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Shaji Kumar - One of the best experts on this subject based on the ideXlab platform.
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clinical features and outcomes of Plasmacytoma in the united states analysis using the national cancer data base
Blood, 2016Co-Authors: Gaurav Goyal, Wilson I Gonsalves, Ronald S Go, Shaji KumarAbstract:Introduction Plasmacytomas comprise approximately 3% of all plasma cell malignancies. There is a paucity of large studies assessing clinical features and outcomes of this relatively uncommon disease. Our objective was to describe the patterns of clinical presentation and survival of Plasmacytomas using the National Cancer Data Base (NCDB). Methods This is a retrospective study of patients with histologically confirmed diagnosis of Plasmacytoma from 2000-2011 using International Classification of Diseases for Oncology version 3 (ICD-0-3) code: 9930. Patients who had bone marrow involvement were excluded from the analysis. Plasmacytomas were grouped into two broad categories: extramedullary Plasmacytoma (P-EM) and bone Plasmacytoma (P-bone) based on their anatomical locations. Overall survival was analyzed using Kaplan-Meier estimates. Results A total of 6,939 patients were included in the study (median age 64 years; range 18-90 years). Approximately 62% of these patients were males and 46% patients were ≥ 70 years. Racial/ethnic distribution of the disease was as follows: 76% Whites, 13% Blacks, 7% Hispanics, and 4% others. The anatomical distribution and survival of patients is depicted in the Table. P-EM comprised 30% of the patients, with remaining 70% presenting as P-bone. Among P-EM, most common sites of presentation were upper aero-digestive tract (41%) and connective/soft tissue (20%). After a median follow up of 65 months, the overall survival of P-EM was significantly better than P-bone (113 vs. 78 months; Figure). Based on outcomes, we can categorize the P-EM patients into 3 groups: good (median overall survival > 120 months: upper aero-digestive tract, lymph nodes, endocrine and digestive system), intermediate (median overall survival 90-120 months: nervous system, eye/orbit, pulmonary), and poor (median overall survival Conclusions This is the largest registry-based study on Plasmacytoma in the United States. Plasmacytomas have varied clinical presentation, along with significantly different survival based on sites of presentation, race, sex, and treatment facility. Our study results point toward important differences in disease biology based on location and may aid in assessing prognosis for planning treatment. Table. Anatomical distribution and median overall survival of Plasmacytomas. Disclosures Kumar: Noxxon Pharma: Consultancy, Research Funding; Kesios: Consultancy; Skyline: Honoraria, Membership on an entity9s Board of Directors or advisory committees; Onyx: Consultancy, Research Funding; BMS: Consultancy; Array BioPharma: Consultancy, Research Funding; Sanofi: Consultancy, Research Funding; Celgene: Consultancy, Research Funding; Millennium: Consultancy, Research Funding; Glycomimetics: Consultancy; AbbVie: Research Funding; Janssen: Consultancy, Research Funding.
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solitary Plasmacytoma is radiation therapy sufficient
American Journal of Hematology, 2008Co-Authors: Shaji KumarAbstract:Solitary Plasmacytomas are characterized by a localized collection of malignant plasma cells without evidence of a systemic plasma cell proliferative disorder. It accounts for 5–10% of all plasma cell neoplasms and may present with a single bone lesion [solitary bone Plasmacytoma (SBP)] or as a single extramedullary or extraosseous lesion [1–3]. In spite of local radiation with curative intent, over half of these patients eventually develop multiple myeloma (MM) [2,4–6]. Patients with SBPs tend to be a decade younger compared with those with myeloma and often present with pain or soft tissue swelling related to extramedullary extension [6]. SBPs can result in pathological fractures, which can occasionally lead to significant neurological symptoms when they affect the vertebral bodies. The axial skeleton is more often involved than appendicular skeleton and the appendicular lesions when present usually involves the proximal bones. As suggested by the terminology, diagnosis of a solitary Plasmacytoma requires a complete work up to rule out the presence of a systemic plasma cell disorder [7]. In addition to pathological confirmation of the Plasmacytoma, complete skeletal surveys should be done to rule out other bone lesions, and bone marrow should be free of any clonal plasma cells. It is not unusual for the serum or urine to demonstrate presence of monoclonal protein, but these often tend to be small. More recently, additional imaging studies such as MRI of the spine or PET scans are increasingly being utilized in the work up of these patients. Clearly these techniques increase the ability to pick up the presence of additional lesions and exclude the diagnosis of a solitary Plasmacytoma, but their role as part of routine evaluation of these patients require further study. Given that over half of patients with SBPs eventually develop MM, there has been significant interest in identifying patients