Soft-tissue extramedullary multiple myeloma prognosis is significantly worse in comparison to bone-related extramedullary relapse

Pour, Luděk; Ševčı́ková, Sabina; Grešlíková, Henrieta; Kupská, Renata; Májková, Petra; Zahradová, Lenka; Sandecká, Viera; Adam, Zdeněk; Krejčí, Marta; Kuglík, Petr; Hájek, Roman

doi:10.3324/haematol.2013.094409

Cited by 148 publications

(158 citation statements)

References 24 publications

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“…The presence of EMM at the initial diagnosis is correlated with poor prognosis [11,14]. However, at the time of relapse, extramedullary disease has even worse prognosis with an overall survival that does not exceed 6 months [15]. The diagnostic and therapeutic approach of EMM is very challenging especially due to the adverse prognostic features and heterogeneity.…”

Section: Introductionmentioning

confidence: 99%

Successful treatment with lenalidomide dexamethasone for second relapse of a patient with non-secretory multiple myeloma with extramedullary liver plasmacytomas: A case report

Gavriatopoulou¹,

Terpos²,

Ρούσσου³

et al. 2017

Hematol Med Oncol

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MM is defined as the presence of ≥ 10% of monoclonal plasma cells (PCs) in the bone marrow (or a biopsy-proven extramedullary plasmacytoma) along with endorgan damage due to the underlying disorder. For the majority of myeloma patients, plasma cells are restricted in the bone marrow. However, a small subgroup of patients might develop extramedullary myeloma (EMM), which is defined as the presence of clonal PCs outside the bone marrow. Magnetic resonance imaging (MRI) and positron emission tomography/computed tomography (PET-CT) are the gold standard imaging techniques for the diagnosis, while biopsy of the lesion is highly recommended. The prognosis is very poor with a median overall survival that does not exceed 6 months. Patients with extramedullary disease are considered as high-risk patients and should be treated accordingly. In this case report we describe the management of a patient with nonsecretory myeloma who relapsed twice with extramedullary disease and we summarize the recommendations for therapeutic approaches of these patients that remain very challenging.

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Section: Introductionmentioning

confidence: 99%

Successful treatment with lenalidomide dexamethasone for second relapse of a patient with non-secretory multiple myeloma with extramedullary liver plasmacytomas: A case report

Gavriatopoulou¹,

Terpos²,

Ρούσσου³

et al. 2017

Hematol Med Oncol

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“…Total survival was significantly shorter than for other forms of MM relapse 15 . The era of new drugs, such as immunomodulatory drugs and proteasome inhibitors, has increased survival of MM patients but reports of increased incidence of EM are being published 3,16 . As of now, it is still unclear if the reported incidence is true or if it is connected to better imaging methods, such as PET/CT (ref.…”

Section: Introductionmentioning

confidence: 99%

“…2 ). Even though many advances in therapy have been made, MM is still considered an incurable disease since most patients eventually relapse 3 . Based on the newest guidelines of the International Myeloma Working Group (IMWG) published in 2014, high-risk MM is defined by ISS stage II or III, the presence of either t (4,14) or del17p13 and comprises about 20% of patients 4 .…”

Section: Introductionmentioning

confidence: 99%

“…The above mentioned adverse factors lead to resistance to treatment 11,12 . Clinically, three types of extramedullary lesions can be described: a) tumor mass adjacent to the bone and extending into soft tissues, b) soft tissue or visceral tumor that is not connected to the bone or c) diffuse infiltration of organs by PC without any obvious focal lesion 3 . Pour et al noted a marked decrease in survival for EM patients in comparison to MM patients (109 vs 38 months).…”

Section: Introductionmentioning

confidence: 99%

“…Extramedullary relapse of MM (EM) is defined by tumor cells found outside of the BM (ref. 3 ). It is proposed that EM is caused by secondary mutations in PC subclones that cause unresponsiveness of these cells to the microenvironment of BM, and these PC then leave the BM (ref.…”

