Outcome of paraosseous extra-medullary disease in newly diagnosed multiple myeloma patients treated with new drugs

Vittorio Montefusco*, Francesca Gay, Stefano Spada, Lorenzo de Paoli, Francesco Di Raimondo, Rossella Ribolla, Caterina Musolino, Francesca Patriarca, Pellegrino Musto, Piero Galieni, Stelvio Ballanti, Chiara Nozzoli, Nicola Cascavilla, Dina Ben-Yehuda, Arnon Nagler, Roman Hajek, Massimo Offidani, Anna Marina Liberati, Pieter Sonneveld, Michele CavoPaolo Corradini, Mario Boccadoro

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

34 Scopus citations


Extramedullary disease is relatively frequent in multiple myeloma, but our knowledge on the subject is limited and mainly relies on small case series or single center experiences. Little is known regarding the role of new drugs in this setting. We performed a meta-analysis of eight trials focused on the description of extramedullary disease characteristics, clinical outcome, and response to new drugs. A total of 2,332 newly diagnosed myeloma patients have been included; 267 (11.4%) had extramedullary disease, defined as paraosseous in 243 (10.4%), extramedullary plasmocytoma in 12 (0.5%), and not classified in 12 (0.5%) patients. Median progression-free survival was 25.3 months and 25.2 in extramedullary disease and non-extramedullary disease patients, respectively. In multivariate analysis the presence of extramedullary disease did not impact on progression-free survival (hazard ratio 1.15, P=0.06), while other known prognostic factors retained their significance. Patients treated with immunomodulatory drugs, mainly lenalidomide, or proteasome inhibitors had similar progression-free survival and progression-free survival-2 regardless of extramedullary disease presence. Median overall survival was 63.5 months and 79.9 months (P=0.01) in extramedullary and non-extramedullary disease patients, respectively, and in multivariate analysis the presence of extramedullary disease was associated with a reduced overall survival (hazard ratio 1.41, P<0.001), in line with other prognostic factors. With the limits of the use of low sensitivity imaging techniques, that lead to an underestimation of extramedullary disease, we conclude that in patients treated with new drugs the detrimental effect of extramedullary disease at diagnosis is limited, that lenalidomide is effective as are proteasome inhibitors, and that these patients tend to acquire a more aggressive disease in later stages. (EUDRACT2005-004714-32, NCT01063179. NCT00551928, NCT01091831, NCT01093196, NCT01190787, NCT01346787, NCT01857115).

Original languageAmerican English
Pages (from-to)193-200
Number of pages8
Issue number1
StatePublished - 2020
Externally publishedYes

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© 2020 Ferrata Storti Foundation


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