at increased risk of progression. Multiple risk factors have been identified for increased risk and include persistence of monoclonal protein after radiation therapy, large lesions, involvement of axial skeleton, older patients, suppression of uninvolved immunoglobulin levels, abnormal immunoglobulin free light chain ratio, and presence of increased neovascularization in the tumor. In contrast, extramedullary Plasmacytomas arising outside of the bone marrow tend to occur most commonly in the head and neck region, especially in the upper airways and upper gastrointestinal tract. However, these lesions can involve nearly any part of the body, and the symptoms typically are related to the pressure effect on the adjacent tissue, or blockage of the respiratory of digestive tract. As with SBPs, diagnosis hinges on exclusion of a systemic plasma cell proliferative disorder and presence of monoclonal protein in the serum or urine does not exclude the diagnosis. It is critical to distinguish these lesions from lymphomas with plasmacytic differentiation of the tumor cells as well as poorly differentiated carcinomas. In contrast to bone Plasmacytomas the risk of development of MM is only 10–15%, but risk of local recurrence is higher than with bone lesions. The management of solitary Plasmacytomas has traditionally included local radiation therapy (RT) with surgical intervention required only in the event of pathological fractures involving bone lesions or when surgical resection of a soft tissue mass led to the initial diagnosis of a Plasmacytoma. In this issue of the journal, Kilciksiz et al. have reviewed the collective experience from Turkish Oncology Group-Sarcoma Working Party and have once again raised questions on the role of surgery in addition to radiation in these patients[8]. The study included 80 patients with solitary Plasmacytomas, 57 of who had SBPs, from several centers in Turkey. In contrast to some of the earlier series, this study has the advantage of reasonably long follow-up. The demographic and clinical features of the patients were as expected and as reported in the previous series, the risk of progression was higher among those with bone Plasmacytomas. Older patients with bone Plasmacytomas are more likely to have progression to myeloma and this study again confirms this observation. In their analysis, the authors raise the question whether the dose of radiation and the use of surgical resection in addition to radiation impact the risk of progression to myeloma. In the univariate analysis of risk factors for progression to myeloma, surgery in addition to RT and presence of macroscopic tumor prior to RT, both influenced the risk. Although the results suggest that surgical intervention and complete resection of the tumor may have added benefit over and above RT, this needs to be interpreted with caution. Clearly patients with extramedullary Plasmacytomas are more likely to have undergone surgical resection given that the diagnosis is often made after resection of a suspicious mass, as reflected by the smaller proportion of these patients (43.5% vs. 82.5%) with macroscopic tumors prior to RT. The prognostic value of this finding is likely a reflection of the higher proportion of patients with extramedullary lesion without macroscopic tumor prior to RT, a group with inherently lower risk of progression to myeloma and this is further borne out by the lack of prognostic value in the multivariate analysis. Similarly, the effect of surgery on the risk of recurrence may be a reflection of other disease characteristics rather than a direct beneficial effect of surgery. Patients with bone and extramedullary Plasmacytomas often undergo surgical procedures for different reasons, symptomatic bony lesions or fractures that need surgical intervention in the former versus resection of a suspicious mass for diagnosis or palliation in the latter. It is often difficult to determine the rational behind the surgical procedure in a retrospective study. It is
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prognostic value of angiogenesis in solitary bone Plasmacytoma
Blood, 2003Co-Authors: Shaji Kumar, Rafael Fonseca, Angela Dispenzieri, Martha Q Lacy, John A Lust, Linda Wellik, Thomas E Witzig, Morie A Gertz, Robert A Kyle, Philip R GreippAbstract:Angiogenesis plays an important role in the biology of multiple myeloma (MM) and has prognostic importance in this disease. Solitary Plasmacytoma is a localized plasma cell malignancy that progresses to MM in a significant number of patients. We examined if angiogenesis is increased in solitary Plasmacytoma and if it can help identify patients likely to progress to myeloma. We studied angiogenesis in Plasmacytoma biopsy samples and bone marrow biopsies from 25 patients. High-grade angiogenesis was present in 64% of Plasmacytomas. In contrast, bone marrow angiogenesis was low in all patients. Patients with high-grade angiogenesis in the Plasmacytoma sample were more likely to progress to myeloma and had a shorter progression-free survival compared with patients with low-grade angiogenesis (P = .02). Angiogenesis is increased in solitary Plasmacytoma and is a significant predictor of progression to myeloma and provides further evidence of its importance in the pathogenesis of myeloma.
