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Purpose: Despite the development of novel drugs and the widespread use of hematopoietic cell transplantation, the prognosis of patients (pts) with multiple myeloma and extramedullary involvement (soft-tissue plasmacytoma, STP) is rather unfavorable., Methods: A retrospective analysis of 120 pts with STP treated between 2007 and 2022 was performed. The effects of demographic and clinical characteristics on treatment response, progression-free survival (PFS), and overall survival (OS) were evaluated., Results: The rate of serological response to first-line STP treatment (at least partial remission) was 67%, and the rate of imaging response was 59%. With a median follow-up of 84.2 months, the median PFS was 10.5 months (primary STP: 20.2 months; secondary STP: 5.8 months), and the median OS was 24.5 months (primary STP: 34.5 months; secondary STP: 12.4 months). Based on the multivariate regression analysis, secondary STP (HR PFS 2.75; HR OS 2.63) and organ involvement (HR PFS 1.45; HR OS 1.68) were found to be negative prognostic factors of both PFS and OS. In a prognostic model, pts with at least one of these factors had a significantly worse PFS (HR PFS 3.31) and OS (HR OS 3.45) than those with none risk factor., Conclusion: In pts with STP, risk-adapted treatment strategies including immunotherapies and cell therapies are urgently required., (© 2024. The Author(s).)

Importance: There are significant gaps in the literature pertaining to the locoregional control and survival rates of extramedullary plasmacytoma (EMP) with respect to various treatment approaches., Objective: To systematically evaluate the differences in radiotherapy and surgical outcomes in EMP., Data Sources: Databases including PubMed, Scopus, Web of Science, Embase, and ScienceDirect were systematically searched from their inception up to November 2023., Study Selection: Articles reporting radiotherapy and surgical outcomes of head and neck EMP were included., Data Extraction and Synthesis: A random-effects model for meta-analysis was used to obtain pooled estimates and calculate hazard ratios for survival and odds ratios for recurrence and progression of EMP., Main Outcomes and Measures: Survival, tumor control, and progression rates to multiple myeloma (MM) between radiation therapy and surgery for EMP of the head and neck., Results: Of 742 included patients from 12 studies, 527 (71.0%) were male, and the median (IQR) age was 59.1 (53-62) years. A total of 505 patients (68.1%) received radiotherapy only, while 237 (31.9%) underwent surgery-only treatment for EMP. All included patients had an initial diagnosis of EMP without MM. Comparable trends were observed in overall survival and disease-free survival (DFS) rates at 2, 3, 5, and 10 years between patients with EMP treated with radiotherapy only and surgery only. Notably, there were no significant differences in recurrence rate (odds ratio, 0.65; 95% CI, 0.20-2.06) between radiotherapy-only and surgery-only treatment. However, radiotherapy-only treatment of EMP was associated with decreased odds of progression to MM compared with surgery (odds ratio, 0.4; 95% CI, 0.1-0.9). Sensitivity analysis revealed that the radiotherapy-only population had significantly better 5-year DFS (hazard ratio, 0.55; 95% CI, 0.31-0.96) compared with surgery-only treatment., Conclusions and Relevance: This systematic review and meta-analysis provides evidence that patients with EMP receiving radiotherapy had significantly lower chances of progression to MM compared with surgery-only therapy. Additionally, radiotherapy had better 5-year DFS outcomes compared with surgery. Comparable outcomes in terms of overall survival rates, recurrence, and mortality rates were noted between radiotherapy-only and surgery-only EMP treatment groups.

Background: A solitary plasmacytoma is classified into a solitary plasmacytoma of the bone (SBP) and a solitary extramedullary (soft tissue mass) plasmacytoma, based on the site of the lesion. Despite the high local control rate with radiotherapy, approximately half of patients' conditions progress to multiple myeloma (MM) within 3-5 years after diagnosis, with SBP having a worse prognosis., Patients and Methods: We retrospectively assessed the treatment and outcomes of patients with SBP in a hospital in China from 2008 to 2021. Twenty-four patients treated over 13 years with SBP were enrolled in this retrospective study., Results: The most common sites for SBP were the axial skeleton and femur. The M protein was detected in 11 patients (46 %), of which 8 (33 %) had light chains, 2 (8 %) had immunoglobulin G kappa and 1 (4 %) had immunoglobulin D kappa. Flow cytometry revealed that 5 patients (21 %) had minimal bone marrow involvement. The treatment included chemotherapy, surgery, and radiotherapy in 18 (75 %), 12 (50 %), and 9 (38 %) patients, respectively, of whom 13 (54 %) received combined treatment. Over a median follow-up period of 67.2 months, 9 patients (38 %) developed MM in a median time of 101.5 months. The 5- and 10-year progression-free survival rates were 67.3 % and 37.4 %, respectively. One patient died due to pneumonia without progression and the other died due to relapse., Conclusion: This study confirmed the high rate of progression of SBP to MM, indicating a need for adjunct chemotherapy for the management of SBP., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Abstract: Due to the rarity of solitary bone plasmacytoma (SBP), few studies reported the prognosis and survival predictors of SBP, especially for patients with extremity SBP.A total of 552 patients with extremity SBP were identified from the Surveillance Epidemiology and Ends Results (SEER) database between 1973 and 2016. In order to obtain independent predictors of survival, we performed both univariate and multivariate analysis via Cox proportional hazards model. Additionally, we used the Kaplan-Meier method to construct survival curves.The mean and median age at diagnosis of all patients were 64 and 65 years, respectively. The ratio of male versus women was 1.3:1. Overall survival for this special population was 51.2% and 34.9% at 5 and 10 years, respectively. Cancer-specific survival (CSS) for this special population was 63.5% and 47.5% at 5 and 10 years, respectively. Age at diagnosis and radiotherapy treatment were found to be significant independent predictors of both overall survival and CSS. Additionally, multivariate analysis showed that year of diagnosis and marital status were significantly correlated with CSS.This is the first study to identify prognostic factors of extremity SBP by using the SEER database. Our findings highlight that radiotherapy is the mainstream treatment for extremity SBP. Additionally, age, year of diagnosis, and marital status were significant independent predictors of survival. Knowledge of these survival predictors may help clinicians provide appropriate management for extremity SBP patients., Competing Interests: The authors report no conflicts of interest., (Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.)

Objectives: Solitary plasmacytoma (SP) is characterized by a single mass of clonal plasma cells. Definitive RT can result in long-term local control of the SP. Due to the small number of patients and narrow range of doses, phase III randomized trials are lacking. The aim of this study is to further support the potential use of RT for the treatment of SP., Methods: Clinical data of all patients treated for SP at our Institution between 1992 and 2018 were reviewed. A total of 42 consecutive patients were analyzed., Results: The median follow-up was 84.8 months. Radiation dose did not differ significantly as a function of sex, type of SP (solitary bone plasmacytoma or as extramedullary plasmacytoma), tumor size; conversely differs significantly as a function of age (p = 0.04). The 5y-OS and 10y-OS were, respectively, 96 and 91%. Local recurrences developed in 21.4% of patients (9/42). 16 patients progressed to MM (38.1%). The 5y-progression to MM free survival (PMFS) and the 10y-PMFS were, respectively, 68.6 and 61.9%., Conclusions: Our data confirm that good results are achievable with RT to treat SP, but they don't allow defining a dose-effect correlation; therefore, it remains uncertain which is the most effective dose and whether lower doses can guarantee adequate disease control.

The aim is to analyze the clinical outcomes of patients with extramedullary plasmacytoma (EMP) and review the characteristics of this disease. We retrospectively reviewed the clinical data of a cohort of 22 patients diagnosed with EMP between 1983 and 2017. Baseline characteristics and progression data were collected to calculate the incidence of progression to multiple myeloma (MM) and to analyze survival rates and outcomes. Previous major cohort studies were reviewed and compared with this study. Extramedullary plasmacytomas were located in the head and neck in 17 (77%) of the 22 patients. The median time between onset and diagnosis was 7 months, and the median age at diagnosis was 52.5 years (range 15-72 years). Extramedullary plasmacytoma occurred more frequently in men with an approximate ratio of 2.1:1. The majority of patients underwent surgery (13/22, 59.1%) and half received radiation (11/22, 50%). A small proportion of patients received chemotherapy (2/22, 9.1%). There was a median follow-up time of 98.5 months. The 5-year overall survival, progression-free survival, and MM-free survival rates were 84.2%, 67.3% and 75.9%, respectively. Complete resection without major functional damage is a promising option that can favorably improve prognosis in patients with resectable disease. Patients with regional lymph node metastasis or positive immunoelectrophoresis results require more aggressive treatment and may have poorer prognosis.

