Bone Plasmacytoma Research Articles

Extramedullary Plasmacytoma Over the scar from Long-lasting Past Surgery as Presenting Feature of Multiple Myeloma

Plasmacytomas can have different clinical presentations. They can be observed as a single tumor mass without bone marrow involvement (solitary plasmacytoma) or in the context over multiple myeloma. In rare instances plasmacytomas are triggered by invasive surgical procedures. These different types of plasmacytomas can be found in the same patient which suggests a special biology of the malignant plasma cell that facilitates the spread outside the bone marrow. Here, we report the unique case of a 37-year-old man with solitary bone plasmacytoma with soft-tissue involvement who progressed to multiple myeloma with a huge mass at the scar of inguinal hernia surgery performed 10 years before. Throughout the course of the disease, he developed extensive multiple paraskeletal and extramedullary plasmacytomas at different locations.

Solitary Plasmacytoma of Rib Origin in a Young Adult- A Rare CaseReport

Solitary plasmacytoma of bone, specially of a single rib, is a rare disease and is characterized by onlyone or two isolated bone lesions with no evidence of disease dissemination. Here we present a caseof 35-year-old male who was presented with a recurrent swelling in left lower lateral chest wall withsymptoms of localized pain. Radiological evaluation revealed a solitaryexpanding lesion in left lowerlateral chest wall. Fine needle aspiration showed numerous small round cells having hyperchromaticnuclei with scanty cytoplasm. en-bloc resection of the tumor, including involved part of left 9th rib,the intercostal muscle and the parietal pleura with primary closure of chest wall. Histopathology ofthe resected mass revealed plasma cell neoplasm, highly suggestive of solitary plasmacytoma. Thepatient referred to oncologist for further management.
 Journal of Surgical Sciences (2017) Vol. 21 (1) :39-41

First-in-Human Assessment of Feasibility and Safety of Multiplexed Genetic Engineering of Autologous T Cells Expressing NY-ESO -1 TCR and CRISPR/Cas9 Gene Edited to Eliminate Endogenous TCR and PD-1 (NYCE T cells) in Advanced Multiple Myeloma (MM) and Sarcoma

Stadtmauer: Celgene: Consultancy; Takeda: Consultancy; Janssen: Consultancy; Amgen: Consultancy; Novartis: Consultancy, Research Funding; Tmunity: Research Funding; Abbvie: Research Funding. Cohen:Poseida Therapeutics, Inc.: Research Funding. Lacey:Novartis: Patents & Royalties: Patents related to CAR T cell biomarkers; Tmunity: Research Funding; Novartis: Research Funding. Melenhorst:Incyte: Research Funding; Novartis: Research Funding, Speakers Bureau; Parker Institute for Cancer Immunotherapy: Research Funding; Genentech: Speakers Bureau; Stand Up to Cancer: Research Funding; IASO Biotherapeutics, Co: Consultancy; Simcere of America, Inc: Consultancy; Shanghai Unicar Therapy, Co: Consultancy; Colorado Clinical and Translational Sciences Institute: Membership on an entity's Board of Directors or advisory committees; National Institutes of Health: Research Funding. Fraietta:Tmunity: Research Funding; Cabaletta: Research Funding; LEK Consulting: Consultancy. Mangan:amgen: Speakers Bureau; takeda: Speakers Bureau; celgene: Speakers Bureau; janssen: Speakers Bureau. Lancaster:novartis: Research Funding. Suhoski:novartis: Research Funding. Fesnak:Novartis: Research Funding. Young:novartis: Research Funding. Chew:tmunity: Other: Scientific Founder, Research Funding; novartis: Research Funding. Zhao:Tmunity: Membership on an entity's Board of Directors or advisory committees, Research Funding; novartis: Research Funding. Hwang:Novartis: Research Funding; Tmunity: Research Funding. Hexner:novartis: Research Funding. June:Novartis: Research Funding; Tmunity: Other: scientific founder, for which he has founders stock but no income, Patents & Royalties.

