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.

Amyloidosis Research and Treatment Center Department of molecular Medicine University of Pavia Fondazione IRCCS Policlinico San Matteo Pavia Italy

Berlín Germany

Clinica São Germano São Paulo Brazil

Dana Farber Cancer Institute Harvard Medical School Boston MA USA

Department Hematology and Immunology Vriji Universiteit Brussel Brussels Belgium

Department of Clinical Hematology and Cellular Therapy Hospital Saint Antoine Sorbonne University París France

Department of Clinical Hematology Centre Hospitalier Universitaire de Liège Liège Belgium

Department of Clinical Molecular Medicine St Olavs Hospital NTNU Trondheim Trondheim Norway

Department of Haematooncology University of Ostrava Ostrava Czech Republic

Department of Hematologic Oncology and Blood Disorders Levine Cancer institute Atrium Health Charlotte NC USA

Department of Hematology Amsterdam UMC VU University Amsterdam the Netherlands

Department of Hematology Ankara University Ankara Turkey

Department of Hematology Hospital Clínic IDIBAPS University of Barcelona Barcelona Spain

Department of Internal Medicine 2 University Hospital Würzburg Würzburg Germany

Department of Medicine Karolinska Institutet Huddinge Stockholm Sweden

Division of Hematology Mayo Clínic Rochester MN USA

Erasmus MC Cancer Institute Erasmus University of Rotterdam Rotterdam the Netherlands

Hematology and Medical Oncology Department of Clinical Therapeutics National and Kapodistrian University of Athens Alexandra Hospital Athens Greece

Hematology Department University Hospital Hotel Dieu Nantes France

IBSAL Cancer Research Center University Hospital of Salamanca Salamanca Spain

Instituto Português de Oncologia Lisboa Portugal

Interdisciplinary Tumor Center University of Freiburg Freiburg Germany

Istituto di Ematologia Seràgnoli Dipartamento di Medicina Specialistica Diagnostica e Sperimentale Università degli Studi Azienda Ospedaliero Universitaria di Bologna Bologna Italy

John Theurer Cancer Hackensack Meridian School of Medicine Hackensat NJ USA

Medical College Department of Hematology Jagiellanian University Krakow Poland

Multiple Myeloma and Amyloidosis Service Columbia University New York NY USA

Myeloma Unit Città della Salute e della Scienza University of Torino Torino Italy

Poitiers University Hospital Poitiers France

Princess Margaret Cancer Center University of Toronto Toronto Canada

University Medical Center Hamburg Eppendorf Hamburg Germany

University of Maryland at Baltimore Baltimore MD USA

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$a Expert review on soft-tissue plasmacytomas in multiple myeloma: definition, disease assessment and treatment considerations / $c L. Rosiñol, M. Beksac, E. Zamagni, NWCJ. Van de Donk, KC. Anderson, A. Badros, J. Caers, M. Cavo, MA. Dimopoulos, A. Dispenzieri, H. Einsele, M. Engelhardt, C. Fernández de Larrea, G. Gahrton, F. Gay, R. Hájek, V. Hungria, A. Jurczyszyn, N. Kröger, RA. Kyle, F. Leal da Costa, X. Leleu, S. Lentzsch, MV. Mateos, G. Merlini, M. Mohty, P. Moreau, L. Rasche, D. Reece, O. Sezer, P. Sonneveld, SZ. Usmani, K. Vanderkerken, DH. Vesole, A. Waage, S. Zweegman, PG. Richardson, J. Bladé

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$a 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.

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