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International Journal of Radiation... Jul 2018To develop guidelines for the work-up and radiation therapy (RT) management of patients with plasma cell neoplasms. (Review)
Review
PURPOSE
To develop guidelines for the work-up and radiation therapy (RT) management of patients with plasma cell neoplasms.
METHODS AND MATERIALS
A literature review was conducted covering staging, work-up, and RT management of plasma cell neoplasms. Guidelines were developed through consensus by an international panel of radiation oncologists with expertise in these diseases, from the International Lymphoma Radiation Oncology Group. RT volume definitions are based on the International Commission on Radiation Units and Measurements.
RESULTS
Plasma cell neoplasms account for approximately one-fifth of mature B-cell neoplasms in the United States. The majority (∼95%) are diagnosed as multiple myeloma, in which there has been tremendous progress in systemic therapy approaches with novel drugs over the last 2 decades, resulting in improvements in disease control and survival. In contrast, a small proportion of patients with plasma cell neoplasms present with a localized plasmacytoma in the bone, or in extramedullary (extraosseous) soft tissues, and definitive RT is the standard treatment. RT provides long-term local control in the solitary bone plasmacytomas and is potentially curative in the extramedullary cases. This guideline reviews the diagnostic work-up, principles, and indications for RT, target volume definition, treatment planning, and follow-up procedures for solitary plasmacytoma. Specifically, detailed recommendations for RT volumes and dose/fractionation are provided, illustrated with specific case scenarios. The role of palliative RT in multiple myeloma is also discussed.
CONCLUSIONS
The International Lymphoma Radiation Oncology Group presents a standardized approach to the use and implementation of definitive RT in solitary plasmacytomas. The modern principles outlining the supportive role of palliative RT in multiple myeloma in an era of novel systemic therapies are also discussed.
Topics: Aged; Bone Neoplasms; Consensus; Diagnostic Imaging; Female; Humans; Male; Middle Aged; Multiple Myeloma; Neoplasm Staging; Palliative Care; Plasmacytoma; Radiation Oncology; Radiotherapy Dosage; Soft Tissue Neoplasms
PubMed: 29976492
DOI: 10.1016/j.ijrobp.2018.05.009 -
Current Hematologic Malignancy Reports Apr 2019Solitary plasmacytoma is a rare plasma cell dyscrasia, classified as solitary bone plasmacytoma or solitary extramedullary plasmacytoma. These entities are diagnosed by... (Review)
Review
PURPOSE OF REVIEW
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.
RECENT FINDINGS
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.
Topics: Bone Neoplasms; Chemoradiotherapy; Fluorodeoxyglucose F18; Humans; Magnetic Resonance Imaging; Multiple Myeloma; Myeloma Proteins; Plasma Cells; Plasmacytoma; Positron Emission Tomography Computed Tomography
PubMed: 30788667
DOI: 10.1007/s11899-019-00499-8 -
Journal of Hematology & Oncology Jan 2018Solitary plasmacytoma is an infrequent form of plasma cell dyscrasia that presents as a single mass of monoclonal plasma cells, located either extramedullary or... (Review)
Review
Solitary plasmacytoma is an infrequent form of plasma cell dyscrasia that presents as a single mass of monoclonal plasma cells, located either extramedullary or intraosseous. In some patients, a bone marrow aspiration can detect a low monoclonal plasma cell infiltration which indicates a high risk of early progression to an overt myeloma disease. Before treatment initiation, whole body positron emission tomography-computed tomography or magnetic resonance imaging should be performed to exclude the presence of additional malignant lesions. For decades, treatment has been based on high-dose radiation, but studies exploring the potential benefit of systemic therapies for high-risk patients are urgently needed. In this review, a panel of expert European hematologists updates the recommendations on the diagnosis and management of patients with solitary plasmacytoma.