Clinical Characteristics, Treatment Patterns and Outcomes of Solitary Plasmacytoma - a Multicenter Retrospective Cohort Study

Background: Solitary plasmacytoma (SPC) is a rare plasma cells disorder, which may present as a single bone lesion or an extramedullary plasmacytoma. High dose radiotherapy may be curative, yet most patients eventually develop Multiple Myeloma (MM). Due to rarity of the disease, there is uncertainty regarding the optimal treatment strategy, especially in patients that present with known risk factors. The role of adjuvant anti-myeloma therapy is still under debate, whereas few studies have reported efficacy of novel agents. We aimed to explore clinical characteristics and treatment patterns and to identify predictors for relapse and progression to myeloma in a real world setting. Methods: Consecutive patients, diagnosed and treated for solitary plasmacytoma in 13 centers, between 2008 and 2019 (defined by the IMWG criteria) were identified, based on medical centers patient registries and pathological reports. Patient's medical charts were reviewed, and data regarding patient and disease characteristics, treatment regimen, and clinical endpoints, focusing on response to treatment and time to disease progression were documented. The treatment response categories were defined according to the IMWG criteria. Results: 62 patients (pts) were identified. Baseline characteristics and workup are detailed in the table. Median follow-up was 39 months (mo) (IQR: 13.5, 63.3). Fifty nine pts (95.2%) were treated with radiotherapy, with a median dose of 45.0 Gy (range: 30-54 Gy); radiotherapy dose was 45-54 in 31 pts, < 45Gy in 11 pts. Twenty pts (33.9%) underwent surgery in addition to radiotherapy, 3 more pts underwent a surgery alone. The median time to radiotherapy since diagnosis was 2.7 months (range: 1day-18.2 months), 8 pts received any anti-myeloma therapy for treatment of plasmacytoma including Melphalan/dexamethasone (n=1), bortezomib based induction followed by ASCT (n=2), VD/VTD (n=2) and KRD (n=1). Among 25 patients with evaluable post-therapy PET-CT response (performed 12 months post radiotherapy), 26% had no remaining FDG uptake, 20% had a 50% or greater decrease in FDG uptake. Among 40 patients with CT or MRI post therapy assessment, 20% had disappearance of SPC, 27% had ≥50% reduction, 7% had <50% reduction and 25% remained without change. Non-bone SPC were more likely to achieve CR than bone SPCs, 86% vs 29%, respectively, p=0.021. During the follow-up period, 16 pts (25.8%) experienced evolution to MM (15 pts had bony and 1 soft tissue plasmacytoma in diagnosis). Among patients with bone SPC (n=49), 30.5% evolved to MM. A total of 6 pts had a recurrence of SPC (5 pts had a bony and 1 patient upper aerodigestive plasmacytoma in diagnosis). 5 pts died (3 without recurrence or evolution to MM). Median time to plasmacytoma recurrence in pts that achieved CR was 36.7 mo (range: 3 -67.9 mo); Median time of evolution to MM was 37.5 mo (range: 6.7-87.2 mo). Progression free survival (PFS) was 67.7 mo for all pts (Fig1A), 48 mo for bone SPC and was not reached for non-bone SPC (p=0.11) (Fig1B). Median overall survival was not reached. Overall survival at median follow-up was 93.6%. Notably, all 8 pts who were treated with anti-myeloma therapy had a bony plasmacytoma and received radiotherapy in addition. None of these pts had recurrence of plasmacytoma or evolution to MM. Of the 11 pts that had SPC in an upper aerodigestive tract site, none evolved to MM; 10 of these pts were treated with only radiotherapy, one had a plasmacytoma recurrence in the same site. Conclusions: We present real world data of a multicenter patient cohort with SPC. As previously suggested, prognosis is more favorable for none bone SPCs. Though numbers are modest, our analysis suggests a benefit of the early addition of systemic therapy to standard radiotherapy for patients with solitary bone plasmacytoma. Disclosures Yael: Janssen: Consultancy; Amgen: Consultancy, Research Funding; Neopharm: Consultancy; Takeda: Consultancy; Madison: Consultancy; VBL Therapeutics: Employment.

Does Radiotherapy with Surgery Improve Survival and Decrease Progression to Multiple Myeloma in Patients with Solitary Plasmacytoma of Bone of the Spine?

To investigate the outcomes of radiotherapy with or without surgery during treatment of patients with solitary plasmacytoma of bone (SBP) of the spine. Patients diagnosed with SBP of the spine treated with radiotherapy with or without surgery were identified and extracted from the SEER database. Propensity score matched (PSM) analysis was performed to balance patient characteristics between radiotherapy alone and radiotherapy with surgery groups. Patients in different age-groups were stratified and analyzed. A total of 1275 patients with SBP of the spine treated with radiotherapy with or without surgery were extracted from the SEER database. Before PSM, the unadjusted Kaplan-Meier curve showed that the radiotherapy with surgery group had worse overall survival than did the radiotherapy without surgery group (both P < 0.05), whereas the difference of overall survival was attenuated after PSM. Stratified analysis found that the radiotherapy with surgery group had less progression to multiple myeloma for young patients (age <45 years) with SBP of the spine than did the radiotherapy without surgery group. The results of our study suggest that radiotherapy with surgery may show less progression to multiple myeloma for younger patients with SBP of the spine.

