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M. Beksac et al.
AB
Figure 1. Overall survival (OS) estimates comparing patients with extramedullary plasmacytomas (EMP) to those with paraosseous (PO) lesions (A) at diagnosis and (B) at relapse. EMD: extramedullary disease.
Table 2. Comparison of response, survival outcomes of extramedullary plasmacytomas (EMP) or paraosseous (PO) patients either at diagnosis or at relapse.
EMP
diagnosis (n=92)
relapse (n=84)
PO
diagnosis (n=38) relapse (n=12)

46.5 (95% CI: 25.5-67.5)
P<0.001
P<0.001
CR (%)
19.3 9
34.2 54.5
38.9 P<0.001 (95% CI: 23.6-54.2)
13.6 P=0.002 (95% CI: 11.6-15.6)

PFS (mos)
OS (mos)
P=0.034
11.4
(95% CI:*.6-16.2)
NR 39.8
(95% CI: 12.7-66.9)

P=0.001
51.7
95% CI: 13.5-89.9) 20.9
(95% CI: 10.3-31.5)
P=0.005
CR: complete remission; PFS: progression-free survival; OS: overall survival; n: number.
Therapeutic interventions and response
Treatments of patients are summarized in Table 1. The most commonly used treatment was combination chemotherapy with or without radiotherapy followed by cyclophosphamide/bortezomib/dexamethasone (45.6%). A total of 100 patients received ASCT, of which 67 (51.5%) with EMD at diagnosis. Median interval from EMD diagnosis to ASCT was 11.3 months (2-91 months). Transplant was a more frequent treatment approach among patients presenting with PO (31 of 38) compared to those with EMP (36 of 92) In addition, 29 patients (12.8%) had already been transplanted prior to the diagno- sis of EMD, which developed after a median of 30.8 months post ASCT. Only four EMD patients diagnosed at relapse underwent ASCT. The entire group received a median of two lines of treatment following the diagnosis of EMD. Seventy-five (57.7%) myeloma patients with EMD at diagnosis went on to receive second line of ther- apy and 48 (37.2%) received more than two lines of ther- apy. Among 96 patients with EMD at relapse, 56 (58.3%) of them received more than two lines therapy.
As can be seen in Table 2 there were significant differ-
ences in outcomes when EMP was compared with PO. A statistically significant difference in complete response rate (CR) (PO: 38.8% vs. EMP: 14.8%; P=0.001) was observed following first line of treatment (not shown in Table 2). Of the 88 newly diagnosed EMP patients, with response to induction available, 35 had received radiother- apy without (n=6) or with (n=29) systemic treatment. These patients achieved a CR rate (11.4 %) that was con- siderably less than the CR (24.5%) achieved with chemotherapy alone. Among those who received ASCT, there was an improved CR rate of 29% versus 19% (at diagnosis) 41.7% versus 9.5% (at relapse). However, regardless of treatment, 51.4% of even those who achieved CR progressed within median 18.1 months versus 12.1 months in PO and EMP groups, respectively (P=NS). Among the newly diagnosed patients who under- went ASCT (n=67), the median PFS from diagnosis was 49 months (95%CI: 22.7-75.3) (PO: 51.7 months (95%CI: 18.3-85.1) and EMP: 46.5 months (95%CI: 32.8-60.2); P=NS). Among those who did not receive ASCT the medi- an PFS was 28.1 months (95%CI: 20.3-35.9) (P<0.001). Post-ASCT depth of response (>VGPR vs. <VGPR) did not
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haematologica | 2020; 105(1)