Topics: Disease Management; Europe; Humans; Magnetic Resonance Imaging; Plasmacytoma; Positron Emission Tomography Computed Tomography; Prognosis; Treatment Outcome
PubMed: 29338789
DOI: 10.1186/s13045-017-0549-1 -
British Journal of Haematology Aug 2021In this review, two types of soft-tissue involvement in multiple myeloma are defined: (i) extramedullary (EMD) with haematogenous spread involving only soft tissues and... (Review)
Review
In this review, two types of soft-tissue involvement in multiple myeloma are defined: (i) extramedullary (EMD) with haematogenous spread involving only soft tissues and (ii) paraskeletal (PS) with tumour masses arising from skeletal lesions. The incidence of EMD and PS plasmacytomas at diagnosis ranges from 1·7% to 4·5% and 7% to 34·4% respectively. EMD disease is often associated with high-risk cytogenetics, resistance to therapy and worse prognosis than in PS involvement. In patients with PS involvement a proteasome inhibitor-based regimen may be the best option followed by autologous stem cell transplantation (ASCT) in transplant eligible patients. In patients with EMD disease who are not eligible for ASCT, a proteasome inhibitor-based regimen such as lenalidomide-bortezomib-dexamethasone (RVD) may be the best option, while for those eligible for high-dose therapy a myeloma/lymphoma-like regimen such as bortezomib, thalidomide and dexamethasone (VTD)-RVD/cisplatin, doxorubicin, cyclophosphamide and etoposide (PACE) followed by SCT should be considered. In both EMD and PS disease at relapse many strategies have been tried, but this remains a high-unmet need population.
Topics: Animals; Antineoplastic Combined Chemotherapy Protocols; Bortezomib; Cisplatin; Cyclophosphamide; Dexamethasone; Disease Management; Doxorubicin; Etoposide; Hematopoietic Stem Cell Transplantation; Humans; Lenalidomide; Multiple Myeloma; Plasmacytoma; Prognosis; Transplantation, Autologous
PubMed: 33724461
DOI: 10.1111/bjh.17338 -
Oral Oncology Jul 2018
Topics: Aged; Chemoradiotherapy; Female; Humans; Mouth Neoplasms; Palate; Plasmacytoma
PubMed: 29779914
DOI: 10.1016/j.oraloncology.2018.05.009 -
The Veterinary Clinics of North... Jan 2023This review provides current information on myeloma-related disorders, a group of plasma cell or immunoglobulin (Ig) secreting neoplasms including multiple myeloma,... (Review)
Review
This review provides current information on myeloma-related disorders, a group of plasma cell or immunoglobulin (Ig) secreting neoplasms including multiple myeloma, extramedullary plasmacytoma (both cutaneous and noncutaneous variants), solitary osseous plasmacytoma, Waldenström macroglobulinemia/lymphoplasmacytic lymphoma, Ig-secretory B-cell lymphoma, plasma cell leukemia, and monoclonal gammopathy of undetermined significance. The diagnostic procedures commonly used to characterize myeloma-related disorders, including cytopathology, histopathology, polymerase chain reaction for antigen receptor rearrangement, flow cytometry, and electrophoretic techniques are outlined and discussed.
Topics: Animals; Multiple Myeloma; Plasmacytoma; Paraproteinemias; Waldenstrom Macroglobulinemia
PubMed: 36270842
DOI: 10.1016/j.cvsm.2022.07.009 -
Current Hematologic Malignancy Reports Jun 2018To discuss the diagnostic approach, treatment options, and future considerations in the management of plasmacytomas, either solitary or in the context of overt multiple... (Review)
Review
PURPOSE OF REVIEW
To discuss the diagnostic approach, treatment options, and future considerations in the management of plasmacytomas, either solitary or in the context of overt multiple myeloma (MM).
RECENT FINDINGS
Advanced imaging techniques such as whole-body magnetic resonance imaging and positron emission tomography/computerized tomography are essential for the diagnostic workup of solitary plasmacytomas (SP) to rule out the presence of other disease foci. The role of flow cytometry and clonal plasma cell detection is currently under study together with other prognostic factors for the identification of patients with SP at high risk of progression to overt MM. Solitary plasmacytomas are treated effectively with local radiotherapy whereas systemic therapy is required at relapse. Clonal plasma cells that accumulate at extramedullary sites have distinct biological characteristics. Patients with MM and soft tissue involvement have poor outcomes and should be treated as ultra-high risk. A revised definition of SP that distinguishes between true solitary clonal PC accumulations and SP with minimal bone marrow involvement should be considered to guide an appropriate therapeutic and follow-up approach. Future studies should be conducted to determine optimum treatment approaches for patients with MM and paraskeletal or extramedullary disease.