Section: Introductionmentioning

confidence: 99%

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Extramedullary relapse of multiple myeloma defined as the highest risk group based on deregulated gene expression data

Ševčı́ková¹,

Paszekova

Bešše³

et al. 2015

Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub

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Background. Multiple myeloma (MM) is characterized by malignant proliferation of plasma cells (PC) which accumulate in the bone marrow (BM). The advent of new drugs has changed the course of the disease from incurable to treatable, but most patients eventually relapse. One group of MM patients (10-15%) is considered high-risk because they relapse within 24 months. Recently, extramedullary relapse of MM (EM) has been observed more frequently. Due to its aggressivity and shorter survival, EM is also considered high-risk. Aims. The goal of this study was to determine if the so-called high-risk genes published by the University of Arkansas group (UAMS) are even more deregulated in EM patients than in high-risk MM patients and if these patients may be considered high-risk. Methods. Nine samples of bone marrow plasma cells from MM patients as well as 9 tumors and 9 bone marrow plasma cells from EM patients were used. Quantitative real-time PCR was used for evaluation of expression of 15 genes connected to the high-risk signature of MM patients. Results. Comparison of high-risk plasma cells vs extramedullary plasma cells revealed 4 significantly deregulated genes (CKS1B, CTBS, NADK, YWHAZ); moreover, comparison of extramedullary plasma cells vs extramedullary tumors revealed significant differences in 9 out of 15 genes. Of these, 6 showed significant changes as described by the UAMS group (ASPM, SLC19A1, NADK, TBRG4, TMPO and LARS2). Conclusions. Our data suggest that increasing genetic abnormalities as described by the gene expression data are associated with increased risk for EM relapse.

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Progression with clinical features is associated with worse subsequent survival in multiple myeloma

et al. 2019

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Response rate and survival in multiple myeloma (MM) has improved in the era of proteasome inhibitors and immunomodulatory drugs. However, most patients eventually relapse with biochemical progression (BP) alone or with clinical features of end‐organ damage (CP: clinical progression), without or without extramedullary (EM) disease. We conducted a retrospective cohort study of 252 patients with MM experiencing first relapse (time, T0) to evaluate survival following CP with and without EM as a function of BP. Patients were divided into three groups: BP (n = 134; 53%), CP/EM‐ (n = 87; 35%) and CP/EM+ (n = 31; 12%). The median time from diagnosis to T0 was significantly shorter in CP/EM+ compared to CP/EM‐ and BP groups (13 vs 25 vs 25 months; P < 0.001). The incidence of abnormal metaphase cytogenetics at diagnosis was significantly higher in CP/EM+ compared to CP/EM‐ and BP groups (46% vs 18% vs 11% respectively; P < 0.001). At a median follow‐up of 26 months from T0, median overall survival was 50, 19 and 10 months for BP, CP/EM‐ and CP/EM+ groups, respectively (P < 0.001). On multivariable analysis, pattern of progression was a significant prognostic factor for OS (HR for CP/EM‐ vs BP: 3.6; CP/EM+ vs BP: 8.7 and CP/EM+ vs CP/EM‐: 2.42; P < 0.001 for all comparisons), along with age at T0. In conclusion, progression pattern is an important prognostic factor in the current era, with subsequent survival being dismal in patients with end‐organ damage or EM disease at relapse. Clinical trials in relapsed MM should consider reporting patterns of progression at baseline to ensure balance between study arms.

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Soft-tissue extramedullary multiple myeloma prognosis is significantly worse in comparison to bone-related extramedullary relapse

Cited by 148 publications

References 24 publications

Successful treatment with lenalidomide dexamethasone for second relapse of a patient with non-secretory multiple myeloma with extramedullary liver plasmacytomas: A case report

Successful treatment with lenalidomide dexamethasone for second relapse of a patient with non-secretory multiple myeloma with extramedullary liver plasmacytomas: A case report

Extramedullary relapse of multiple myeloma defined as the highest risk group based on deregulated gene expression data

Progression with clinical features is associated with worse subsequent survival in multiple myeloma

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