Expert review on soft-tissue plasmacytomas in multiple myeloma: definition, disease assessment and treatment considerations

Laura Rosiñol, Meral Beksac, Elena Zamagni, Niels W.C.J. Van de Donk, Kenneth C. Anderson, Ashraf Badros, Jo Caers, Michele Cavo, Meletios Athanasios Dimopoulos, Angela Dispenzieri, Hermann Einsele, Monika Engelhardt, Carlos Fernández de Larrea, Gösta Gahrton, Francesca Gay, Roman Hájek, Vania Hungria, Artur Jurczyszyn, Nicolaus Kröger, Robert A. KyleFernando Leal da Costa, Xavier Leleu, Suzanne Lentzsch, Maria V. Mateos, Giampaolo Merlini, Mohamad Mohty, Philippe Moreau, Leo Rasche, Donna Reece, Orhan Sezer, Pieter Sonneveld, Saad Z. Usmani, Karin Vanderkerken, David H. Vesole, Anders Waage, Sonja Zweegman, Paul G. Richardson, Joan Bladé

Research output: Contribution to journalReview articleAcademicpeer-review

64 Citations (Scopus)


In this review, two types of soft-tissue involvement in multiple myeloma are defined: (i) extramedullary (EMD) with haematogenous spread involving only soft tissues and (ii) paraskeletal (PS) with tumour masses arising from skeletal lesions. The incidence of EMD and PS plasmacytomas at diagnosis ranges from 1·7% to 4·5% and 7% to 34·4% respectively. EMD disease is often associated with high-risk cytogenetics, resistance to therapy and worse prognosis than in PS involvement. In patients with PS involvement a proteasome inhibitor-based regimen may be the best option followed by autologous stem cell transplantation (ASCT) in transplant eligible patients. In patients with EMD disease who are not eligible for ASCT, a proteasome inhibitor-based regimen such as lenalidomide-bortezomib-dexamethasone (RVD) may be the best option, while for those eligible for high-dose therapy a myeloma/lymphoma-like regimen such as bortezomib, thalidomide and dexamethasone (VTD)-RVD/cisplatin, doxorubicin, cyclophosphamide and etoposide (PACE) followed by SCT should be considered. In both EMD and PS disease at relapse many strategies have been tried, but this remains a high-unmet need population.

Original languageEnglish
Pages (from-to)496-507
Number of pages12
JournalBritish journal of haematology
Issue number3
Publication statusPublished - Aug 2021


  • extramedullary disease
  • multiple myeloma
  • paraskeletal plasmacytomas
  • plasmacytoma
  • prognosis
  • soft tissue
  • treatment

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