Advances in Therapy

, Volume 28, Supplement 7, pp 1–6 | Cite as

Multiple myeloma with extramedullary disease



Plasmacytoma is a tumor mass consisting of atypical plasma cells. Incidence of plasmacytomas associated with multiple myeloma range from 7% to 17% at diagnosis and from 6% to 20% during the course of the disease. In both situations, occurrence of extramedullary disease has been consistently associated with a poorer prognosis of myeloma. Extramedullary relapse or progression occurs in a variety of clinical circumstances and settings, and therefore requires individualization of treatment. Alkylating agents, bortezomib, and immunomodulatory drugs, along with corticoids, have been used to treat extramedullary relapse but, because of the relatively low frequency or detection rate of extramedullary relapse, no efficacy data are available from controlled studies in this setting.


extramedullary disease multiple myeloma plasmacytoma 


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  1. 1.
    Dores GM, Landgren O, McGlynn KA, Curtis RE, Linet MS, Devesa SS. Plasmacytoma of bone, extramedullary plasmacytoma, and multiple myeloma: incidence and survival in the United States, 1992–2004. Br J Haematol. 2009;144:86–94.PubMedCrossRefGoogle Scholar
  2. 2.
    Varettoni M, Corso A, Pica G, Mangiacavalli S, Pascutto C, Lazzarino M. Incidence, presenting features and outcome of extramedullary disease in multiple myeloma: a longitudinal study on 1003 consecutive patients. Ann Oncol. 2010;21:325–330.PubMedCrossRefGoogle Scholar
  3. 3.
    Cerny J, Fadare O, Hutchinson L, Wang SA. Clinicopathological features of extramedullary recurrence/relapse of multiple myeloma. Eur J Haematol. 2008;81:65–69.PubMedCrossRefGoogle Scholar
  4. 4.
    Wu P, Davies FE, Boyd K, et al. The impact of extramedullary disease at presentation on the outcome of myeloma. Leuk Lymphoma. 2009;50:230–235.PubMedCrossRefGoogle Scholar
  5. 5.
    Sher T, Miller KC, Deeb G, Lee K, Chanan-Khan A. Plasma cell leukaemia and other aggressive plasma cell malignancies. Br J Haematol. 2010;150:418–427.PubMedCrossRefGoogle Scholar
  6. 6.
    Katodritou E, Gastari V, Verrou E, et al. Extramedullary (EMP) relapse in unusual locations in multiple myeloma: is there an association with precedent thalidomide administration and a correlation of special biological features with treatment and outcome? Leuk Res. 2009;33:1137–1140.PubMedCrossRefGoogle Scholar
  7. 7.
    Sheth N, Yeung J, Chang H. p53 nuclear accumulation is associated with extramedullary progression of multiple myeloma. Leuk Res. 2009;33:1357–1360.PubMedCrossRefGoogle Scholar
  8. 8.
    Dawson MA, Patil S, Spencer A. Extramedullary relapse of multiple myeloma associated with a shift in secretion from intact immunoglobulin to light chains. Haematologica. 2007;92:143–144.PubMedCrossRefGoogle Scholar
  9. 9.
    López-Anglada L, Gutiérrez NC, García JL, Mateos MV, Flores T, San Miguel JF. P53 deletion may drive the clinical evolution and treatment response in multiple myeloma. Eur J Haematol. 2010;84:359–361.PubMedCrossRefGoogle Scholar
  10. 10.
    Weber DM, Chen C, Niesvizky R, et al. Lenalidomide plus dexamethasone for relapsed multiple myeloma in North America. N Engl J Med. 2007;357:2133–2142.PubMedCrossRefGoogle Scholar
  11. 11.
    Dimopoulos MA, Chen C, Spencer A, et al. Long-term follow-up on overall survival from the MM-009 and MM-010 phase III trials of lenalidomide plus dexamethasone in patients with relapsed or refractory multiple myeloma. Leukemia. 2009;23:2147–2152.PubMedCrossRefGoogle Scholar