Solitary Plasmacytoma of bilateral temporal bone in a patient with multiple myeloma

Plasmacytomas are tumors that results from monoclonal proliferation of plasma cells. Disseminated form is called Multiple Myeloma (MM) which is characterized by multiple lytic bone lesions on imaging and monoclonal gammopathy.

Solitary Plasmacytoma of bilateral temporal bone in a patient with multiple myeloma

Introduction: Plasmacytomas are tumors that results from monoclonal proliferation of plasma cells. Disseminated form is called Multiple Myeloma (MM) which is characterized by multiple lytic bone lesions on imaging and monoclonal gammopathy.

1943 Gastrointestinal Plasmacytomas

INTRODUCTION: Plasmacytomas are tumors consist of abnormal plasma cells.They are divided into solitary plasmacytomas of the bone and extramedullary plasmacytomas (EMP). GI tract involvement is seen in 7% of EMP cases. The small intestine is the most commonly involved GI site, followed by stomach, colon, and esophagus. CASE DESCRIPTION/METHODS: A 57-year-old African American female with a history of multiple myeloma treated with chemotherapy followed by autologous stem cell transplant with failure to achieve remission. She presented with a two day history of melena, hematochezia and fatigue. Vital signs: BP 85/45 mmHg, HR 98 bpm, T 98.6 F, RR 18, Oxygen sat 96% on RA. Labs showed a drop in hemoglobin from 8.4 g/dL to 4.9 g/dL. Upper endoscopy showed 1.5 cm polyp in the gastric body (Figure 1a) that was removed. Colonoscopy showed multiple colonic polyps with a 1.5 cm polyp (Figure 1b) in the ascending colon that was removed. Histopathologic examination of stomach polyp (Figure 2a,d) revealed a malignant neoplasm consisting of plasmacytoid cells with inconspicuous nucleoli and abundant mitotic activity. The neoplastic cells were directly infiltrating the residual gastric epithelium and extending beyond the muscularis mucosa. Immunohistochemical staining showed the cells to be strongly positive for CD138 and lambda light chain, while negative for CD20, CD3, and Kappa light chain. These findings were consistent with a diagnosis of plasmacytoma. Histopathologic examination of the colon polyp (Figure 2e,f) showed similar morphology to the gastric polyp. DISCUSSION: Gastrointestinal plasmacytomas are rare, accounting for approximately 7% of all EMPs. Endoscopically, GI plasmacytomas have variable morphological appearances including ulcers, polyposis, thickened folds, ulcerative masses, and constricting lesions. Differentiating GI plasmacytomas from other tumors require histopathological examination. GI plasmacytomas should be considered in the differential diagnosis of myeloma patients presenting with GI bleed.

1621 Extramedullary Plasmacytoma of the Colon

INTRODUCTION: Plasma cell tumors can be categorized into multiple myeloma or plasmacytomas. Plasmacytoma is the proliferation of neoplastic monoclonal plasma cells in sites other than the bone marrow. This case illustrates a rare case of plasmacytoma in the colon. CASE DESCRIPTION/METHODS: A 49-year-old man with medical history of type 1 DM and GERD presented for average risk screening colonoscopy. Colonoscopy showed a 12mm transverse colon polyp that was resected via hot snare polypectomy. Pathology of the 12mm polyp showed lamina propria expansion with large sheets of plasma cells. On immunohistochemistry (IHC), the plasma cells showed kappa light chain restriction. IHC gene rearrangement studies showed a low level of immunoglobulin heavy chain amplification. Pathology was consistent with plasmacytoma associated with tubular adenoma without high grade dysplasia. Margins were negative. A complete blood count obtained by primary care physician demonstrated Hgb of 14.1 g/dl. Complete metabolic panel was normal. Serum protein electrophoresis, urine Bence Jones protein, bone marrow biopsy, skeletal bone survey and PET CT scan were recommended to exclude multiple myeloma and involvement at other sites. DISCUSSION: Plasmacytoma can be either be solitary bone plasmacytoma or solitary extramedullary plasmacytoma. The diagnosis of solitary plasmacytoma depends on the exclusion of multiple myeloma via normal bone marrow biopsy, normal skeletal survey and absence of end organ damage. If multiple myeloma is present, the lesion is characterized as an extramedullary manifestation of multiple myeloma. Solitary bone plasmacytoma occurs most commonly in the vertebrae. Solitary extramedullary plasmacytoma involves sites other than bone or bone marrow. It most commonly involves the nasopharynx or upper respiratory tract and is observed in 3-5% of plasma cell tumors. Colonic extramedullary plasmacytoma is rare and occurs in approximately 10% of extramedullary cases. Clinical presentations of extramedullary plasmacytoma are variable and depend on site of involvement. There are no current guidelines for the treatment of patients with colonic plasmacytoma. It is unclear whether endoscopic or surgical resection alone suffices as conclusive management or additional treatment modalities like radiotherapy or chemotherapy is necessary.

