scholarly journals Successful Treatment for Solitary Bone Plasmacytoma of the Maxilla: A Rare Case Report

2020 ◽  
Vol 13 (6) ◽  
Author(s):  
Arghavan Etebarian ◽  
Mohamadreza Moravej

Introduction: Plasma cell tumors are monoclonal neoplastic proliferation of plasma cells which are divided into three groups: solitary bone plasmacytoma (SBP) and extramedullary plasmacytoma (EMP) which are localized forms, and multiple myeloma (MM) which is the disseminated form. Skull, long bones, and vertebrae are common sites for SBP and its presence in maxillofacial area is rare. There is a possibility that SBP converts to MM and some authors believe that it can be the early stage of MM. Case Presentation: Here, we present a 59-year old male patient with a mass in maxillary bone enlarging after tooth extraction. Cone beam computed tomography (CBCT) confirmed the existence of a poorly defined lesion in the left maxilla. The microscopic evaluation and immunohistochemical studies confirmed the diffuse and monoclonal population of cells, indicating the diagnosis of plasma cell tumor. There was also no evidence of bone marrow involvement in the aspiration. Conclusions: Early and precise diagnosis of SBP is a crucial element to rule out the possibility of MM. It must be kept in mind that there is possibility of recurrence or progression to MM after treatment which can completely change the course of disease, and this emphasizes the importance of regular follow up.

Author(s):  
Francesca Lalla ◽  
Alessandro Vinciguerra ◽  
Alessandra Lissoni ◽  
Gianluigi Arrigoni ◽  
Francesca Lira Luce ◽  
...  

Solitary plasmacytoma (SP) is a rare malignant tumor of plasma cells with no systemic spread; however, when it disseminates and affects multiple skeletal sites, it is called multiple myeloma (MM). The etiology of solitary plasmacytoma is unknown, with two possible subtypes: solitary extramedullary plasmacytoma (EMP) and solitary bone plasmacytoma (SBP). We present a case of EMP arising as asymptomatic erythroplakia of the palate, which is rarely described in the literature. The definitive diagnosis was obtained with immunohistochemical studies, after which the lesion was subjected to excisional biopsy. At present, after two years of close follow-up, the patient has shown no signs of relapse or conversion to MM. The uniqueness of the case highlights the possibility of an atypical EMP lesion in the head and neck, thus posing a diagnostic and therapeutic challenge for physicians.


2011 ◽  
Vol 2011 ◽  
pp. 1-2 ◽  
Author(s):  
Kevin C. Huoh ◽  
Annemieke Van Zante ◽  
David W. Eisele

Plasma cell tumors are a diverse group of neoplasms characterized by monoclonal proliferation of plasma cells. Extramedullary plasmacytoma (EMP) is a rare form of localized plasma cell tumor that arises most often in the head and neck region. We present an unusual case of EMP of the palatine tonsil from a tertiary care university hospital. We discuss the histopathologic and radiologic evaluation as well as treatment of EMP.


Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 5178-5178
Author(s):  
Patricia T. Greipp ◽  
Rafael Fonseca ◽  
Dewald Dewald ◽  
S. Vincent Rajkumar ◽  
William G. Morice ◽  
...  

