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

TY - JOUR

T1 - Expert review on soft-tissue plasmacytomas in multiple myeloma: 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 - © 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.

U2 - 10.1111/bjh.17338

DO - 10.1111/bjh.17338

M3 - SCORING: Review article

C2 - 33724461

VL - 194

SP - 496

EP - 507

JO - BRIT J HAEMATOL

JF - BRIT J HAEMATOL

SN - 0007-1048

IS - 3

ER -