Reossification and prognosis following radiotherapy with/without surgery for spinal solitary plasmacytoma of the bone: a retrospective study of 39 patients

BACKGROUND CONTEXTSolitary plasmacytoma of bone (SPB) can progress to multiple myeloma (MM). Little attention has been paid to the reossification findings on computed tomography (CT) and their correlation with prognosis after radiotherapy with/without surgery. PURPOSETo evaluate reossification after radiotherapy and prognostic factors of spinal SPB using single-center data. STUDY DESIGNRetrospective observational study. PATIENT SAMPLEPatients who had spinal SPB and received radiotherapy with/without surgery, without chemotherapy, denosumab or zoledronic acid. OUTCOME MEASURESMM progression rate, mortality rate, and reossification rate at 12 months. METHODSThis retrospective clinical review included 39 patients who underwent radiotherapy as first-line treatment for SPB in the spine. External radiation was divided into 20–25 fractions with a total dose of 35–46 Gy. At the 12-month follow-up after the index radiotherapy, significant and mild reossification, defined as bone formation with ≥30% or 0%–30% increase, respectively, in bony area based on increase in CT values were documented, along with progressive disease, which was a decrease in bony area with lesion enlargement. This study was funded by AO Foundation, AOSpine (AOSDIA2019-026) (CHF45,000), Peking University Medicine Seed Fund for Interdisciplinary Research (BMU2018MX022) (¥40,000), and Peking University Third Hospital (No. Y71508-01) (¥400,000). RESULTSTwenty-six men and 13 women (mean age, 51.5 years) were included. Solitary plasmacytomas were located in the cervical, thoracic, and lumbar vertebrae in 16, 17, and 6 patients, respectively. The mean clinical follow-up period after treatment was 72 (range 12–216) months. Sixteen patients (41.0%) had significant reossification after radiotherapy, 21 (53.8%) showed mild reossification, and 2 (5.2%) had progressive bony destruction (after 7 and 23 months, respectively). There were no significant differences in age among the three groups (p=.127). At a mean follow-up of 37 (range 6–90) months after radiosurgery, 14 (35.8%) patients developed MM, including 9 patients who died at a mean duration of 55 (range 19–102) months. In the significant reossification group, only 1 patient (6.3%, 1 of 16) had MM progression 82 months after treatment (p=.044). In the mild reossification group, 56.5% (13 of 23) of patients had MM progression. The significant reossification rates of the radiotherapy dose groups of <40 Gy and ≥40 Gy were 35.7% and 44% (p=.614), respectively. In the univariate analysis, age ≥65 years (p<.001), tumor ≥5 cm (p=.009), Spinal Instability Neoplastic Score scores ≥11.5 (p=.040), radiotherapy (RT) combined with surgery (p<.001), and progression to MM (p=.007) were the independent prognostic factors for overall survival; whereas, age >44 years (p=.045) and RT combined with surgery (p<.001) were for multiple myeloma-free survival. In the multivariate analyses, age >65 years (p=.004) and progression to MM (p=.007) were the unfavorable independent factors for overall survival, whereas RT combined with surgery (p=.004) was the only factor for multiple myeloma-free survival. CONCLUSIONSIn patients with spinal SPB, 41.0% lesions showed significant reossification after radiotherapy. Patients with significant reossification had a better prognosis with less possibility of MM progression. Radiotherapy may be a safe and effective treatment choice for spinal SPB; more attention should be paid to reossification.

