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Expert review on soft‐tissue plasmacytomas in multiple myeloma: definition, disease assessment and treatment considerations
Author(s) -
Rosiñol Laura,
Beksac Meral,
Zamagni Elena,
Van de Donk Niels W. C. J.,
Anderson Kenneth C.,
Badros Ashraf,
Caers Jo,
Cavo Michele,
Dimopoulos MeletiosAthanasios,
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
Publication year - 2021
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/bjh.17338
Subject(s) - medicine , bortezomib , multiple myeloma , lenalidomide , regimen , thalidomide , autologous stem cell transplantation , oncology , etoposide , melphalan , population , cyclophosphamide , dexamethasone , surgery , chemotherapy , environmental health
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.