Abstract Background: Multiple myeloma (MM) is a plasma cell malignancy involving complex cytogenetic dysregulation of genes and heterogeneous expression of multiple B cell and plasma cell (PC) surface antigens. 60% of MM patients have cytogenetic translocations of the IGH locus. In 16% of myeloma patients, the partner oncogene is cyclin D1 on 11q13 causing t(11;14)(q13;q32) (Fonseca et al. Blood. 2002). This translocation is more common in patients with a lymphoplasmacytic morphology and confers a more favorable prognosis (Fonseca et al. Blood. 2003). A subset of MM patients (20%) harbor a clonotypic population of CD20+ bone marrow plasma cells (BMPC). These patients have a shortened survival time (SanMiguel et al. Br J Haematol. 1991). There is a strong association with CD20+ surface expression and t(11;14)(Robillard et al. Blood. 2003) and CD20+ PC has been suggested to be clonogenic and important in the pathogenesis of MM. A small Phase II study showed only a modest response to the humanized anti-CD20 monoclonal antibody rituximab in MM patients with CD20+PC (1 PR and 5SD) (Treon et al. J. Immunother. 2002). We report a response to treatment with rituximab in a MM patient with CD20+ BMPC and t(11;14)(q13;q32) by FISH. Case: The patient is a 68 year old male with a 1.5 year history of MM. Initial diagnosis of ISS stage I disease was made after a three year history of IgA kappa monoclonal gammopathy of undetermined significance (MGUS). At diagnosis of MM, he had symptomatic anemia Hgb10.5g/dL, rising serum IgA of 3950 mg/dL, serum m-spike of 4.1 g/dL (IgA kappa), PCLI (plasma cell labeling index) of 1%, and a small monoclonal IgA kappa with kappa fragment in the urine. BM showed 44% monoclonal kappa plasma cells with 90% CD20+,clonal PC on flow cytometry. Karyotype was normal; 46, XY, but FISH showed evidence of an IGH translocation t(11;14)(q13;q32) in 60% of PC. Immunohistochemical staining was positive for cyclin D1. Given the marked elevation of CD20+ PC and early stage of disease, initial treatment was employed with four weekly cycles of rituximab (375mg/m2). IgA fell from 4750 mg/dL to a nadir of 2990 mg/dL, (m-spike; 4.5g/dL to 3.2 g/dL) five weeks after completion of treatment. The monoclonal protein (MP) in the urine disappeared. Hgb improved to 12.5g, with erythropoietin. Three months later, IgA rose to 3260mg/dL (m-spike 3.2 g/dL) and urinary MP returned. The patient remained asymptomatic. A second course of weekly rituximab was started. IgA level and serum m-spike remained fairly stable over the next 7 months. All treatment was tolerated well except for initial transient rigors, fever, and nausea. Despite bi-monthly maintenance rituximab, the patient devloped a rising IgA of 4940 mg/dL, m-spike of 4.7, falling Hgb and a rising PCLI of 1.5%, 15 months after his original diagnosis of MM. Of particular concern is the development of new, unfavorable, cytogenetic aberrations by FISH with BMPC now showing deletion 13 in 93% and mutation of p53 (17p13) in 98%.100% show fusion of CCND1 and IGH and PC now only partially express CD20. Conclusion: This report suggests that MM patients with CD20+ BMPC and t(11;14)(q13;q32) may represent a target population for anti-CD20 therapy. Our report supports the concept proposed that targeting this clonotypic subset of B cells possibly interrupts a critical oncogenic pathway that is important in the pathogenesis of the clonogenic development of MM.


2013 ◽  
Vol 2013 ◽  
pp. 1-5 ◽  
Author(s):  
Zhongchuan W. Chen ◽  
Ioanna Kotsikogianni ◽  
Jay S. Raval ◽  
Christine G. Roth ◽  
Marian A. Rollins-Raval

Biclonal plasma cell myelomas producing two different isotypes of immunoglobulins are extremely rare entities; to date, the combination of IgD and IgM secretion by a biclonal plasma cell myeloma has not been reported. Bone marrow biopsy immunohistochemical studies in two cases revealed neoplastic plasma cells coexpressing IgD and IgM, but serum protein electrophoresis identified only the IgM monoclonal paraprotein in both cases. Biclonal plasma cell myelomas, while currently not well characterized in terms of their clinical behavior, should be distinguished from B-cell lymphoma with plasmacytic differentiation, given the different therapeutic implications. Both cases reported herein demonstrated chemotherapy-resistant clinical courses.


2021 ◽  
Vol 9 (1) ◽  
pp. 01-06
Author(s):  
Manuela B. Pucca ◽  
Pablo A. Brito-Souza ◽  
Gabriel M. Alexandre-Silva ◽  
Ana C. S. Oliveira ◽  
Kim Tavares Mesquita ◽  
...  

The Solitary Bone Plasmacytoma (SBP) is a rare oncologic disease corresponding to less than 5% of the malignant neoplasms of the plasma cells. It is characterized as a localized aggressive tumor consequent to the accumulation of monoclonal plasma cell neoplasms and, due to the rarity of the disease, there are only few clinical studies reporting it especially regarding prognostic factors and treatment. Here, we report a fatal clinical case of SPB in Roraima, the northernmost state of Brazil. The study shows a case of solitary bone plasmacytoma on the femur, where the patient was submitted to the preconized radiotherapy cycles. However, over approximately four-years following the diagnose, the patient required multi-modal approach to guarantee quality of life during the survival time. Finally, this study explores SBP-related issues and examines the challenges physicians face when managing the care of patients with SBP.


Author(s):  
Leart Berdica ◽  
◽  
Teona Bushati ◽  
Alfred Aga ◽  
Emirjona Vajushi ◽  
...  