Clinicopathological spectrum of solitary Plasmacytoma: a single center experience from coastal India

BackgroundPlasma cell disorders are a rare group of hematological malignancies that accounts for 10% of all hematological neoplasms. Solitary plasmacytomas are rarer entities accounting for less than 5% of all the plasma cell dyscrasias. They encompass three subtypes - Solitary Plasmacytoma of Bone (SPB) and Solitary Extramedullary Plasmacytoma (SEP) and multiple solitary plasmacytomas (MSP). In this study, we discuss the clinical, histopathological and immunohistochemical characteristics of solitary plasmacytomas.MethodsA 13 year retrospective analysis of solitary plasmacytomas was performed from a single tertiary care center. Bone marrow evaluation was done concurrently at the time of diagnosis to rule out the presence of multiple myeloma.ResultsA total of 29 cases fulfilled the diagnostic criteria for SP during the study period. SPB accounted for 55.2%, SEP for 44.4% and MSP for 3.4% of the cases. The most common sites involved were the paranasal sinuses and vertebrae. Other infrequent sites included lymph node, tonsil and lungs. The mean age of presentation of SPB was a decade later than SEP. A male preponderance was observed in both subtypes.ConclusionSolitary plasmacytoma is a rare entity, the diagnosis of which requires a systematic approach. There is limited data available in the literature on the clinico-pathological characteristics of SP from India.

Hematogenous extramedullary relapse in multiple myeloma - a multicenter retrospective study in 127 patients.

The current study assesses the characteristics and outcomes of multiple myeloma (MM) patients, treated with novel agents for hematogenous extramedullary (HEMM) relapse. Consecutive patients diagnosed with HEMM between 2010-2018 were included. Patients' characteristics at diagnosis and at HEMM presentation, response to treatment, survival and factors predicting survival were recorded and analyzed. A group of 127 patients, all diagnosed with HEMM by imaging (87.3%) and/or biopsy (79%), were included. Of those, 44% were initially diagnosed with ISS3, 57% presented with plasmacytomas, and 30% had high-risk cytogenetics. Median time to HEMM was 32 months. In multivariate analysis, ISS3 and bone plasmacytoma predicted shorter time to HEMM (P = .005 and P = .008, respectively). Upfront autograft was associated with longer time to HEMM (P = .002). At HEMM, 32% of patients had no BM plasmacytosis, 20% had non-secretory disease and 43% had light-chain disease. Multiple HEMM sites were reported in 52% of patients, mostly involving soft tissue, skin (29%), and pleura/lung (25%). First treatment for HEMM included proteasome inhibitors (50%), immunomodulatory drugs (IMiDs) (39%), monoclonal antibodies (10%), and chemotherapy (53%). Overall response rate (ORR) was 57%. IMiDs were associated with higher ORR (HR 2.2, 95% CI 1.02-4.7, P = .04). Median survival from HEMM was 6 months (CI 95% 4.8-7.2). Failure to achieve ≥VGPR was the only significant factor for worse OS in multivariate analyses (HR = 9.87, CI 95% 2.35 - 39, P = .001). In conclusion, HEMM occurs within 3 years of initial myeloma diagnosis and is associated with dismal outcome. The IMiDs might provide a higher response rate, and achievement of ≥VGPR predicts longer survival.

Multifocal Extramedullary and Multiple Solitary Bone Plasmacytoma: A Case Report and Review of the Literature

Introduction: Solitary bone plasmacytoma and extramedullary plasmacytoma are the proliferation of plasma cells in bone or soft tissues, without bone marrow involvement or other systemic presentation of multiple myeloma. A separate category of plasmacytomas is identified that “occur as multiple sites of disease in soft tissue, bone, or both soft tissue and bone”. Multiple extramedullary plasmacytoma or multiple solitary plasmacytoma is defined as when there is more than one extramedullary tumor of plasma cells or multiple sites of disease in bone. Case Presentation: We report such rare case of multiple extramedullary plasmacytoma involving skin and soft tissues of abdomen wall, pelvic, chest, and solitary plasmacytoma bone in rib 6 chest and in bone of sacrum also diagnostic work up, treatment and care to the patients we have done. Conclusions: Prognosis is very poor, and if it is appropriate to stem cell, transplantation is considered to prevent recurrence.