Solitary plasmacytoma of bone (SPB) is a single, isolated plasmacytoma originated from the bone. The survival trends of patients with SPB in recent years remain unknown. And the prognostic system of SPB may also need to be refined. The 18 Surveillance, Epidemiology, and End Results (SEER) databases of the National Cancer Institute in the United States were used to extract data for this study. The third edition of the International Classification of Disease for Oncology (ICD-O-3) code 9731 was used to identify cases of SPB. For each case, factors including age at the time of diagnosis, sex, race, marital status, insurance status, primary sites of tumors, and the use of surgery were collected. The outcomes of patients with SPB were compared between two groups. And the prognostic impacts of baseline characteristics and use of surgery was studied. A total of 4103 (from 1976 to 2016) cases of SPB were identified. The median age was 65 years old. Patients in time period-2 (2008-2016) show better survival as compared to those in time period-1(1976-2007) (median overall survival: 88 months vs. 73 months, p = 0.0332). Age ≤ 65 years and being male were associated with better outcomes. The widowed individuals had significantly inferior survival and myeloma-specific survival than the single, married, or divorced individuals (p values all <0.0001). Patients with lesions in bones of skull and face and associated joints had longer survival as compared with those with bone lesions in other sites (median overall survival: 107 months vs. 79 months, p = 0.0694). The use of surgery was significantly associated with improved survival (median survival: surgery performed 98 months vs. not performed 73 months, hazards ratio [HR]: 0.7623, 95% CI: 0.7009-0.8472; p < 0.0001) and myeloma-specific survival (median myeloma-specific survival: surgery 160.0 months vs. no surgery 143.0 months, HR: 0.8469, 95% CI: 0.7493-0.9572; p = 0.0078). Multivariable analysis revealed that surgery, marital status, and age were independent prognostic factors for overall survival in patients with SPB. The improvement in the survival of patients with SPB has been observed in recent years. And several potential prognostic factors were identified. Future prospective studies are warranted to explore the roles of novel agents, surgery, and systemic chemotherapy in the treatment of SPB., (© 2020 The Authors. Cancer Medicine published by John Wiley & Sons Ltd.)

Background: There have been reports on the use of hypofractionated stereotactic body radiotherapy (SBRT) for bone plasmacytomas, but no prospective data are available. We present the initial analysis of an ongoing prospective protocol on SBRT addressing the feasibility and safety of this treatment for solitary bone plasmacytomas., Patients and Methods: A prospective cohort of SBRT for solitary bone plasmacytoma was developed. Patients could receive different doses depending on the index bone, from single fraction for skull base lesions, 24 Gy in 3 fractions for spine lesions, and 30 Gy in 5 fractions for other bones. Overall survival, bone events, local control, and progression to multiple myeloma (MM) were measured and compared to our retrospective cohort of patients treated with conformal standard-dose radiotherapy. Quality of life was assessed via the EORTC QLQ-C30 questionnaire, and toxicities were assessed by the CTCAE v5.0 criteria. After 1 year or the inclusion of 5-10 patients, a feasibility and safety analysis was programmed., Results: Between April 2018 and April 2019, 5 patients were included. All were male, with a median age of 53.1 years. The median follow-up was 21.8 months. No patient had local progression, bone event, or died. Two patients had progressions to MM. The mean survival free of progression to MM was 18.6 months, compared to 19 months in the retrospective cohort; median values were not reached. There were no grade 3 toxicities., Conclusion: SBRT for plasmacytoma is safe and feasible. More robust data are awaited., (© 2021 S. Karger AG, Basel.)

Plasma cell myeloma (also called multiple myeloma), solitary plasmacytoma, and extramedullary plasmacytoma are primarily diseases of the elderly. Evidence suggests an association between excess body weight and multiple myeloma. Few population-based studies have examined incidence and mortality of each site in one study. We analyzed incidence and death rates by site (solitary plasmacytoma, extramedullary plasmacytoma, and multiple myeloma) by gender, age, race/ethnicity, and rural-urban status among adult males and females (aged 20 years or older) in the United States during 2003-2016. Trends were characterized as average annual percentage change (AAPC) in rates. During 2003-2016, overall incidence rates among adults were 0.45 for solitary plasmacytoma, 0.09 for extramedullary plasmacytoma, and 8.47 for multiple myeloma per 100,000 persons. Incidence rates for multiple myeloma increased during 2003-2016 among non-Hispanic whites (AAPC = 1.78%) and non-Hispanic blacks (2.98%) 20-49 years of age; non-Hispanic whites (1.17%) and non-Hispanic blacks (1.24%) 50-59 years of age; and whites non-Hispanic (0.91%), and non-Hispanic blacks (0.96%). During 2003-2016 overall myeloma (extramedullary plasmacytoma and multiple myeloma) death rates among adults was 4.77 per 100,00 persons. Myeloma death rates decreased during 2003-2016 among non-Hispanic white (AAPC = -1.23%) and Hispanic (-1.34%) women; and non-Hispanic white (-0.74%), non-Hispanic American Indian/Alaska Native (-3.05%) men. The US population is projected to become older and will have a larger proportion of persons who have had an earlier and longer exposure to excess body weight. The potential impact of these population changes on myeloma incidence and mortality can be monitored with high-quality cancer surveillance data., (Published 2020. This article is a U.S. Government work and is in the public domain in the USA. Cancer Medicine published by John Wiley & Sons Ltd.)

Thirty dogs with macroscopic plasma cell tumours (PCTs) were treated with radiation therapy (RT). Twelve patients were treated with palliative-intent prescriptions (range, 4-10 Gy/fraction (median, 7 Gy/fraction) for a total dose of 20 to 35 Gy (median total dose 30 Gy). Eighteen patients received definitive-intent prescriptions (range, 3.0-4.2 Gy/fraction (median, 3 Gy/fraction) for a total dose of 42 to 54 Gy (median total dose 48 Gy). Involved sites included the oral cavity, skin, multiple myeloma (MM)-associated lytic bone lesions, bone (solitary osseous plasmacytoma; SOP), nasal cavity, larynx, retrobulbar space, lymph node and rectum. Ninety-five percent of evaluable dogs had a complete (CR; 16/22) or partial response (PR; 5/22). Patients with MM experienced significant analgesia. The median progression-free survival (PFS) was 611 days (range: 36-2001 days). Events in the non-MM cases included in-field progression (5/26, 19%) and disseminated disease (5/26, 19%). The median survival time (MST) for all dogs was 697 days (range: 71-2075 days), and when only non-MM cases were considered, MST was 771 days (range: 71-2075 days). Fourteen patients were alive without disease progression or had died of unrelated causes. Achievement of a PR was associated with an inferior PFS and MST as compared with CR. Palliative-intent RT was associated with inferior MST as compared with definitive-intent RT. RT is a useful therapeutic modality for PCTs and tumour responses are often complete and durable, with protracted survivals. The optimal radiation dose and schedule are yet to be defined., (© 2020 John Wiley & Sons Ltd.)

We retrospectively analyzed 51 patients with solitary plasmacytoma diagnosed from October 2002 to September 2018 from a cohort of 3575 patients with plasma cell dyscrasias registered in the Kansai Myeloma Forum. Twenty-seven patients had solitary bone plasmacytoma (SBP) and 24 had extramedullary plasmacytoma (EMP), with prevalence of 0.8% and 0.7%, respectively. The most frequent M protein was IgG (40%) in SBP, whereas non-secretory proteins were most frequent (50%) in EMP. Five-year overall survival was 78.2% in SBP and 80.8% in EMP (P = 0.894). Among patients with SBP, 44% progressed to MM with a median time of 10.5 months (2.4-93.3 months), whereas 8% of EMP patients progressed to MM with a median time of 18.6 months (13.0-24.2 months). The most frequent treatment was radiotherapy (41%) or observation (41%) in SBP, and chemotherapy (54%) in EMP. No statistically significant difference was observed upon univariate analysis of prognostic factors including age, sex, performance status, and IgG M protein. Our results suggest that there are biological differences between SBP and EMP in real-world settings.