  12. 12.
    Rosinol L, Cibeira MT, Blade J, et al. Extramedullary multiple myeloma escapes the effect of thalidomide. Haematologica. 2004;89:832–836.PubMedGoogle Scholar
  13. 13.
    Nakazato T, Suzuki K, Mihara A, Sanada Y, Kakimoto T. Refractory plasmablastic type myeloma with multiple extramedullary plasmacytomas and massive myelomatous effusion: remarkable response with a combination of thalidomide and dexamethasone. Inter Med. 2009;48:1827–1832.CrossRefGoogle Scholar
  14. 14.
    Gonzalez-Porras JR, Gonzalez M, Garcia-Sanz R, et al. Thalidomide in combination with cyclophosphamide and dexamethasone (thacydex) is effective in soft-tissue plasmacytomas. Br J Haematol. 2002;119:883–884.PubMedCrossRefGoogle Scholar
  15. 15.
    Rosiñol L, Cibeira MT, Mateos, MV, et al. Results of pre-and post-autologous stem cell transplantation (asct) with three induction regimens in multiple myeloma (MM): superiority of VTD (bortezomib/thalidomide/dexamethasone) over TD and VBCMP/VBAD plus bortezomib. Haematologica. 2011;96(s1):69.Google Scholar
  16. 16.
    Chim CS, Ooi GC, Loong F, Au AW, Lie AK. Side effects and good effects from new chemotherapeutic agents. Case 3. Bortezomib in primary refractory plasmacytoma. J Clin Oncol. 2005;23:2426–2428.PubMedCrossRefGoogle Scholar
  17. 17.
    Paubelle E, Coppo P, Garderet L, et al. Complete remission with bortezomib on plasmocytomas in an end-stage patient with refractory multiple myeloma who failed all other therapies including hematopoietic stem cell transplantation: possible enhancement of graft-vs-tumor effect. Leukemia. 2005;19:1702–1704.PubMedCrossRefGoogle Scholar
  18. 18.
    Krauth MT, Bankier A, Valent P, Kalhs P, Drach J. Sustained remission including marked regression of a paravertebral plasmacytoma in a patient with heavily pretreated, relapsed multiple myeloma after treatment with bortezomib. Leuk Res. 2005;29:1473–1477.PubMedCrossRefGoogle Scholar
  19. 19.
    Laura R, Cibeira MT, Uriburu C, et al. Bortezomib: an effective agent in extramedullary disease in multiple myeloma. Eur J Haematol. 2006;76:405–408.PubMedCrossRefGoogle Scholar
  20. 20.
    Rajkumar SV, Jacobus S, Callander NS, et al. Lenalidomide plus high-dose dexamethasone versus lenalidomide plus low-dose dexamethasone as initial therapy for newly diagnosed multiple myeloma: an open-label randomised controlled trial. Lancet Oncol. 2010;11:29–37.PubMedCrossRefGoogle Scholar
  21. 21.
    Dimopoulos M, Spencer A, Attal M, et al. Lenalidomide plus dexamethasone for relapsed or refractory multiple myeloma. N Engl J Med. 2007;357:2123–2132.PubMedCrossRefGoogle Scholar
  22. 22.
    Wang M, Dimopoulos MA, Chen C, et al. Lenalidomide plus dexamethasone is more effective than dexamethasone alone in patients with relapsed or refractory multiple myeloma regardless of prior thalidomide exposure. Blood. 2008;112:4445–4451.PubMedCrossRefGoogle Scholar
  23. 23.
    Calvo-Villas JM, Alegre A, Calle C, Hernández MT, García-Sánchez R, Ramírez G. Lenalidomide is effective for extramedullary disease in relapsed or refractory multiple myeloma. Eur J Haematol. 2011;87:281–284.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Healthcare 2011

Authors and Affiliations

  1. 1.Department of Hematology, Hospital Germans Trias i PujolCarretera de CanyetBadalonaSpain

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