PF650 AGE DOES NOT CONFER DIFFERENT FEATURES AND OUTCOMES IN PATIENTS WITH NEWLY DIAGNOSED ACTIVE MULTIPLE MYELOMA YOUNGER THAN 66 YEARS OF AGE TREATED IN A RECENT PERIOD

Background:Multiple myeloma (MM) is a disease of the elderly, with median age at diagnosis of 66 years. However, 10% of patients are younger than 50 years. Publications regarding age as prognostic factor for clinical characteristics at diagnosis and outcomes are heterogeneous, with some authors reporting aggressive presentation and worse outcomes in young patients and others showing better outcomes.Aims:To describe clinical and laboratory characteristics, response to treatment and survival in active newly‐diagnosed MM (NDMM) under 66 years. To compare outcomes between &lt;51 and 51–65 years of age.Methods:Retrospective review of medical records of active MM diagnosed from jan2010 to dec2018 at Hospital de Clínicas, Montevideo, Uruguay. Smoldering MM and plasma cell leukemia were excluded. Characteristics at diagnosis were analyzed using descriptive statistics. Survival outcomes were calculated by Kaplan‐Meier method and compared using log‐rank test. In all cases, p &lt; 0,05 was considered significant.Results:131 patients &lt;66 years were included, 36 (27.5%) &lt;51 years. Characteristics at diagnosis are shown in Table 1. Most patients were diagnosed at an advanced Durie‐Salmon and prognostic score (ISS) stages, showing no differences according to age. The mean monoclonal protein was lower in younger patients (p = 0,048). CRAB features and bone marrow infiltration were comparable. Bone plasmacytomas and extramedullary infiltration were more frequent in younger patients. Most used induction regimens were CyBorD and CTD in both groups. Rates of upfront autologous stem cell transplant (ASCT) consolidation were similar. Overall response rate (&gt; = PR [partial remission]) was 77,4% in &lt;51 and 79,9% in 51–65 years (p = 0,590) with comparable depth of response. Median progression free survival (PFS) was 25 months in &lt;51 years and 26 months in 51–65 years (p = 0,683). Mortality rate was 33.3% in &lt;51 years and 31.5% in older. The main causes of death were progression (53,2%) and infections 14,9%, similar in both groups. With a median follow up of 34 months, overall survival (OS) was 68,1%, median not reached in patients &lt;51 years and 75 months in patients 51 – 65 years (p = 0,528).Summary/Conclusion:Clinical and laboratory features are similar in NDMM patients &lt;50 and 51–65 years, although bone plasmacytoma and extramedullary disease were more frequent in the younger. However, this finding did not translate into differences in response or survival. Despite its efficacy and availability, upfront ASCT rate is low.imageDisclosures: no conflicts of interest to declare.Funding: none.

PB2119 CLINICAL PRESENTATION AND OUTCOMES OF SOLITARY PLASMACYTOMA IN A LARGE TERTIARY HOSPITAL: A RETROSPECTIVE COHORT STUDY