Purpose: The study evaluates the results of the concurrent use of lenalidomide-dexamethasone with intensity modulated radiation therapy (IMRT) for solitary plasmacytoma in terms of toxicity and outcome., Methods and Materials: Forty-six patients were treated for histologically proven solitary plasmacytoma (SP) between June 2007 and June 2018 in our Department (Curie Institute, Paris, France). All patients received IMRT. The median total dose was 40 Gy (range, 40-46). Prescription of concurrent lenalidomide-dexamethasone with radiation therapy was left to the discretion of the referring hematologist-oncologist and started the first day of radiation therapy for 4 cycles., Results: Twenty-seven solitary plasmacytoma were treated with IMRT alone and 19 with lenalidomide-dexamethasone in association with IMRT. At 5 years, the local control, multiple myeloma-free survival (MMFS), and progression-free survival (PFS) rates were 96.3%, 85.4%, and 60%. MMFS and PFS were significantly higher in the IMRT plus lenalidomide-dexamethasone group compared with IMRT alone group (100% vs 77.1%, P = .02 and 81.7% vs 48.4%, P = .047, respectively). No major toxicity was found in either group., Conclusions: Lenalidomide-dexamethasone in association with IMRT in the treatment of solitary plasmacytoma is safe and improves MMFS and PFS. Further prospective and comparative studies are needed to confirm these results., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Given the rarity of plasmacytoma, large-scale database analysis can provide useful information regarding the clinical presentation and patient-related factors impacting overall survival (OS)., Materials and Methods: The National Cancer Data Base was queried for patients with plasmacytoma between 2004 and 2013, excluding patients with systemic disease. Plasmacytomas were classified as originating in bone (P-bone), in extramedullary tissue (P-EM), or unspecified. Survival was estimated using the Kaplan-Meier and log-rank test method. We used Cox regression to determine specific outcomes adjusting for demographic, socioeconomic, geographic, facility type, year of diagnosis, and comorbid factors., Results: In total, 6225 patients were identified, of which 61.5% were men. The median age at diagnosis was 64 years (range, 18-90 years), and the median follow-up was 58 months. The primary site of disease was P-bone in 4056 (65.1%) patients and P-EM in 1468 (23.6%), and the remaining 701 patients were P-unspecified. The unadjusted median survival for solitary P-bone was 89 months (95% confidence interval, 82.9-95.0 months), and for solitary P-EM was 117.3 months (95% confidence interval, 108.8 months to not reached). Factors associated with improved OS include younger age, private insurance, higher income, solitary lesion, and lower comorbidity score. Patients with P-bone disease treated at academic facilities had improved OS. Only 65% of patients with solitary plasmacytoma lesions received radiation treatment. Age greater than 75 years and increased distance to treatment facility was associated with a decreased likelihood of receiving radiation., Conclusions: This is the largest study examining outcomes of patients with plasmacytoma using a large database analysis, revealing unique aspects of P-EM versus P-bone and underutilization of radiation treatment., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Patients with plasma-cell neoplasia usually suffer from systemic disease, although a minority (< 5%) may present with solitary involvement of bone or soft tissue (extramedullary plasmacytoma (EMP)). Radiotherapy (RT) is a state-of-the-art treatment for these tumors offering long term curation., Methods and Materials: Between January 2005 and January 2017, twenty-seven patients underwent RT at our institution. The aim of this study was to analyse the effectiveness of various RT doses for different forms of EMP., Results: A total of 33 radiation courses were administered to 27 patients with a median age of 56 years. The median RT dose was 45 Gy (range: 12-55.8). The local control rate was 76% (93% for primary EMP vs. 61% for the secondary EMP lesions; P < 0.05). A complete response (CR) rate to local RT was achieved for 42% lesions (67% for primary EMP vs. 22% for the secondary EMP lesions; P < 0.01). The overall response rate (ORR) for the EMP lesions treated with high-dose regimens (> 45 Gy) versus low-dose regimens (≤ 45 Gy) was 87% versus 67%, respectively (P = 0.2). The median survival with high-dose RT group was significantly longer (P = 0.02). In subgroups analysis, primary EMP patients treated with high-dose RT had a non-significant higher ORR (100% vs. 80%, respectively; P = 0.3,) longer duration of LC (P = 0.3) with a longer survival (P = 0.05) than patients in low-dose group. No significant difference has been detected in secondary EMP patients treated with high-dose RT regarding ORR (60% vs. 62%, respectively; P = 1), and survival (P = 0.4)., Conclusion: RT is an efficacious treatment modality in the treatment of EMP. A radiation dose ≤45 Gy confer a comparable CR rate to high-dose regimens and appears to be an effective treatment for controlling local EMP progression. Radiation dose-escalation may be beneficial for particular subgroups of patients.

Solitary plasmacytomas are uncommon plasma cell disorders, which may present as a single bone lesion (P-bone) or extramedullary plasmacytoma (P-EM). There is a paucity of large studies analyzing prognostic factors and outcomes of plasmacytomas. While the treatment of choice is radiation therapy (RT), there is a lack of data evaluating optimal RT dose. In this study, we sought to answer these questions by utilizing the National Cancer Database plasmacytoma data from 2000 to 2011. A total of 5056 patients were included in the study (median age 62 years; range 52-72). To obtain a pure plasmacytoma cohort, potential multiple myeloma patients were excluded from the study (bone marrow involvement, systemic chemotherapy use). P-bone constituted 70% of the patients. The median overall survival (OS) of P-EM was significantly longer than P-bone (132 vs. 85 months), and for soft/connective tissue it was worse than remainder of P-EM (82 vs. 148 months). On multivariable analysis, factors associated with worse OS included older age (≥65), presence of P-bone, and treatment with a radiation dose <40 Gy.

In this single-center, population-based, and retrospective study, we analyzed the outcome of 49 patients with solitary bone plasmacytoma (SBP) and 28 patients with solitary extramedullary plasmacytoma (SEP), all treated with radiotherapy. Laminectomy was performed in 18/30 SBP patients with vertebral involvement and tumour resection in 10 SEP patients. Overall survival and cause of death for each patient were compared to 5 sex-, age-, and residency-matched individuals from the normal population. Response (complete and partial) was achieved in 94% of SBP and 96% of SEP patients. Relapse rates were higher in SBP (65%) compared to patients with SEP (18%) (P < .01). Only one in-field relapse was identified for the whole series. Ten- and 15-year overall survival, progression free survival (PFS) and multiple myeloma free survival (MMFS) for patients with SBP were 60%/41%, 25%/17%, and 33%/33%. Corresponding values for patients with SEP were 67%/54%, 57%/44%, and 91%/91%. SBP patients had significantly shorter PFS and MMFS compared to SEP patients (P < .01 for both). Only two of the SEP patients developed multiple myeloma and no patient in the whole series progressed to multiple myeloma later than 10 years after diagnosis. Unlike for SEP, the major cause of death among SBP patients was multiple myeloma (49%). Compared to matched normal population, no increased risk of death from secondary malignancies or cardiovascular disease was observed. Positive predictors in SBP patients were for overall survival age <60 years, combined laminectomy and radiotherapy and radiotherapy dose >40 gray, for PFS tumour size <6 cm and combined laminectomy and radiotherapy and for MMFS tumour size <6 cm. Radiotherapy confers excellent local control in both SEP and SBP patients; however, the challenge is to prevent development of multiple myeloma in patients with SBP., (Copyright © 2017 John Wiley & Sons, Ltd.)

Introduction: Head and neck extramedullary plasmacytoma is a rare localized plasma cell neoplasm. We intended to perform this review of the published literature to assess the demographic profile, pattern of care and survival outcomes., Methods: Two authors independently searched PubMed, Google search and Cochrane library for eligible studies from 1950 till July 1, 2016, published in English language., Results: Median age of the cohort was 57 years (range 11-85). Site-wise distributions were paranasal sinuses 22.3% (70), nasal cavity 17.5% (55), nasopharynx 10.8% (34). Median size of SEMP was 3 cm (range 0.3-12 cm). Treatment distribution was radiotherapy (RT) in 52% (164), surgery (S) 19% (60), chemotherapy (C) 5% (16), S + RT 23.49% (74),CRT 1.9% (6), S + C 0.6% (2), S + RT + C 0.95% (3).Radiation was used as a modality in 78.4%(247), surgery in 44.1%(139), chemotherapy in 4.8%(15). Median radiation dose used was 45 Gy with range 20-61 Gy. Median overall survival (OS) was 40 months (range 0.5-298). Median local progression-free survival was 36 months (range 0-298). Median myeloma relapse-free survival was 36 months (range 0.5-298). Five- and 10-year OS was 78.33 and 68.61%. Five-year cause-specific survival (CSS) and 10-year CSS was 90.15 and 83.31%. Five-year LPFS was 94.78%, and 10-year LPFS was 88.43%. Five-year myeloma progression-free survival was 84.46%, and 10-year myeloma PFS was 80.44%. The factors associated with risk of local relapse were site of disease (sinonasal), secretory EMP, type of treatment received (surgery + RT > RT alone > surgery on univariate analysis). Risk factors for myeloma relapse were coexisting diseases, site of disease (sinonasal), bony erosion, size of lesion > 5 cm and type of treatment received on univariate analysis., Conclusion: Our study shows that combined modality S + RT is superior compared to uni-modality in preventing local recurrence. Radiation dose of 45 Gy is optimal. Nodal irradiation has no impact on local recurrence.

