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

Abstract

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.

• Authors: 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 Kyle, Fernando 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
• Journal: British journal of haematology (Br J Haematol) Vol. 194 Issue 3 Pg. 496-507 (08 2021) ISSN: 1365-2141 [Electronic] England
• PMID: 33724461 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Review)
• Copyright: © 2021 British Society for Haematology and John Wiley & Sons Ltd.

Chemical References

• Bortezomib
• Etoposide
• Dexamethasone
• Doxorubicin
• Cyclophosphamide
• Lenalidomide
• Cisplatin

Topics

• Animals
• Antineoplastic Combined Chemotherapy Protocols (therapeutic use)
• Bortezomib (therapeutic use)
• Cisplatin (therapeutic use)
• Cyclophosphamide (therapeutic use)
• Dexamethasone (therapeutic use)
• Disease Management
• Doxorubicin (therapeutic use)
• Etoposide (therapeutic use)
• Hematopoietic Stem Cell Transplantation
• Humans
• Lenalidomide (therapeutic use)
• Multiple Myeloma (complications, diagnosis, pathology, therapy)
• Plasmacytoma (complications, diagnosis, pathology, therapy)
• Prognosis
• Transplantation, Autologous