Background:Solitary plasmacytoma is a rare neoplasm of monoclonal plasma cells presenting as either Solitary Bone Plasmacytoma (SBP) or Solitary Extramedullary Plasmacytoma (SEP), without evidence of systemic disease. Standard treatment typically involves radical radiotherapy resulting in prolonged remissions in the majority of cases. However, previous studies have identified a high‐risk group of patients with an increased risk of progression to multiple myeloma. Due to the rarity of this plasma cell dyscrasia, there are few published clinical trials.Aims:To identify risk factors for progression to myeloma, evaluate survival outcomes and determine the incidence of secondary malignancies in a cohort of solitary plasmacytoma patients.MethodsWe retrospectively reviewed case records of adult patients with plasmacytoma treated in a 15‐year period (2002‐2017) at Addenbrookes Hospital, the regional tertiary hospital for the Eastern region of England. Patients with multiple plasmacytomas or meeting the criteria for multiple myeloma at the time of diagnosis were excluded. Clinical, laboratory and radiological features at diagnosis and during the course of treatment were analyzed. Time to progression to myeloma were recorded and survival outcomes analyzed using the Kaplan‐Meier method. The data cut off date was the 1st of July 2018.Results:Thirty‐three (33) cases of solitary plasmacytoma were identified (Table 1): 26 (79%) SBP and 7 (21%) SEP. The mean age at diagnosis was 63 years with a 2:1 male preponderance. The thoracic vertebrae were the commonest sites for SBP while SEP occurred most frequently in skin and soft tissue. Pain and spinal cord compression were the most frequent presenting symptoms. A paraprotein was detectable in 19 (58%) patients at the time of diagnosis, predominantly IgG (68%). The majority of patients received radiotherapy alone (79%) while two patients received wide local excision as the sole modality of treatment. The median total dose of radiation received was 50 gray in 25 fractions (range: 40Gy‐65 Gy). Over a median follow up of 48 months, 13 patients (39%) progressed to multiple myeloma. Median time of progression to myeloma was 22 months but notably, two patients progressed more than 5 years after radiotherapy. There was a trend towards progression for patients with non‐IgG paraprotein and paraproteinemia &gt;10 g/l (relative risk 2.2, 95% CI 0.8‐5.8). From pooled analysis of overall survival, 5‐and 10‐year survival rates were 80% and 57% respectively (Figure 1). The median progression‐free survival was 85 months (Figure 2). Following progression to myeloma, the median survival was 48 months. Over the course of follow up, 5 patients (15%) developed a second malignancy with lung cancer being the most frequent.Summary/Conclusion:Solitary plasmacytoma generally has a good prognosis but progression to multiple myeloma predicts inferior survival. Previous studies have suggested a number of predictive factors for progression but these have not been validated in large prospective studies. In this retrospective cohort, the presence of a significant (&gt;10 g/l) paraprotein and non‐IgG type appeared to increase the likelihood of progression to myeloma but this observation was limited by the relatively small sample size. In the era of novel therapies for myeloma, there is a need for clinical trials investigating adjuvant therapies for high risk solitary plasmacytoma. We also suggest long‐term monitoring of solitary plasmacytoma patients due to the finding of late progression (&gt;5 years) and the incidence of secondary cancers in this study.image

Solitary plasmacytoma of bone over sacrum: a case report

Solitary plasmacytoma of bone (SPB), also called osseous plasmacytoma, is more common in males than in females with an estimated ratio of 2:1. The median age of SPB patients is approximately 55 years. Localized radiation therapy is the first-line treatment to avoid progression to multiple myeloma. A 46-year-old man visited our hospital due to lower back pain radiating to his lower limbs for 3 months. L-spine MRI showed S2 to S4 bone erosion with a large (8.8×6.2 centimeter) tumor over sacrum. Following partial tumor removal, pathology showed plasmacytoma. Adjuvant radiotherapy with 50 Gy in 25 fractions was administered to the gross tumor based on volumetric modulated arc therapy (VMAT). No adjuvant chemotherapy was given. Pelvic CT was arranged every 3 months during 24-month follow-up. There was no distant metastasis or newly diagnosed multiple myeloma during the follow-up period.

Surgical Approach of Cauda Equina Syndrome Secondary to L5 Lumbar Solitary Bone Plasmocytoma with Partial Resection and Lumbopelvic Fixation

AbstractThe solitary bone plasmacytoma is an uncommon cause of chronic lumbar pain. It belongs to a tumor group of plasmatic cells that usually disturbs the axial skeleton and can progress to multiple myeloma. The treatment of choice is radiotherapy; however, surgical management can also be done. Regardless of the no reliable data and little evidence, it is clear that the intervention is based on the classification systems of Enneking and Weinstein–Boriani–Biagini. This article will show a 66-year-old female patient with lumbar pain of 1 month with B symptoms. A solitary bone plasmacytoma diagnosis was made with pathology confirmation. A surgical procedure with improvement of symptoms was achieved, with 2 years of follow-up without relapse.

Solitary Plasmacytoma: a Review of Diagnosis and Management

Solitary plasmacytoma is a rare plasma cell dyscrasia, classified as solitary bone plasmacytoma or solitary extramedullary plasmacytoma. These entities are diagnosed by demonstrating infiltration of a monoclonal plasma cell population in a single bone lesion or presence of plasma cells involving a soft tissue mass, respectively. Both diseases represent a single localized process without significant plasma cell infiltration into the bone marrow or evidence of end organ damage. Clinically, it is important to classify plasmacytoma as having completely undetectable bone marrow involvement versus minimal marrow involvement. Here, we discuss the diagnosis, management, and prognosis of solitary plasmacytoma. There have been numerous therapeutic advances in the treatment of multiple myeloma over the last few years. While the treatment paradigm for solitary plasmacytoma has not changed significantly over the years, progress has been made with regard to diagnostic tools available that can risk stratify disease, offer prognostic value, and discern solitary plasmacytoma from quiescent or asymptomatic myeloma at the time of diagnosis. Despite various studies investigating the use of systemic therapy or combined modality therapy for the treatment of plasmacytoma, radiation therapy remains the mainstay of therapy. Much of the recent advancement in the management of solitary plasmacytoma has been through the development of improved diagnostic techniques.