Background: -The values of SEER site recode variables are based on the primary site and histology data fields submitted to SEER by the registries. The site recode variables define the major cancer site/histology groups that are commonly used in the reporting of cancer incidence data and are added to the SEER databases as a convenience for researchers. These codes and definitions are periodically updated and changed by the National Cancer Institute as newer and more applicable information becomes available. Because this myeloma analysis includes cases diagnosed 2010+, the ICD-O-3 recode-updates with adjustment for WHO 2008 hematopoietic histologies that account for changes in the obsolete classification of hematopoietic histology codes, and the assignment of new names (ie, multiple myeloma-MM - to - plasma cell myeloma-PCM) is adhered to and used here. Plasma cell myeloma (PCM) is a bone-marrow based multifocal plasma cell malignancy (primary site C421). PCM is characterized by a single clone of plasma cells, believed to be derived from lymphoid B cells, and spans a clinical spectrum from asymptomatic to aggressive forms, plus disorders caused by the deposition of abnormal immunoglobulin chains in tissue. The current myeloma group ICD-O-3 histologic morphology types consists of: ICD-O-3 9731: Plasmacytoma, NOS, occurring in bone (osseous plasmacytoma malignancy data reportable to SEER only beginning since 1986); ICD-O-3 9732: Plasma cell myeloma - composed of three clinical variants: a) asymptomatic, b) Non-secretory myeloma, and c) Plasma cell leukemia (all coded to 9732); ICD-O-3 9734: Extramedullary plasmacytoma; anatomic sites other than bone., Objective: -Using the statistical database of SEER*Stat 8.3.4 (produced 4/14/2017 for diagnosis years 1973-2014), to assess, determine, compare, and summarize the occurrence, long-term survival and mortality indices of the three morphologic types of myeloma by age, sex, race and stage in two-cohort entry time-periods (1973-1994 and 1995-2014). All analyses are accomplished within the context of current SEER Site Recode ICD-O-3 (1/27/2003) definitions, terminologies and descriptions, and also in accordance with the rules of the consolidated Hematopoietic and Lymphoid Neoplasm Coding Manual data base (effective 1/1/2010 - release date January 2015)., Methods: -Population data including 111,041 cases collected by the National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) Frequency Database (18 SEER Registries Research Data + Hurricane Katrina Impacted Louisiana Cases, November 2016 Submission, 1973-2014 varying) for diagnosis years 1973-2014: Relative Survival Statistics were analyzed in two cohorts: 1973-1994 and 1995-2014. Survival statistics were derived from: SEER*Stat Database: Incidence - SEER 9 Regs Research Data, November 2016 Submission (1973-2014) Released April 2017., Results: -Tables 1-3 provide basic SEER comparative survival and mortality data of the three myeloma oncotypes by age, sex, stage and disease duration of patients in the 1973-2014 time-period. Epidemiologic, demographic, and case statistics data extracted from the most current NCI Cancer Statistics Review (CSR 2010-2014) are included., Conclusions: -Recent SEER age-adjusted incidence trends, 2011-2014, for all races has been downward, with an annual percentage change (APC) of -2.5% per year. Mean age in plasma cell myeloma (PCM) patients was about 1-year less in males (67.8 yrs) than in females (69.2 yrs). PCM is accompanied by a very high excess mortality and much reduced 5-year relative survival ratio especially in older age groups. Generally, first year excess death rates (EDRs) decreased with duration but increased with advancing entry age, and there was no sex difference. First year EDRs in blacks, all ages combined, was quite high but lower than EDRs in whites. Median survival, actual survival and 5-year relative survival ratios diminished precipitously to extremely low levels with increasing entry age attesting to the lethal character of this disease especially in older patients.

Extramedullary solitary plasmacytoma (EMP) is a rare type of malignancy. This paper presents a retrospective review of the experience with EMP at the Krakow Oncology Center. Records of 17 patients with head and neck EMP, treated with definitive radiotherapy between 1976 and 2009, were analyzed. The total tumour dose ranged from 45 to 70 Gy (median 56 Gy). In four patients with partial response after radiotherapy, adjuvant melphalan-based chemotherapy was applied. The median follow-up period was 8.6 years. The treatment was well tolerated. The estimated 10-year overall survival, disease-free survival, and multiple myeloma-free survival were 68.4%, 49.3%, and 55%, respectively. The 10-year local control rate was 90.9%. No in-field local recurrence was observed. During the follow-up, progression into multiple myeloma was observed in five patients, with a mean time to conversion of 24 months. The only factor adversely affecting overall survival on univariate analysis was the age >56 years, whereas a complete tumour regression after radiotherapy was associated with a significant improvement in both disease-free survival and multiple myeloma-free survival. Despite the high effectiveness of local radiotherapy, there is still a significant treatment failure risk due to the EMP conversion into generalized disease. An attempt to identify prognostic factors may facilitate selection of patients with a high risk of progression to multiple myeloma. Copyright © 2015 John Wiley & Sons, Ltd., (Copyright © 2015 John Wiley & Sons, Ltd.)

Solitary plasmacytoma (SP) and plasma cell leukemia (PCL) are uncommon (3-6%) types of plasma cell disease. The risk of progression to symptomatic multiple myeloma (MM) is probably important for the outcome of SP. PCL is rare and has a dismal outcome. In this study, we report on incidence and survival in PCL/SP, and progression to MM in SP, using the prospective observational Swedish Multiple Myeloma Register designed to document all newly diagnosed plasma cell diseases in Sweden since 2008. Both solitary bone plasmacytoma (SBP) (n=124) and extramedullary plasmacytoma (EMP) (n=67) have better overall survival (OS) than MM (n=3549). Progression to MM was higher in SBP than in EMP (35% and 7% at 2 years, respectively), but this did not translate into better survival in EMP. In spite of treatment developments, the OS of primary PCL is still dismal (median of 11 months, 0% at 5 years). Hence, there is a great need for diagnostic and treatment guidelines as well as prospective studies addressing the role for alternative treatment options, such as allogeneic stem cell transplantation and monoclonal antibodies in the treatment of PCL., (© 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Objectives: Extramedullary plasmacytoma in the sinonasal tract or nasopharynx is rare. The aim of the study was to review data on symptoms, clinical findings, treatment and follow-up of plasmacytomas in the sinonasal and nasopharyngeal regions in order to delineate the main clinical characteristics and the optimal management., Method: Twenty-five patients with sinonasal or nasopharyngeal plasmacytoma, diagnosed and treated at the Helsinki University Hospital during a 39-year period from 1975 to 2013 were retrospectively reviewed., Results: There were 18 males and 7 females with a median age of 66 years (range, 36-80). Sixty-eight percent received only radiotherapy or (chemo)radiotherapy. Forty-seven percent of them had a complete response to primary radiotherapy and one patient had a complete response after receiving additional brachytherapy. Four patients were treated primarily with surgery only. Two of them had a local recurrence, but were then successfully treated with radiotherapy. Altogether, four patients received a combination of surgery and (chemo)radiotherapy. Forty-four percent were alive with no evidence of disease after a median follow-up time of 78 months. Forty percent died of their disease and 16% died of other causes., Conclusions: Our study supports radiotherapy as a treatment of choice, but for small tumours surgery alone or in combination with radiotherapy may also be considered.