Joo Seop Chung - One of the best experts on this subject based on the ideXlab platform.
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comparison of outcomes after autologous stem cell transplantation between myeloma patients with skeletal and soft tissue Plasmacytoma
European Journal of Haematology, 2014Co-Authors: Hojin Shin, Mookon Song, Joo Seop ChungAbstract:: 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.
Nancy P Mendenhall - One of the best experts on this subject based on the ideXlab platform.
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solitary Plasmacytoma of bone and soft tissues
American Journal of Otolaryngology, 2003Co-Authors: William M Mendenhall, Charles M Mendenhall, Nancy P MendenhallAbstract:Abstract Purpose To define the optimal treatment and outcomes for patients with solitary Plasmacytoma of bone and soft tissue. Methods Review of the literature. Results Solitary Plasmacytomas are uncommon and account for less than 5% of plasma cell neoplasms. Solitary Plasmacytomas of bone (SPB) usually occur in the vertebra and skull and are more common than extramedullary Plasmacytomas (EMP) that almost always arise in the head and neck and may spread to regional lymph nodes. The optimal treatment is moderate-dose radiotherapy (40–50 Gy) and occasionally surgery. Adjuvant chemotherapy does not improve survival. Patients with EMP have a relatively low risk of progressing to multiple myeloma and have improved survival compared with those who present with SPB. Conclusion Solitary Plasmacytoma is an uncommon neoplasm that often arises in the head and neck. Optimal treatment is moderate-dose radiotherapy. Prognosis is relatively good and is better for patients with EMP compared with those presenting with SPB.
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solitary Plasmacytoma of bone and soft tissue
International Journal of Radiation Oncology Biology Physics, 1996Co-Authors: Timothy W Bolek, Robert B Marcus, Nancy P MendenhallAbstract:Abstract Between 1962 and 1978, 15 patients presenting with a solitary Plasmacytoma were treated with curative intent by radiotherapy alone at the University of Florida. Criteria for admission to this study were: 1) a biopsy-proven, apparently solitary focus of Plasmacytoma; 2) bone marrow biopsy showing less than 10% plasma cells; and 3) no evidence of disseminated disease. In 9 patients the primary site was osseous and in 6 patients extramedullary; 5 of the 6 extramedullary lesions were located in the upper respiratory passages. Two of the 6 extramedullary Plasmacytomas progressed to multiple myeloma at 2 and 7 months. The remaining 4 patients have been disease free for periods ranging from 2 2 3 years to over 16 years. Of the 9 patients with osseous lesions, 3 developed multiple myeloma in 3–23 months and one developed a solitary second bone lesion at 9 years. One patient with a large sacral lesion developed a local recurrence following an initial radiation dose of 3000 rad. The recurrence was re-treated with radiation, and local control was obtained. No other local failures occurred. This study presents a detailed analysis of the time-dose relationship required for local control, based on a study of our own patients and a review of the literature.
Hojin Shin - One of the best experts on this subject based on the ideXlab platform.
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comparison of outcomes after autologous stem cell transplantation between myeloma patients with skeletal and soft tissue Plasmacytoma
European Journal of Haematology, 2014Co-Authors: Hojin Shin, Mookon Song, Joo Seop ChungAbstract:: 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.
Melek Ergin - One of the best experts on this subject based on the ideXlab platform.
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a case of extramedullary Plasmacytoma in the sphenoid sinus with unilateral loss of vision
Journal of Cranio-maxillofacial Surgery, 2013Co-Authors: Suleyman Ozdemir, Ozgur Tarkan, Ulku Tuncer, Ozgur Surmelioglu, Murat Dogrusoz, Melek ErginAbstract:Abstract Extramedullary Plasmacytomas are localized tumours formed of monoclonal plasma cells in an extra-skeletal area. They constitute approximately 3% of all neoplasms originating from plasma cells. They generally display a destructive course. When the literature in English was reviewed, only 19 cases with the primary disease localized in the sphenoid sinus were found. We present the case of a 50-year-old male patient who presented with gradually increasing visual loss over 6 weeks, whose radiological tests revealed a formation of mass in the sphenoid sinus pressing against the optic nerve and internal carotid artery. A biopsy obtained by endoscopic sinus surgery was reported to be a Plasmacytoma. A diagnosis of extramedullary Plasmacytoma was made after investigations for other neoplastic plasma cell conditions proved negative. Extramedullary Plasmacytomas were assessed by reviewing the literature.