Background: Tongue extramedullary plasmacytoma is a very rare pathology. Despite rare cases, extramedullary plasmacytoma should be considered as a differential diagnosis in case of a mass in the tongue. A total of 19 cases were reported with EMP in English literature along with the case we will address. It is characterized by a monoclonal neoplastic proliferation of plasma cells in the absence of multiple myeloma (MM). Histopathology and immunohistochemistry are very important for the diagnosis and differential diagnosis. Case presentation: The case we will describe is an 80-year-old lady from Albania who presents with a vegetative lesion in the form of a thick plate on the dorsal part of the tongue with dimensions 6 X 5 X 1.5 cm. A material of 0.5 cm diameter was taken from the lesion for the biopsy. After histopathological examination, immunohistochemical examinations, and after correlations with laboratory, clinical and imaging data, the diagnosis of extramedullary plasmacytoma of the tongue was reached. The patient underwent radiotherapy treatment. Conclusions: EMP is a rare tumor, accounting for 3% of plasma cell neoplasms and <1% of all head and neck tumors. The diagnosis of EMP, in this case, was reached with biopsy, immunohistochemistry, and the correlation with laboratory and imaging data. We will show the importance of biopsy along with immunohistochemistry in the diagnosis and differential diagnosis of extramedullary plasmocytoma of the tongue. Keywords: plasmacytoma; immunohistochemistry; biopsy; plasma cell. Abbreviations: EMP: Extramedullary plasmacytoma; MM: Multiple myeloma; Cm: centimeter


Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 5050-5050
Author(s):  
Maged Khalil ◽  
Candice Ruby ◽  
Zili He ◽  
Shetra Sivamurthy ◽  
Steier Williams ◽  
...  

Abstract Plasma cell tumors are lymphoid neoplastic proliferations of B cells that may be classified as multiple myeloma (MM), solitary bone plasmacytoma (SBP) and extramedullary plasmacytoma (EMP). The extramedullary plasmacytoma account for 1–2% of the total number of plasma-cell growths of which 80 % are originated on the head and neck and upper airways. Males are more frequently affected at sixth-seventh decade. Herein we are presenting a case of 51 years old male with synchronous multiple extramedullary plasmacytomas involving lung, stomach and spine, Presentation of a case 51 years old black male from St. Lucia with no significant past medical history, presented to the local hospital in St. Lucia with hematemesis. Endoscopy was performed and a growth in the stomach was found. He came to the US for treatment. When seen in our hospital, patient complained of black tarry stool, severe right sided chest pain radiating to the back, generalized body aches, fatigue and 10 Lbs. weight loss within the last 2 months Physical examination: revealed tenderness on the right side of chest and back, and decreased breath sound on the righ upper lobe, otherwise unremarkable Work up including CT scan of the chest/abdomen /pelvis showed an irregular right apical mass posteriorly with destruction of the adjacent second and third ribs posteriorly and in T2 and T3 vertebrae, diffuse lytic lesions involving the spines, sacrum, ribs and sternum. There was also a large irregular soft tissue mass the posterior aspect of the fundus of the stomach. Liver, spleen and lymph nodes were normal. Laboratory studies showed WBCs 9.8, Hg 6.9, Platlets 218, BUN 71, Cr. 5.2, Ca 13.4, albumin 3.4, B2 microglobulin 7.5, TP 11.4, LDH 1063, LFT’s all normal, Cea 00 ng/ml, AFP 6.0 ng/ml, Ca19-9 9.4 U/ml, PSA.97 ng/ml, iron study, folate, B12 all within normal range, serum protein electropheresis and immunofixation showed monoclonal spike in the Gamma region 53.8% (IgG Kappa and IgA Kappa), IgG 10917 mg/dl, IgA 85 mg/dl, IgM 16 mg/dl, urine protien elctrophersis showed 88 mg/dl M-spike in beta region, 24 hours urine was 2400 mg/24 h Bone marrow biopsy showed extensive infiltration with poorly differentiated plasma cells, flow cytometry consistent with plasma cell neoplasm, cytogenetics and FISH did not show any evidence of chromosome 13 deletion or trisomy 11. Gastric mass biopsy and lung mass biopsy showed plasma cells similar to the bone marrow infiltrate consistent with plasmacytoma. Diagnosis of multiple myeloma and multiple extramedullary plasmacytomas were made. Plasmaphersis was started because of worsening renal function despite aggressive hydration. Kidney function and calcium level normalized after 5 sessions of Plasmaphersis. Chemotherapy with Doxil, Vincrestine and dexamethasone (DVd) was started. Because of the persistent drop in hemoglobin from gastric mass bleeding, Radiation therapy to the gastric area was given (2300 cGy in 4 weeks) While on treatment he developed severe bilateral lower extremities weakness, MRI showed 8 cm epidural mass at the T8 level, the field of radiation was increased to include the new lesion along with Decadron. He developed severe oral mucositis, esophagitis pancytopenia, continue to bleed from the gastric mass, and finally developed an overwhelming VRE sepsis and shock. He was transferred to MICU and expired despite aggressive supportive care. Conclusion: MM can present as multiple extramedullary plasmacytomas. The response to chemotherapy is very poor The prognosis is very poor,


Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 4808-4808
Author(s):  
Shuang Geng ◽  
Jing Wang ◽  
Mingyi Chen ◽  
Wenming Wang ◽  
Yuhong Pang ◽  
...  