Objectives To investigate the clinical characteristics, survival and prognosis of patients with multiple myeloma (MM) and head extramedullary plasmacytoma (EMP). Methods Forty MM patients were enrolled in the study (18 men, 22 women; median age, 55 years). Results Median overall survival (OS) and progression-free survival (PFS) were 24 (5-78) months and 17 (2-36) months, respectively. The 2-, 3- and 5-year OS rates were 51%, 20% and 7%, respectively. The 2-year PFS was 15%. Median OS and PFS in patients administered velcade were 26 (18-50) and 22.5 (5-78) months, compared with 20 (10-30) and 13.5 (2-36) months in patients without velcade, respectively. Median OS was 23.5 (5-50) months in patients with EMP at MM diagnosis ( n = 25) and 36 (22-78) months in patients with head EMP diagnosed during the disease course ( n = 15). Sixteen MM patients had EMP invasion of the head only and 24 had invasion at multiple sites. Median OS was 25 (22-78) months in patients with EMP of the head only and 22 (5-78) months in patients with EMP invasion at multiple sites. Conclusion MM patients with head EMP show a more aggressive disease course and shorter OS and PFS. The prognosis of these patients is poor, especially in patients with head EMP at MM diagnosis, though combined chemotherapy and radiotherapy may prolong survival.

Background: Multiple myeloma/plasmacytoma-like posttransplantation lymphoproliferative disorder (PTLD-MM) is a rare complication of solid organ transplantation. Case series have shown variable outcomes, and survival data in the modern era are lacking., Patients and Methods: A cohort of 212 PTLD-MM patients was identified in the Scientific Registry of Transplant Recipients between 1999 and 2011. Overall survival (OS) was estimated by the Kaplan-Meier method, and the effects of treatment and patient characteristics on OS were evaluated by Cox proportional hazards models. OS in 185 PTLD-MM patients was compared to 4048 matched controls with multiple myeloma (SEER-MM) derived from Surveillance, Epidemiology, and End Results (SEER) data., Results: Men comprised 71% of patients; extramedullary disease was noted in 58%. Novel therapeutic agents were used in 19% of patients (more commonly during 2007-2011 vs. 1999-2006; P = .01), reduced immunosuppression in 55%, and chemotherapy in 32%. Median OS was 2.4 years and improved in the later time period (adjusted hazard ratio [aHR], 0.64, P = .05). Advanced age, creatinine > 2 g/dL, white race, and use of OKT3 were associated with inferior OS in multivariable analysis. OS of PTLD-MM patients is significantly inferior to SEER-MM patients (aHR, 1.6, P < .001). Improvements in OS over time differed between PTLD-MM and SEER-MM. Median OS of patients diagnosed from 2000 to 2005 was shorter for PTLD-MM than SEER-MM patients (18 vs. 47 months, P < .001). There was no difference among those diagnosed from 2006 to 2010 (44 months vs. median not reached, P = .5; interaction P = .08)., Conclusion: Age at diagnosis, elevated creatinine, white race, and OKT3 were associated with inferior survival in patients with PTLD-MM. Survival of PTLD-MM is inferior to SEER-MM, although significant improvements in survival have been documented., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Few studies describe the comprehensive immunophenotypic pattern of blastic plasmacytoid dendritic cell neoplasm (BPDCN) in the bone marrow and its treatment. This retrospective analysis evaluates the diagnostic flow cytometry (FCM) pattern and outcome of nine patients diagnosed with BPDCN. A four-tube 10-color FCM panel used for diagnosis of acute leukemia (AL), showed cells in the blast gate (CD45dim/low SSC) and were positive for CD4(bright), CD33(dim), CD56(heterogenous), CD123(bright), CD36, CD38, HLA-DR, CD71. Seven patients received front-line induction therapy with HyperCVAD with an overall response rate of 86%. Five of six responders underwent planned allogeneic hematopoietic cell transplantation (allo-HCT). For a median follow up of 13.3 months, the 1-year disease free survival and overall survival were 56 and 67%, respectively. An accurate diagnosis of BPDCN can be made by 10-color FCM using a four-tube AL panel demonstrating a characteristic pattern of antigen expression. Front-line induction chemotherapy with HyperCVAD can yield high remission rates, but allo-HCT is required for long-term durable remissions., (© 2015 Wiley Periodicals, Inc.)

This study aimed to review the clinical features and outcome of 53 patients with solitary plasmacytoma managed at our Institution between 1976 and 2012. Thirty-five patients had bone solitary plasmacytoma and 18 extramedullary solitary plasmacytoma. Tumour sizes were larger in patients with bone involvement (P = 0·003). Treatment consisted of local radiotherapy (n = 26), radiotherapy + chemotherapy (n = 15), surgery (n = 4) and chemotherapy (n = 8); the local control rate was 94·3%. Progression to multiple myeloma was recorded in 20/35 (57·1%) patients with bone involvement and in 1/18 (5·5%) patients with extramedullary disease (P = 0·0003). The 5-year overall survival (OS) rate was 78·4%; bone solitary plasmacytoma patients had a significantly worse OS (71·9% vs. 88·2%, respectively; P = 0·029) and 5-year progression-free survival (PFS; 53·0% vs. 88·5%; P = 0·0003) compared to extramedullary solitary plasmacytoma patients. On univariate analysis, bone disease and size (≥5 cm) impacted negatively on PFS (P = 0·0027 and P = 0·04, respectively). Bone disease also affected OS (P = 0·04). In multivariate analysis bone location was the only independent prognostic factor for PFS (P = 0·0041) and OS (P = 0·021). Patients with bone solitary plasmacytoma have a significantly worse prognosis than extramedullary solitary plasmacytoma cases., (© 2015 John Wiley & Sons Ltd.)

Myeloma-related disorder (MRD) is an uncommon disease in cats, for which there is no established standard of care. In this retrospective study, we evaluated presentation, response to treatment, and toxicity in cats with MRD receiving systemic treatment. Previously reported prognostic factors were evaluated for their impact on survival in cats receiving chemotherapy. Of fifteen cases identified, thirteen received melphalan or cyclophosphamide +/- corticosteroids as first-line therapy. Chlorambucil was commonly used as rescue therapy in cats with progressive disease, or in cases of chemotherapy-related toxicity with first line agents. Overall response rates were 71% and 83% for melphalan- and cyclophosphamide-treated cats, respectively. Discontinuation of melphalan due to toxicity was common. Survival times for cats initially treated with melphalan or cyclophosphamide were not significantly different (median 252 and 394 days, respectively), and no statistically significant prognostic factors were identified. This study suggests that the combination of cyclophosphamide and corticosteroids is well tolerated and may be considered as first-line therapy for cats with systemic MRD.

We sought to clarify the role of high-dose chemotherapy followed by autologous hematopoietic stem cell transplantation (auto-HSCT) and allogeneic hematopoietic stem cell transplantation (allo-HSCT) to treat blastic plasmacytoid dendritic cell neoplasm (BPDCN). We retrospectively identified 25 BPDCN patients (allo-HSCT, n = 14; auto-HSCT, n = 11) from registry data of the Japan Society for Hematopoietic Cell Transplantation and analyzed clinicopathologic data and clinical outcomes after transplantation. The median age at HSCT was 58 years (range, 17-67 years). All 11 patients who underwent auto-HSCT were in the first complete remission (CR1). With a median follow-up of 53.5 months, the overall survival rates at 4 years for patients who underwent auto-HSCT and allo-HSCT were 82% and 53% (P = .11), respectively, and progression-free survival rates were 73% and 48% (P = .14), respectively. Auto-HSCT for BPDCN in CR1 appears to provide promising results and deserves further evaluation in the setting of prospective trials., (© 2015 by The American Society of Hematology.)

The prognostic impact of extramedullary plasmacytomas (EMPs) on newly diagnosed symptomatic multiple myeloma (MM) was evaluated in the context of treatment approach including autologous stem cell transplantation (ASCT) and chemotherapy alone. A total of 275 consecutive patients with newly diagnosed MM were included, and 54 patients (19.6 %) had EMPs at diagnosis. Patients with initial EMPs were more likely to have myeloma bone disease but favorable laboratory parameters in hemoglobin and β2-microglobulin. Patients were treated with different schemas based on transplant eligibility (154 in ASCT-eligible vs. 121 in ASCT-ineligible). After a median follow-up of 24.6 months (range, 0.2-56.3 months) in survivors, patients with initial EMPs had significantly worse progression-free survival (PFS) (P = 0.035) and overall survival (OS) (P = 0.006) compared to those without initial EMPs. In the multivariate analyses, the presence of initial EMPs was an independent prognostic factor for PFS (relative risk (RR) of 2.24, P = 0.024) and OS (RR of 2.47, P = 0.027) in the transplant-ineligible patients, whereas it did not significantly influence PFS (P = 0.341) or OS (P = 0.499) in the transplant-eligible patients. However, the adverse impact of EMPs observed in transplant-ineligible patients was attenuated among the patients treated with bortezomib. These data suggest that ASCT can overcome the negative impact of EMPs and highlight the potential efficacy of bortezomib on EMPs in the non-transplant setting.

