazawa A, Horibe K, et al. Retrospective analysis of 157 patients with pediatric Hodgkin lymphoma in Japan: investigation by four pediatric cancer study groups. Rinsho Ketsueki. 2012;53:443–9.PubMedGoogle Scholar
  15. 15.
    Savage KJ, Monti S, Kutok JL, Cattoretti G, Neuberg D, De Leval L, et al. The molecular signature of mediastinal large B-cell lymphoma differs from that of other diffuse large B-cell lymphomas and shares features with classical Hodgkin lymphoma. Blood. 2003;102:3871–9.CrossRefPubMedGoogle Scholar
  16. 16.
    Rosenwald A, Wright G, Leroy K, Yu X, Gaulard P, Gascoyne RD, 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;198:851–62.CrossRefPubMedPubMedCentralGoogle Scholar
  17. 17.
    Dunleavy K, Pittaluga S, Maeda LS, Advani R, Chen CC, Hessler J, et al. Dose-adjusted EPOCH-rituximab therapy in primary mediastinal B-cell lymphoma. N Engl J Med. 2013;368:1408–16.CrossRefPubMedPubMedCentralGoogle Scholar
  18. 18.
    Woessmann W, Lisfeld J, Burkhardt B. Therapy in primary mediastinal B-cell lymphoma. N Engl J Med. 2013;369:282.CrossRefPubMedGoogle Scholar

Copyright information

© The Japanese Society of Hematology 2016

Authors and Affiliations

  • Tomoo Osumi
    • 1
    Email author
  • Fumiko Tanaka
    • 2
  • Tetsuya Mori
    • 3
  • Reiji Fukano
    • 4
  • Masahito Tsurusawa
    • 5
  • Koichi Oshima
    • 6
  • Atsuko Nakazawa
    • 7
  • Ryoji Kobayashi
    • 8
  1. 1.Children’s Cancer CenterNational Center for Child Health and DevelopmentTokyoJapan
  2. 2.Department of PediatricsSaiseikai Yokohamashi Nanbu HospitalYokohamaJapan
  3. 3.Department of PediatricsSt. Marianna University School of MedicineKawasakiJapan
  4. 4.Department of PediatricsNational Kyushu Cancer CenterFukuokaJapan
  5. 5.Department of PediatricsAichi Medical UniversityNagakuteJapan
  6. 6.Department of PathologyKurume University School of MedicineFukuokaJapan
  7. 7.Department of PathologyNational Center for Child Health and DevelopmentTokyoJapan
  8. 8.Department of PediatricsSapporo Hokuyu HospitalSapporoJapan

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