Topics: Flow Cytometry; Humans; Magnetic Resonance Imaging; Multiple Myeloma; Plasma Cells; Plasmacytoma; Positron-Emission Tomography
PubMed: 29667156
DOI: 10.1007/s11899-018-0452-z -
The Neuroradiology Journal Sep 2014This pictorial review describes the spectrum of CT/MR imaging findings of solitary extramedullary and bone plasmacytomas in different locations related to... (Review)
Review
This pictorial review describes the spectrum of CT/MR imaging findings of solitary extramedullary and bone plasmacytomas in different locations related to neuroradiology. Plasmacytoma is considered a counterpart of multiple myeloma that is described as a solitary and discrete mass of monoclonal neoplastic plasma cells. It may arise from osseous (medullary) or extramedullary sites. Isolated extramedullary plasmacytoma is very rare and comprises less than 4% of all plasma cellular diseases of which more than 80% are localized to the submucosal lymphoid tissue of the nasopharynx, nasal cavity and paranasal sinuses. We will demonstrate imaging findings in ten histopathologically proven plasmacytomas in different locations related to neuroradiology. Extramedullary and osseous plasmacytoma show nonspecific CT and MR imaging findings. MR is the preferred modality for evaluation due to better soft tissue contrast. Features that may suggest the diagnosis of plasmacytoma are bulky soft tissue mass and relatively isointense signal on T2-weighted MR images due to high cellularity.
Topics: Bone Marrow Neoplasms; Humans; Magnetic Resonance Imaging; Nasopharyngeal Neoplasms; Neuroimaging; Paranasal Sinus Neoplasms; Plasmacytoma; Skull Neoplasms; Tomography, X-Ray Computed
PubMed: 25196616
DOI: 10.15274/NRJ-2014-10078 -
Medicina Clinica Sep 2021
Topics: Humans; Plasmacytoma
PubMed: 32843223
DOI: 10.1016/j.medcli.2020.06.040 -
The American Journal of Surgical... Oct 2022Epstein-Barr virus (EBV)-positive plasmacytoma is a rare plasma cell neoplasm. It remains unclear whether EBV-positive plasmacytoma represents a distinct entity or a...
Epstein-Barr virus (EBV)-positive plasmacytoma is a rare plasma cell neoplasm. It remains unclear whether EBV-positive plasmacytoma represents a distinct entity or a variant of plasmacytoma. It shares morphologic features with plasmablastic lymphoma (PBL) and may cause diagnostic uncertainty. To better understand EBV-positive plasmacytoma and explore diagnostic criteria, this study describes 19 cases of EBV-positive plasmacytoma, compared with 27 cases of EBV-negative plasmacytoma and 48 cases of EBV-positive PBL. We reviewed the clinicopathologic findings and performed immunohistochemistry, in situ hybridization for EBV, fluorescence in situ hybridization for MYC , and next-generation sequencing. We found that 63.2% of patients with EBV-positive plasmacytoma were immunocompromised. Anaplastic features were observed in 7/19 cases. MYC rearrangement was found in 25.0% of them, and extra copies of MYC in 81.3%. EBV-positive and EBV-negative plasmacytomas possessed similar clinicopathologic features, except more frequent cytologic atypia, bone involvement and MYC aberrations in the former group. The survival rate of patients with EBV-positive plasmacytoma was comparable to that of patients with EBV-negative plasmacytoma. In comparison to PBL, EBV-positive plasmacytoma is less commonly associated with a "starry-sky" appearance, necrosis, absence of light chain expression, and a high Ki67 index (>75%). The most recurrently mutated genes/signaling pathways in EBV-positive plasmacytoma are epigenetic regulators, MAPK pathway, and DNA damage response, while the most frequently reported mutations in PBL are not observed. Collectively, EBV-positive plasmacytoma should be regarded as a biological variant of plasmacytoma. Thorough morphologic examination remains the cornerstone for distinguishing EBV-positive plasmacytoma and PBL, and molecular studies can be a valuable complementary tool.
Topics: Epstein-Barr Virus Infections; Herpesvirus 4, Human; Humans; In Situ Hybridization, Fluorescence; Ki-67 Antigen; Plasmacytoma
PubMed: 35650679
DOI: 10.1097/PAS.0000000000001923