Background: A moderate dose of radiation is the recommended treatment for solitary plasmacytoma (SP), but there is controversy over the role of surgery. Our study aimed at comparing different treatment modalities in the management of SP., Materials and Methods: Data from 38 consecutive patients with solitary plasmacytoma, including 16 with bone plasmacytoma and 22 with extramedullary plasmacytoma, were retrospectively reviewed. 15 patients received radiotherapy alone; 11 received surgery alone, and 12 received both. The median radiation dose was 50Gy. All operations were performed as radical resections. Local progression-free survival (LPFS), multiple myeloma-free survival (MMFS), progression-free survival (PFS) and overall survival (OS) were calculated and outcomes of different therapies were compared., Results: The median follow-up time was 55 months. 5-year LPFS, MMFS, PFS and OS were 87.0%, 80.9%, 69.8% and 87.4%, respectively. Univariate analysis revealed, compared with surgery alone, radiotherapy alone was associated with significantly higher 5-year LPFS (100% vs 69.3%, p=0.016), MMFS (100% vs 51.4%, p=0.006), PFS (100% vs 33.7%, p=0.0004) and OS (100% vs 70%, p=0.041)., Conclusions: Radiotherapy alone can be considered as a more effective treatment for SP over surgery. Whether a combination of radiotherapy and surgery improves outcomes requires further study.

We aimed to compare the characteristics of skeletal and soft tissue plasmacytomas and to analyze clinical outcomes and prognostic factors of autologous stem cell transplantation (ASCT) in multiple myeloma (MM) patients with plasmacytoma. We retrospectively reviewed data from 93 myeloma patients with detectable extramedullary (EM) plasmacytoma at diagnosis or during the course of the disease, who underwent ASCT. Soft tissue plasmacytoma occurred more frequently in male patients and had higher levels of serum β2-microglobulin and lactate dehydrogenase and high frequency of advanced disease according to International Staging System compared to the skeletal plasmacytoma group. Both soft tissue and skeletal plasmacytoma groups showed similar plasmacytoma relapse patterns after ASCT and relapsed with EM plasmacytoma slightly more frequently in the bone compared to soft tissue sites. Compared to patients with skeletal plasmacytoma, patients with soft tissue plasmacytoma had worse median progression-free survival (PFS) (12 vs. 28 months) (P = 0.001) and overall survival (OS) (37 vs. 67 months) (P = 0.037) after ASCT. In a multivariate analysis, soft tissue plasmacytoma was an only independent poor prognostic factor for both PFS (HR, 2.398; 95% CI, 1.304-4.410) and OS (HR, 2.811; 95% CI, 1.107-7.135) after ASCT. These results demonstrate that, even though ASCT achieved a strong response in myeloma patients with soft tissue plasmacytoma, the presence of EM disease still contributed to a poor prognosis after ASCT compared to skeletal plasmacytoma, and these poor outcomes were not overcome by ASCT., (© 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Introduction: Bortezomib-based therapy is commonly used in treatment for relapsed or refractory multiple myeloma (MM). Unfortunately, many patients show relapse or progression in heterogeneous patterns., Patients and Methods: In this study, we retrospectively evaluated patterns of relapse or progression after bortezomib-based salvage therapy in patients with MM and analyzed prognostic significance according to patterns of relapse or progression. One hundred forty-eight patients were treated with bortezomib-based therapy between November 2004 and April 2012. Of these patients, 104 (70.3%) patients relapsed or progressed after bortezomib-based salvage therapy. We divided the patterns of relapse or progression to the 2 groups: (1) the isoform relapse or progression (group A) in 89 (85.6%) patients as disease findings at initiation of bortezomib-based therapy; and (2) transformed relapse or progression (group B) in 15 (14.4%) patients (plasmacytoma, n = 7; light chain escape, n = 6; and plasma cell leukemia, n = 2) different from initial disease findings., Results: Median overall survival in group A and group B were 32.7 months (95% confidence interval [CI], 21.3-44.1) and 10.7 months (95% CI, 2.0-19.4) (P < .001), respectively., Conclusion: MM patients who relapsed or progressed as the transformed pattern for bortezomib-based salvage therapy have an extremely poor prognosis and might require new innovative approaches., (Copyright © 2014 Elsevier Inc. All rights reserved.)

The purpose of this study was to use multiparameter flow cytometry to detect occult marrow disease (OMD) in patients with solitary plasmacytoma of bone and assess its value in predicting outcome. Aberrant phenotype plasma cells were demonstrable in 34 of 50 (68%) patients and comprised a median of 0.52% of bone marrow leukocytes. With a median follow-up of 3.7 years, 28 of 50 patients have progressed with a median time to progression (TTP) of 18 months. Progression was documented in 72% of patients with OMD vs 12.5% without (median TTP, 26 months vs not reached; P = .003). Monoclonal urinary light chains (ULC) were similarly predictive of outcome because progression was documented in 91% vs 44% without (median TTP, 16 vs 82 months; P < .001). By using both parameters, it was possible to define patients with an excellent outcome (lacking both OMD and ULC, 7.7% progression) and high-risk patients (OMD and/or ULC, 75% progression; P = .001). Trials of systemic therapy are warranted in high-risk patients., (© 2014 by The American Society of Hematology.)

Solitary plasmacytoma represents a heterogeneous group of patients; approximately half develop multiple myeloma (MM) in 2 or 3 years, whereas others remain disease-free at 10 years. By definition, these patients do not have morphologic bone marrow (BM) plasma cell (PC) infiltration. Here, we investigated whether sensitive BM evaluation of patients with solitary bone plasmacytoma (SBP; n = 35) and extramedullary plasmacytoma (EMP; n = 29) through multiparameter flow cytometry (MFC) would unravel the presence of clonal PCs in otherwise disease-free BM, and whether BM clonality predicted higher risk of progression. BM clonal PCs were detected in 17 of 35 SBP (49%) and 11 of 29 EMP (38%) patients. Seventy-one percent of flow-positive vs only 8% of flow-negative SBP patients evolved to MM (median time to progression of 26 months vs not reached; hazard ratio, 17.4; P < .001). No significant differences were observed among EMP cases. Our results highlight the importance of MFC for sensitive BM evaluation of SBP patients, to predict risk of developing treatment-requiring MM and to plan disease monitoring., (© 2014 by The American Society of Hematology.)

Solitary plasmacytoma (SP) is a rare plasma cell dyscrasia characterized by the presence of bone or extramedullary plasma cell tumors. The treatment of choice is local radiotherapy (R/T) ± surgical excision. The role of adjuvant chemotherapy (C/T) or novel agents (NA) is uncertain. Data related to prognostic factors are inconclusive. Herein, we describe the clinical features, survival and prognosis of 97 consecutive patients, 65 with bone SP (SBP), and 32 with extramedullary SP (SEP), diagnosed and treated in 12 Greek Myeloma Centers. Objective response rate (≥PR) and complete response (CR) was 91.8% and 61.9%, respectively, and did not differ between the 2 groups. Overall, 38 patients relapsed or progressed to multiple myeloma (MM). After a median follow-up of 60 months, 5 and 10-year overall survival (OS) probability was 92% and 89% in SEP and 86% and 69% in SBP, respectively (P = 0.2). The 5- and 10-year MM-free survival (MMFS) probability was 90% and 70% for patients with SEP vs. 59% and 50% for patients with SBP, respectively (P = 0.054). Overall, the 5- and 10-year OS probability, plasmacytoma relapse-free survival (PRFS), progression-free survival and MMFS was 84% and 78%, 72% and 58%, 58% and 43%, and 70% and 59%, respectively. In the multivariate analysis, prolonged PRFS and young age were positive predictors of OS. Achievement of CR was the only positive predictor of PRFS. Immunoparesis was the only negative predictor of progression to MM. The addition of C/T or NA-based treatment increased toxicity without offering any survival advantage over R/T., (© 2014 Wiley Periodicals, Inc.)

