TY - JOUR
T1 - Expert review on soft-tissue plasmacytomas in multiple myeloma
T2 - definition, disease assessment and treatment considerations
AU - Rosiñol, Laura
AU - Beksac, Meral
AU - Zamagni, Elena
AU - Van de Donk, Niels W.C.J.
AU - Anderson, Kenneth C.
AU - Badros, Ashraf
AU - Caers, Jo
AU - Cavo, Michele
AU - Dimopoulos, Meletios Athanasios
AU - Dispenzieri, Angela
AU - Einsele, Hermann
AU - Engelhardt, Monika
AU - Fernández de Larrea, Carlos
AU - Gahrton, Gösta
AU - Gay, Francesca
AU - Hájek, Roman
AU - Hungria, Vania
AU - Jurczyszyn, Artur
AU - Kröger, Nicolaus
AU - Kyle, Robert A.
AU - Leal da Costa, Fernando
AU - Leleu, Xavier
AU - Lentzsch, Suzanne
AU - Mateos, Maria V.
AU - Merlini, Giampaolo
AU - Mohty, Mohamad
AU - Moreau, Philippe
AU - Rasche, Leo
AU - Reece, Donna
AU - Sezer, Orhan
AU - Sonneveld, Pieter
AU - Usmani, Saad Z.
AU - Vanderkerken, Karin
AU - Vesole, David H.
AU - Waage, Anders
AU - Zweegman, Sonja
AU - Richardson, Paul G.
AU - Bladé, Joan
N1 - Funding Information:
This work has been supported in part by grant PI12/01093 from Instituto de Salud Carlos III.
Publisher Copyright:
© 2021 British Society for Haematology and John Wiley & Sons Ltd
PY - 2021/8
Y1 - 2021/8
N2 - 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.
AB - 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.
KW - extramedullary disease
KW - multiple myeloma
KW - paraskeletal plasmacytomas
KW - plasmacytoma
KW - prognosis
KW - soft tissue
KW - treatment
UR - http://www.scopus.com/inward/record.url?scp=85102520623&partnerID=8YFLogxK
U2 - 10.1111/bjh.17338
DO - 10.1111/bjh.17338
M3 - Review article
C2 - 33724461
AN - SCOPUS:85102520623
SN - 0007-1048
VL - 194
SP - 496
EP - 507
JO - British Journal of Haematology
JF - British Journal of Haematology
IS - 3
ER -