Solitary Plasmacytoma of Bone of the Spine: Results From Surveillance, Epidemiology, and End Results (SEER) Registry.

Retrospective analysis. To determine the prognostic indicators in patients with solitary plasmacytoma of bone (SPB) of the spine. Population-level estimates for prognosis among patients with SPB of the spine are still lacking. Sociodemographic and clinical predictors of outcome have not been well characterized. The Surveillance, Epidemiology, and End Results Registry was used to identify all patients with SPB of the spine from 1995 through 2014. Associated population data were used to determine annual incidence and limited-duration prevalence. Overall survival (OS) estimates were obtained using the Kaplan-Meier method and compared across groups using log-rank test. A Cox regression model was used for multivariate analysis of survival. Logistic regression was performed to identify predictors of the progression to multiple myeloma (MM). The incidence and prevalence of the disease increased during the study period. Spinal SPB most commonly affected older people (>50) with a male preponderance. The median OS were 74.0 months. The 5 and 10-year survival rates for these patients were 56.1% and 36.7%, respectively. On multivariable analyses, older age, and surgery without radiotherapy were correlated with poor survival of patients with spinal SPB. The 3-year probability of progression to MM was 10.1%. Patients aged >70 years were associated with progression to MM. There was no significant association between the methods of surgical resection (radical or local/partial) and OS or progression to MM. The findings of this study provide population-based estimates of the incidence, prevalence and prognosis for patients with SPB of the spine. This analysis indicated that the only identifiable prognostic indicators were older age and surgery without radiotherapy. Moreover, the methods of surgical resection did not influence the OS or progression to MM. 4.

Expression of Adhesion Molecule CD56 in Tumor Plasma Cells in Bone Marrow as a Prognostic Factor in Multiple Myeloma

Aim. To study immunohistochemical parameters of tumor plasma cells in bone marrow and to assess how the expression of adhesion molecule CD56 impacts overall survival (OS) of multiple myeloma (MM) patients. Materials &amp; Methods. The trial included 35 patients (19 men and 16 women) aged 23 to 73 years (with median age of 58 years) with newly diagnosed MM. At disease onset plasmacytoma was diagnosed in 21 patients. In all patients bone marrow core biopsy was performed followed by histologic and immunohistochemical (IHC) examinations. IHC examination was based on the panel of CD56, CD166, CXCR4, Ki-67, and c-MYC/CD138 antibodies. Kaplan-Meier survival curves and significance assessment by means of Cox's F-Test were used. Results. Expression mean values of most of studied markers (CD56, CXCR4, c-MYC, and Ki-67) in bone marrow of patients without plasmacytoma (n = 14) appeared to be higher than in patients with plasmacytoma at MM onset. Expression mean value is understood as percentage ratio of plasma cells expressing a studied marker to total cell count of tumor substrate. High expression of chemo-kine receptors (CXCR4), and adhesion molecules (CD56) probably inhibits plasma cell migration and impedes ex-tramedullary tumor progression. Comparison of protein expression by tumor plasma cells in bone marrow in the groups with bone extramedullary plasmacytoma shows a distinct regularity referring to CD56 adhesion molecule. For example, CD56 expression is significantly (р &lt; 0.05) lower in terms of the count of tumor plasma cells with marker expression in bone marrow of MM patients with extramedullary plasmacytoma compared with patients with bone plasmacytoma (1 ± 1 % vs. 65.71 ± 12.12 %). Comparison of MM patients' OS depending on CD56 expression by tumor plasma cells in bone marrow showed that 4-year OS of patients with CD56 expression in bone marrow was significantly higher being 80 % vs. 38 % in the group with CD56 expression less than in 10 % of tumor cells. Conclusion. Expression of adhesion molecule CD56 in tumor plasma cells in bone marrow can be regarded as a prognostic factor in MM. Probably, when at disease onset CD56 expression is identified in less than 10 % of tumor cells in bone marrow, more detailed additional examination of patients should be carried out to rule out extramedullary lesions in different organs and tissues.