Expert review on soft‐tissue plasmacytomas in multiple myeloma: definition, disease assessment and treatment considerations. (16th March 2021)

• Record Type: Journal Article
• Authors: Rosiñol, Laura; Beksac, Meral; Zamagni, Elena; Van de Donk, Niels W. C. J.; Anderson, Kenneth C.; Badros, Ashraf; Caers, Jo; Cavo, Michele; Dimopoulos, Meletios‐Athanasios; Dispenzieri, Angela; Einsele, Hermann; Engelhardt, Monika; Fernández de Larrea, Carlos; Gahrton, Gösta; Gay, Francesca; Hájek, Roman; Hungria, Vania; Jurczyszyn, Artur; Kröger, Nicolaus; Kyle, Robert A.; Leal da Costa, Fernando; Leleu, Xavier; Lentzsch, Suzanne; Mateos, Maria V.; Merlini, Giampaolo; Mohty, Mohamad; Moreau, Philippe; Rasche, Leo; Reece, Donna; Sezer, Orhan; Sonneveld, Pieter; Usmani, Saad Z.; Vanderkerken, Karin; Vesole, David H.; Waage, Anders; Zweegman, Sonja; Richardson, Paul G.; Bladé, Joan;
• Is Part Of: British journal of haematology. Volume 194:Number 3(2021)
• Page Start: 496
• Page End: 507
• Publication Date: 2021-03-16
• Subjects: multiple myeloma -- plasmacytoma -- soft tissue -- extramedullary disease -- paraskeletal plasmacytomas -- prognosis -- treatment; Hematology -- Periodicals; Blood -- Diseases -- Periodicals; 616.15
• DOI: 10.1111/bjh.17338
• Languages: English
• ISSNs: 0007-1048

Abstract

Summary: 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.