Purpose: Solitary plasmacytoma (SP) is a localized proliferation of monoclonal plasma cells in either bone or soft tissue, without evidence of multiple myeloma (MM), and whose prognosis is marked by a high risk of transformation to MM., Experimental Design: We studied the impact of FDG-PET/CT (2[18F]fluoro-2-deoxy-D-glucose positron emission tomography-computed tomography) on the risk of transformation of SP to overt MM among other markers in a series of 43 patients diagnosed with SP., Results: Median age was 57.5 years; 48% of patients had an abnormal involved serum-free light chain (sFLC) value, and 64% had an abnormal sFLC ratio at diagnosis. Thirty-three percent had two or more hypermetabolic lesions on initial PET/CT, and 20% had two or more focal lesions on initial MRI. With a median follow-up of 50 months, 14 patients transformed to MM with a median time (TTMM) of 71 months. The risk factors that significantly shortened TTMM at diagnosis were two or more hypermetabolic lesions on PET/CT, abnormal sFLC ratio and involved sFLC, and to a lesser extent at completion of treatment, absence of normalized involved sFLC and PET/CT or MRI. In a multivariate analysis, abnormal initial involved sFLC [OR = 10; 95% confidence interval (CI), 1-87; P = 0.008] and PET/CT (OR = 5; 95% CI, 0-9; P = 0.032) independently shortened TTMM., Conclusions: An abnormal involved sFLC value and the presence of at least two hypermetabolic lesions on PET/CT at diagnosis of SP were the two predictors of early evolution to myeloma in our series. This data analysis will need confirmation in a larger study, and the study of these two risk factors may lead to a different management of patients with SP in the future. ., (©2014 American Association for Cancer Research.)

Background: This study reviews the published literature related to extramedullary sinonasal plasmacytomas. Clinical presentation, demographics, treatment, and outcomes of this uncommon disease are reported., Methods: A systematic review of studies for sinonasal plasmacytomas from 1950 to 2012 was conducted. A PubMed database search, both for articles related to this condition along with bibliographies of those selected articles, was performed. Articles were examined for patient data that reported disease outcome., Results: Sixty-seven journal articles were included in this analysis, comprising a total of 175 cases. Radiotherapy was the most common treatment modality, used in 89 cases, followed by a combination of surgery and radiotherapy, and surgery alone. A total of 71.8% of patients were alive after a median follow-up of 39 months, independent of treatment modality. A combination of radiotherapy and chemotherapy was rarely used but had the best treatment outcome, with 88.9% of patients (8/9 patients) alive. Of the 3 most common treatment modalities, a combination of radiotherapy and surgery had the most favorable outcomes. Sixteen patients (9.1%) converted to multiple myeloma, with the majority of these patients (75.0%) receiving radiotherapy alone as their treatment modality., Conclusion: This review contains the largest pool of sinonasal plasmacytoma patients to date and suggests aggressive radiotherapy is the most common treatment modality for this condition. Of the 3 most common treatment modalities, a combination of surgery and radiotherapy was shown to have the best survival outcomes., (© 2013 ARS-AAOA, LLC.)

Plasma cell dyscrasias account for 1% of all malignancies and 10% of hematological malignancies. About 5% of patients with a plasma cell dyscrasia present with either solitary bone plasmacytoma (SBP) or, less commonly, a soft tissue mass, extramedullary plasmacytoma (EMP) of monoclonal plasma cells. In this study we present the clinical features, management and outcome of 60 patients with solitary plasmacytoma, who constituted 5.6% of 1129 patients with plasma cell dyscrasias treated over a 10-year period at All India Institute of Medical Sciences. Median age was 49 years. Fifty-two had SBP and eight had EMP. Forty-nine (82%) patients received radiotherapy with or without surgical excision or chemotherapy. Five-year event-free survival (EFS) and overall survival (OS) were 45.5% and 91%, respectively. Median EFS and OS were 38 and 122 months, respectively. Five-year survival rates in patients who developed multiple myeloma and those who did not were 81% and 100%, respectively. The median time of progression to myeloma was 21 months.

Extramedullary plasmacytoma (EMP) of the skin is a rare indolent neoplasm that shares morphological and immunophenotypic features with plasma cell myeloma (PCM), but the molecular features that distinguish these two entities have not been defined. We reviewed the clinical characteristics, course, and molecular abnormalities in 7 cases of cutaneous EMP (cEMP); 2 patients had primary cEMP and 5 had secondary cEMP. Two patients died of progressive extramedullary plasmacytoma, 1 without PCM; 1 patient who had only a hyperdiploid clone, died within 17 months of the diagnosis of cEMP; and 3 died of PCM. One patient, who had cEMP with a hyperdiploid clone and a 13q deletion, was alive 28 months after diagnosis. Our findings raise questions about the relative prognostic value of molecular aberrations observed in cEMP and PCM. The role of fluorescence in situ hybridization testing in predicting disease progression of cEMP remains to be defined.

We report on 205 patients with haematologic neoplasms of bone treated from 1985 to 2009. There were 77 patients with primary bone lymphoma, 77 with myeloma and 51 with plasmacytoma. All patients had medical treatments; 43 patients had wide and 162 intralesional surgery. Mean follow-up was 5 years (median, 3.5 years); 11 patients were lost to followup. At the latest examination, 99 patients were alive without disease, 20 were alive with disease and 75 were dead of disease; 13 patients (6.7%) had local recurrence; 12 patients (24%) with plasmacytoma developed myeloma. Survival to death was significantly higher after wide resection for lymphoma and plasmacytoma, but not for myeloma. Survival to local recurrence was not statistically different between wide and intralesional surgery for any haematologic neoplasm. Surgical complications including aseptic loosening, infection, neurological deficits and breakage of implants occurred in 21 patients (11%).

Purpose: The purpose of this study was to elucidate the efficacy and optimal method of radiotherapy in the management of solitary extramedullary plasmacytoma occurring in the head and neck regions (EMPHN)., Methods and Materials: Sixty-seven patients (43 male and 24 female) diagnosed with EMPHN between 1983 and 2008 at 23 Japanese institutions were reviewed. The median patient age was 64 years (range, 12-83). The median dose administered was 50 Gy (range, 30-64 Gy). Survival data were calculated by the Kaplan-Meier method., Results: The median follow-up duration was 63 months. Major tumor sites were nasal or paranasal cavities in 36 (54%) patients, oropharynx or nasopharynx in 16 (23%) patients, orbita in 6 (9%) patients, and larynx in 3 (5%) patients. The 5- and 10-year local control rates were 95% and 87%, whereas the 5- and 10-year disease-free survival rates were 56% and 54%, respectively. There were 5 (7.5%), 12 (18%), and 8 (12%) patients who experienced local failure, distant metastasis, and progression to multiple myeloma, respectively. In total, 18 patients died, including 10 (15%) patients who died due to complications from EMPHN. The 5- and 10-year overall survival (OS) rates were 73% and 56%, respectively. Radiotherapy combined with surgery was identified as the lone significant prognostic factor for OS (p = 0.04), whereas age, gender, radiation dose, tumor size, and chemotherapy were not predictive. No patient experienced any severe acute morbidity., Conclusions: Radiotherapy was quite effective and safe for patients with EMPHN. Radiotherapy combined with surgery produced a better outcome according to survival rates. These findings require confirmation by further studies with larger numbers of patients with EMPHN., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Solitary plasmacytoma (SP) is characterized by a mass of neoplastic monoclonal plasma cells in either bone (SBP) or soft tissue without evidence of systemic disease attributing to myeloma. Biopsy confirmation of a monoclonal plasma cell infiltration from a single site is required for diagnosis. The common presentation of SBP is in the axial skeleton, whereas the extramedullary plasmacytoma (EMP) is usually seen in the head and neck. The ratio of SP seen at males to females is 2 : 1 and the median age of patients is 55 years. The incidence rate of SP in black race is approximately 30% higher than the white race. Incidence rate increases exponentially by advancing age. SBP has a significant higher risk for progression to myeloma, and the choice of treatment is radiotherapy (RT) that is applied with curative intent at min. 4000 cGy. By only RT application, long-term disease-free survival (DFS) is possible for approximately 30% of patients with SBP and 65% of patients with EMP.

Background: Extraosseous plasmacytoma, so called extramedullary plasmacytoma (EMP) is relatively rare in China. The aim was investigate the clinicopathologic features of EMP and the role of Immunophenotype and genotype detection in diagnosis of EMP., Methods: Thirty-two cases of EMP were investigated retrospectively by histopathology, immunophenotype, genotype and survival analysis., Results: Clinically, the mean age of the patients was 53.4. Most of the patients received no treatment after the diagnosis was established, and the prognosis was relatively poor. Histologically, in 40% of the cases, the neoplastic cells were grade II or III. The neoplastic cells expressed one or more PC associated antigens. The immunophenotype of EMP and inflammation of sinonasal regions with numerous PC infiltrations were compared and showed some difference in expression of CD45, CD27, CD44v6 and Bcl-2 as well. Ig light chain restriction was detected in 87.5% of the cases., Conclusions: we described 32 Chinese cases of EMP, compare with that reported in the literature, some differences are presented, including higher percentage of grade II and III cases, clinically inconsistent treatment and management as well as poor outcome of the disease.

