Skip to main content
SpringerLink
Log in

Primary mediastinal large B cell lymphoma with coexisting aberrations of C-MYC and BCL-2: a case report and literature review

  • Case Report
  • Published:
Medical Molecular Morphology Aims and scope Submit manuscript

Abstract

Primary mediastinal large B-cell lymphoma (PMLBCL) is an aggressive lymphoma characteristic with distinct clinical, morphologic, and immunophenotypic features. The rearrangements of C-MYC, BCL2 and/or BCL6 which are common in diffuse large B-cell lymphoma (DLBCL) are typically absent in PLMBCL. Here we proved a novel case of PMLBCL with concurrent rearrangements of C-MYC and BCL2. Histology showed typical pathomorphological features of PLMBCL. Fluorescent in situ hybridization (FISH) studies showed rearrangements of C-MYC and copy number variation (CNV) of BCL2 gene. It is not well known on the clinical significance of rearrangements of C-MYC and BCL2 genes in PMLBCL. The case gives evidence that cytogenetic testing is necessary for PMLBCLs to get precise clinical evaluation and appropriate treatment.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4

Similar content being viewed by others

References

  1. Addis BJ, Isaacson PG (1986) Large cell lymphoma of the mediastinum: a B-cell tumour of probable thymic origin. Histopathology 10:379–390

    Article  CAS  Google Scholar 

  2. Miller JB, Variakojis D, Bitran JD, Sweet DL, Kinzie JJ, Golomb HM, Ultmann JE (2015) Diffuse histiocytic lymphoma with sclerosis: a clinicopathologic entity frequently causing superior venacaval obstruction. Cancer 47:748–756

    Article  Google Scholar 

  3. Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW (2008) WHO Classification of tumours of haematopoietic and lymphoid tissues: International Agency for Research on Cancer. WHO, Lyon

    Google Scholar 

  4. Rosenwald A, Wright G, Leroy K, Yu X, Gaulard P, Gascoyne RD, Chan WC, Zhao T, Haioun C, Greiner TC (2003) 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 198:851–862

    Article  CAS  Google Scholar 

  5. Perrone T, Frizzera G, Rosai J (1986) Mediastinal diffuse large-cell lymphoma with sclerosis. A clinicopathologic study of 60 cases. Am J Surg Pathol 10:176–191

    Article  CAS  Google Scholar 

  6. Savage KJ, Monti S, Kutok JL, Cattoretti G, Neuberg D, De LL, Kurtin P, Dal CP, Ladd C, Feuerhake F (2003) 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 102:3871–3879

    Article  CAS  Google Scholar 

  7. Hutchinson CB, Wang E (2011) Primary mediastinal (thymic) large B-cell lymphoma: a short review with brief discussion of mediastinal gray zone lymphoma. Arch Pathol Lab Med 135:394–398

    PubMed  Google Scholar 

  8. Cazals-Hatem D, Lepage E, Brice P, Ferrant A, D'Agay MF, Baumelou E, Brière J, Blanc M, Gaulard P, Biron P (1996) Primary mediastinal large B-cell lymphoma. A clinicopathologic study of 141 cases compared with 916 nonmediastinal large B-cell lymphomas, a GELA ("Groupe d'Etude des Lymphomes de l'Adulte") study. Am J Surg Pathol 20:877–888

    Article  CAS  Google Scholar 

  9. Barth TF, Leithäuser F, Joos S, Bentz M, Möller P (2002) Mediastinal (thymic) large B-cell lymphoma: where do we stand? Lancet Oncol 3:229–234

    Article  Google Scholar 

  10. Johnson PW (2015) IV. Masses in the mediastinum: primary mediastinal lymphoma and intermediate types. Hematol Oncol 33:29–32

    Article  Google Scholar 

  11. Harris NL (2013) Shades of gray between large B-cell lymphomas and Hodgkin lymphomas: differential diagnosis and biological implications. Mod Pathol 26:S57–S70

    Article  CAS  Google Scholar 

  12. Win TT, Kamaludin Z, Husin A (2016) Primary mediastinal large B-cell lymphoma and its mimickers: a rare case report with literature review. Malays J Pathol 38:153–157

    CAS  PubMed  Google Scholar 

  13. Sesques P, Johnson NA (2016) Approach to the diagnosis and treatment of high-grade B cell lymphomas with MYC and BCL2 and/or BCL6 rearrangements. Blood 129:280–288

    Article  Google Scholar 

  14. Joos S, Otañojoos MI, Ziegler S, Brüderlein S, Du MS, Bentz M, Möller P, Lichter P (1996) Primary mediastinal (thymic) B-cell lymphoma is characterized by gains of chromosomal material including 9p and amplification of the REL gene. Blood 87:1571–1578

