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

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Abstract

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.

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Abbreviations

PMLBL:

Primary mediastinal large B-cell lymphoma

NHL:

Non-Hodgkin lymphoma

DLBCL:

Diffuse large B-cell lymphoma

B-NHL:

B-Cell NHL

OS:

Overall survival

EFS:

Event-free survival

CR:

Complete remission

SCT:

Stem-cell transplantation

HL:

Hodgkin lymphoma

References

  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.

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  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.

    Article  PubMed  PubMed Central  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    CAS  PubMed  PubMed Central  Google Scholar 

  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.

    CAS  PubMed  PubMed Central  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  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.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  18. Woessmann W, Lisfeld J, Burkhardt B. Therapy in primary mediastinal B-cell lymphoma. N Engl J Med. 2013;369:282.

    Article  PubMed  Google Scholar 

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Acknowledgements

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.

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Authors and Affiliations

  1. Children’s Cancer Center, National Center for Child Health and Development, 2-10-1, Okura, Setagaya-ku, Tokyo, 157-0074, Japan

    Tomoo Osumi

  2. Department of Pediatrics, Saiseikai Yokohamashi Nanbu Hospital, Yokohama, Kanagawa, Japan

    Fumiko Tanaka

  3. Department of Pediatrics, St. Marianna University School of Medicine, Kawasaki, Kanagawa, Japan

    Tetsuya Mori

  4. Department of Pediatrics, National Kyushu Cancer Center, Fukuoka, Japan

    Reiji Fukano

  5. Department of Pediatrics, Aichi Medical University, Nagakute, Aichi, Japan

    Masahito Tsurusawa

  6. Department of Pathology, Kurume University School of Medicine, Fukuoka, Japan

    Koichi Oshima

  7. Department of Pathology, National Center for Child Health and Development, Tokyo, Japan

    Atsuko Nakazawa

  8. Department of Pediatrics, Sapporo Hokuyu Hospital, Sapporo, Hokkaido, Japan

    Ryoji Kobayashi

Authors
  1. Tomoo Osumi
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  2. Fumiko Tanaka
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  3. Tetsuya Mori
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  4. Reiji Fukano
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  5. Masahito Tsurusawa
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  6. Koichi Oshima
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  7. Atsuko Nakazawa
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  8. Ryoji Kobayashi
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Correspondence to Tomoo Osumi.

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Osumi, T., Tanaka, F., Mori, T. et al. Primary mediastinal large B-cell lymphoma in Japanese children and adolescents. Int J Hematol 105, 440–444 (2017). https://doi.org/10.1007/s12185-016-2147-3

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  • DOI: https://doi.org/10.1007/s12185-016-2147-3

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