Abstract Extramedullary Plasmacytoma (EMP) is a minor yet devastating metastatic form of Multiple Myeloma (MM), shortening patients' survival from 10 years to 6 months on average. Genetic cause of EMP in MM is yet to be defined. Transcriptome difference between EMP+ patients and EMP- patients is studied here on single cell level by RNA Sequencing (RNA-Seq). We sorted CD38+CD138+ malignant plasma cells from bone marrow and peripheral blood samples by flow cytometry, then picked up single malignant plasma cell and performed single cell RNA-Seq with SmartSeq2 protocol followed by Tn5-based library preparation from bone marrow, peripheral blood and extramedullary tissue of EMP patients. From the single cell RNA-Seq results, in bone marrow we found differential gene expression between EMP+ and EMP- samples, such as CTAG2, STMN1 and RRM2. By comparing circulating malignant plasma cells in PBMC and malignant plasma cell from the sample EMP+ patient, we observed metastatic clone in blood with the same VDJ immunoglobulin heavy chain as in bone marrow. Several genes' expression of these metastatic cells are down-regulated than in bone marrow, such as PAGE2, GTSF1, DICER1. These genes may correlate with egress capability of MM cells into peripheral to become circulating plasma cells (cPCs), and EMP eventually. Disclosures No relevant conflicts of interest to declare.


2013 ◽  
Vol 137 (4) ◽  
pp. 503-517 ◽  
Author(s):  
Christopher L. Alley ◽  
Endi Wang ◽  
Cherie H. Dunphy ◽  
Jerald Z. Gong ◽  
Chuanyi M. Lu ◽  
...  

Context.—Plasma cell myeloma and chronic lymphocytic leukemia are both common hematologic malignancies, sharing many epidemiologic features. Concomitant detection of the 2 conditions poses special diagnostic challenges for the pathologist. Objective.—To describe the pathologic findings in cases of concomitant bone marrow involvement by myeloma and CD5+ monoclonal B cells and to outline the differential diagnostic possibilities, suggest a workup for correct diagnosis, and examine clinical outcome. Design.—Fifteen cases that met the diagnostic criteria were identified from pathology databases at 4 participating institutions. Morphologic findings were reviewed, additional immunohistochemical stains performed, and flow cytometric, cytogenetic, and relevant laboratory and clinical information was summarized. Previously published cases were searched from electronic databases and cross-references. Results.—Most patients (13 of 15) were older males. Often (11 of 15) they presented clinically with myeloma, yet had both monotypic plasma cells and B cells in the diagnostic marrow. In 4 patients, myeloma developed 24 months or later after chronic lymphocytic leukemia. In 7 patients, myeloma and CD5+ B cells showed identical immunoglobulin light-chain restriction. Primary differential diagnoses include lymphoplasmacytic lymphoma, marginal zone lymphoma, and chronic lymphocytic leukemia with plasmacytoid differentiation. CD56 and/or cyclin D1 expression by plasma cells was helpful for correct diagnosis. Most patients in our cohort and published reports were treated for plasma cell myeloma. Conclusions.—Concomitant detection of myeloma and chronic lymphocytic leukemia in the bone marrow is a rare event, which must be carefully differentiated from lymphomas with lymphoplasmacytic differentiation for correct treatment.


2017 ◽  
Vol 2017 ◽  
pp. 1-4 ◽  
Author(s):  
Georgia Mitropoulou ◽  
Adamantia Zizi-Sermpetzoglou ◽  
Hippokrates Moschouris ◽  
Athanasios Kountourogiannis ◽  
Despoina Myoteri ◽  
...  

Introduction. Plasmacytoma is an uncommon plasma cell neoplasm and its localized form is solitary plasmacytoma of the bone and solitary extramedullary plasmacytoma. Solitary plasmacytoma of the mesentery is extremely rare, reported only in a handful of cases. Case Presentation. A 47-year-old man with nonspecific abdominal complains was found to have an ill-defined mass on his mesenteric root. Laparoscopic biopsy and stepwise histopathological examination revealed a mesenteric plasmacytoma, and extensive imaging and laboratory investigations led to the diagnosis of the solitary mesenteric plasmacytoma. The patient underwent definitive radiotherapy and remains under remission one year later. Discussion. Plasma cell dyscrasias include a variant of proliferative disease, characterized by clonal expansion of bone marrow plasma cells, producing a massive quantity of monoclonal immunoglobulin called paraprotein or M-protein. Solitary extramedullary plasmacytoma accounts for only 3–5% of all plasma cell neoplasms. Meticulous adherence to the established diagnostic criteria helps the clinician to set the correct, yet very unusual and unexpected diagnosis.


Sign in / Sign up

Export Citation Format

Share Document