    Article  CAS  Google Scholar 

  15. Dunphy CH, O'Malley DP, Liang C, Fodrie TY, Perkins SL, Kathleen KR (2008) Primary mediastinal B-cell lymphoma: detection of BCL2 gene rearrangements by PCR analysis and FISH. J Hematopathol 1:77–84

    Article  Google Scholar 

  16. David KL, Rodney M, Tripp SR, Glenn MJ, Perkins SL, Mohamed S (2015) Clinicopathologic evaluation of MYC expression in primary mediastinal (thymic) large B-cell lymphoma. Am J Clin Pathol 143:598–604

    Article  Google Scholar 

  17. Eva S, Marie J, Edita K, Vratislav S, Marcela M, Roman K (2006) Primary mediastinal (thymic) large B-cell lymphoma with a der(14)t(8;14)(q24;q32) and a translocation of MYC to the derivative chromosome 14 with a deleted IgH locus. Cancer Genet. Cytogen 170:158–162

    Article  Google Scholar 

  18. Scarpa A, Borgato L, Chilosi M, Capelli P, Menestrina F, Bonetti F, Zamboni G, Pizzolo G, Hirohashi S, Fiore-Donati L (1991) Evidence of c-myc gene abnormalities in mediastinal large B-cell lymphoma of young adult age. Blood 78:780–788

    Article  CAS  Google Scholar 

  19. Tsang P, Cesarman E, Chadburn A, Liu YF, Knowles DM (1996) Molecular characterization of primary mediastinal B cell lymphoma. Am J Pathol 148:2017–2025

    CAS  PubMed  PubMed Central  Google Scholar 

  20. Scarpa A, Moore PS, Rigaud G, Inghirami G, Montresor M, Menegazzi M, Todeschini G, Menestrina F (2015) Molecular features of primary mediastinal B-cell lymphoma: involvement of p16(INK4A), p53 and c-myc. Br J Haematol 107:106–113

    Article  Google Scholar 

  21. Campuzano-Zuluaga G, Ortiz D, Peng JH, Francis IO, Fan YS, Barredo JC, Vega F, Chapman JR (2016) Primary mediastinal large B-cell lymphoma with translocations involving BCL6 and MYC (double-hit lymphoma). Am J Clin Pathol 145:710–716

    Article  Google Scholar 

  22. Iqbal J, Neppalli VT, Wright G, Dave BJ, Horsman DE, Rosenwald A, Lynch J, Hans CP, Weisenburger DD, Greiner TC, Gascoyne RD, Campo E, Ott G, Müller-Hermelink HK, Delabie J, Jaffe ES, Grogan TM, Connors JM, Vose JM, Armitage JO, Staudt LM, Chan WC (2006) BCL2 expression is a prognostic marker for the activated B-cell-like type of diffuse large B-cell lymphoma. J Clin Oncol 24:961–968

    Article  CAS  Google Scholar 

  23. Lenz G, Wright G, Dave SS, Xiao W, Powell J, Zhao H, Xu W, Tan B, Goldschmidt N, Iqbal J (2008) Stromal gene signatures in large-B-cell lymphomas. N Engl J Med 359:2313–2323

    Article  CAS  Google Scholar 

Download references

Funding

This work was supported by the National Natural Science Foundation of China (NSFC) (Grant number: 81600117). The FISH analysis in this study was supported by this funder. The funder had no role in study design, data collection and analysis, decision to publish, or preparation of manuscript.

Author information

Authors and Affiliations

  1. Department of Hematology, The First Affiliated Hospital of China Medical University, No.155, Nanjing North Street, Shenyang, 110001, Liaoning, People’s Republic of China

    Yanyan Jiang, Wenbin Mo, Ying Liang, Yan Li & Rui Zhang

  2. Department of Pathology, The First Affiliated Hospital and College of Basic Medical Sciences of China Medical University, Shenyang, 110001, Liaoning, People’s Republic of China

    Yuan Miao

Authors
  1. Yanyan Jiang
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Wenbin Mo
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Yuan Miao
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Ying Liang
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Yan Li
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Rui Zhang
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Rui Zhang.

Ethics declarations

Conflict of interest

All authors have no conflict of interest to report.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Jiang, Y., Mo, W., Miao, Y. et al. Primary mediastinal large B cell lymphoma with coexisting aberrations of C-MYC and BCL-2: a case report and literature review. Med Mol Morphol 53, 124–129 (2020). https://doi.org/10.1007/s00795-019-00237-2

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00795-019-00237-2

Keywords

Search

Navigation