Background: The objective of this study was to review the outcome of patients with solitary plasmacytoma (SP) after definitive radiation therapy., Methods: The authors retrospectively reviewed 84 patients with SP who were diagnosed and treated at The University of Texas MD Anderson Cancer Center during 1988 to 2008. The impact of tumor anatomic site, tumor size, and the presence of serum and urinary paraprotein at diagnosis was assessed on local control, survival, and the risk of developing multiple myeloma (MM)., Results: Fifty-nine patients (70%) had bone SP, and 25 patients (30%) had extramedullary SP. Serum paraprotein was present in 39 patients (46%). The median radiation dose was 45 grays (Gy) (range, 36-53.4 Gy). Local control was achieved in 77 patients (92%). Neither radiation dose nor tumor size predicted local control. The 5-year rate of progression to MM was 47% and was higher for patients with bone SP (56% vs 30% for extramedullary SP; P = .021), and patients who had serum paraprotein detected at diagnosis (60% vs 39%; P = .016). On univariate analysis, patients aged <60 years and men had higher rates of progression to MM, although the differences were not significant (P = .048 and P = .29, respectively). Multivariate analysis revealed that bone location and serum protein at diagnosis were associated statistically with progression to MM. The 5-year overall survival rate for the entire patient cohort was 78%, and no difference was observed between patients who had bone SP versus extramedullary SP (76% vs 85%, respectively; P = .274)., Conclusions: The current results indicated that definitive radiation therapy for SP can provide excellent local control. Progression to MM remains the main problem and is more common among patients with bone SP and those who have serum paraprotein detected at diagnosis., (Copyright © 2011 American Cancer Society.)

Solitary plasmacytoma of the bone (SBP) or extramedullary plasmacytoma (EP) are rare neoplasms amenable to local radiotherapy. In this retrospective analysis, we report the University Heidelberg experience in the treatment of solitary plasmacytoma. From 1995 to 2008, 18 patients were treated with local radiotherapy. Ten patients suffered from SBP, eight patients showed a single extramedullary lesion. Local radiotherapy with a median dose of 45 Gy yielded excellent local control with only one patient suffering from local relapse. SBP and EP had significantly different 5-year multiple myeloma-free survival rates of 36.8% and 86.7%, respectively. However, no significant difference in overall survival could be detected. Radiotherapy can achieve excellent local control of solitary plasmacytoma. Progression to multiple myeloma, especially in the case of SBP, remains to be addressed by further studies.

We compared the differences in a number of angiogenesis-related immunohistochemical parameters, including microvascular density (MVD) and tumor cell activity, between multiple myeloma (MM) and solitary plasmacytoma (SP). Tissue sections from tumors of MM and SP were immunohistochemically stained and analyzed using ImageJ image analysis software for the expression of vascular endothelial growth factor (VEGF), VEGF receptors (Flt-1 and Flk-1), inducible nitric oxide (iNOS), and anti-apoptotic (Bcl-2) protein. Tumor tissues were cytologically graded as high-, intermediate-, or low-grade. Two pathologists determined the MVD of each section independently by recording the average number of CD34+ blood vessels in 500 unit fields. The arithmetic means for MVD were statistically analyzed using the Student's t-test and the significance level was calculated at P-value <0.001. The results indicate a direct correlation between upregulation of iNOS/VEGF in high-grade tumors. For MM, an increase in MVD is also correlated with a high-grade. Tumor survival signaling by Bcl-2 in both SP and MM emphasizes the fact that VEGF has a bimodal role that is mainly angiogenic in MM and tumorigenic, promoting tumor cell survival in SP., (Copyright © 2010 Elsevier GmbH. All rights reserved.)

Background: Blastic plasmacytoid dendritic cell neoplasm is a rare malignancy that typically follows a highly aggressive clinical course in adults, whereas experience in children with this disease is very limited., Design and Methods: This retrospective study analyzed the pathological and clinical findings of nine cases of blastic plasmacytoid dendritic cell neoplasm presenting in patients under the age of 18 years who were reviewed at our institution. We also identified 20 well-documented additional pediatric cases in the literature., Results: In the combined analysis, the overall survival rate among the 25 patients with available follow-up, all having received chemotherapy, was 72% (follow-up ranging from 9 months to 13 years, with a median of 30 months). The event-free survival rate was 64%. Nine patients were alive 5 years after the original diagnosis, although only three of them had undergone hematopoietic stem cell transplantation--one in first complete remission and two in second remission. Of the seven patients who lacked cutaneous disease at presentation, 100% survived, including five who were alive more than 5 years after diagnosis, although only two had undergone stem cell transplantation. Among the 18 patients who presented with cutaneous disease and for whom follow-up data were available, only 11 survived (61%). Detailed immunophenotypic characterization and clinical features of all cases are presented. Unexpectedly, three of four cases of blastic plasmacytoid dendritic cell neoplasm tested showed focal positivity for S-100. S-100 was negative in 28 cases of acute myeloid leukemia evaluated for this marker., Conclusions: In contrast to adult cases, in which long-term survival depends on stem cell transplantation in first complete remission, blastic plasmacytoid dendritic cell neoplasms in children are clinically less aggressive. Treatment with high-risk acute lymphoblastic leukemia-type chemotherapy appears to be effective, and stem cell transplantation may be reserved for children who relapse and achieve a second remission. Outcomes were more favorable in cases that lacked cutaneous disease at presentation, although a comparison of cutaneous and non-cutaneous cases might be confounded by differences in treatment regimens. Focal expression of S-100 may be seen in concert with other markers of plasmacytoid dendritic cells.

Background: Primary extramedullary plasmocytomas (EMPs) are plasma cell tumours that arise outside the bone marrow. As these tumours are rare and can present without a typical clinical picture, correct diagnosis is difficult to confirm, particularly when the lesions occur at uncommon sites., Case Report: This is a case report of a 63-year-old patient who initially presented with an isolated lump on his left knee. Biopsy showed an anaplastic tumour which was first diagnosed as localised rhabdomyosarcoma; therefore, a combined approach with neoadjuvant radiotherapy followed by surgery was recommended. Shortly before radiotherapy started, the patient suffered a stroke with hemiplegia. CT and MRI of the head revealed a single brain lesion. A resection of this cerebral lesion was performed and surprisingly revealed a cerebral EMP. After comprehensive review of this specimen and the previous biopsy, the diagnosis was changed to anaplastic bifocal EMP. Generalised myeloma was excluded; therefore the patient was treated with definitive radiotherapy of the left knee region and postoperative partial brain irradiation. Unfortunately, the patient died several months later due to fulminant progressive disease at both treated sites despite rapidly initiated chemotherapy., Conclusion: Correct diagnosis of EMP may be difficult, particularly as this disease is rare and can present with atypical clinical picture and immunophenotype. Review of the specimen by a histopathologist with special interest in soft tissue tumours or lymphoproliferative disorders is strongly recommended.

Background: Previous reports suggest an as yet unidentifiable subset of patients with plasmacytoma will progress to myeloma. The current study sought to establish the risk of developing myeloma and determine the prognostic factors affecting the progression of disease., Methods: Patients with plasmacytoma diagnosed between 1973 and 2005 were identified in the SEER database(1164 patients). Patient demographics and clinical characteristics, treatment(s), cause of death, and survival were extracted. Kaplan-Meier, log-rank, and Cox regression were used to analyze prognostic factors., Results: The five year survival among patients initially diagnosed with plasmacytoma that later progressed to multiple myeloma and those initially diagnosed with multiple myeloma were almost identical (25% and 23%; respectively). Five year survival for patients with plasmacytoma that did not progress to multiple myeloma was significantly better (72%). Age > 60 years was the only factor that correlated with progression of disease (p = 0.027)., Discussion: Plasmacytoma consists of two cohorts of patients with different overall survival; those patients that do not progress to systemic disease and those that develop myeloma. Age > 60 years is associated with disease progression. Identifying patients with systemic disease early in the treatment will permit aggressive and novel treatment strategies to be implemented.