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Medicine Jan 2018Extramedullary plasmacytomas (EMPs) are rare solitary soft tissue tumors characterized by monoclonal proliferation of plasma cells. Most lesions occur in the head and... (Review)
Review
RATIONALE
Extramedullary plasmacytomas (EMPs) are rare solitary soft tissue tumors characterized by monoclonal proliferation of plasma cells. Most lesions occur in the head and neck, but primary tracheal lesions are very rare.
PATIENT CONCERNS
In this report, we describe a case of tracheal EMP discovered in a 48-year-old man who presented with a history of progressive dyspnea.
DIAGNOSES
Computed tomography (CT) revealed a well-defined nodular mass in the posterior wall of trachea without signs of invasion of the tracheal walls. Then, a reddish mass occluding approximately 90% of the trachea was evidenced by bronchoscopic examination.
INTERVENTIONS
The patient was treated with surgery followed by adjuvant radiotherapy to achieve better local control.
OUTCOMES
After the surgery, there was immediate symptomatic relief. There was no recurrence or metastasis during a 6-month follow-up.
LESSONS
This study presents a rare case of tracheal EMP occluding approximately 90% of the lumen that was successfully managed by surgery followed by radiotherapy.
Topics: Humans; Male; Middle Aged; Plasmacytoma; Tracheal Neoplasms
PubMed: 29504982
DOI: 10.1097/MD.0000000000009594 -
Annals of the Royal College of Surgeons... May 2018Introduction Solitary extramedullary plasmacytoma are rare, solid-mass tumours which appear immunophenotypically similar to multiple myeloma. The diagnosis and... (Review)
Review
Introduction Solitary extramedullary plasmacytoma are rare, solid-mass tumours which appear immunophenotypically similar to multiple myeloma. The diagnosis and management of gastrointestinal plasmacytoma is complex and requires multidisciplinary input. This study presents a narrative review of intra-abdominal extramedullary plasmacytoma, illustrated with two case studies. Methods The PubMed database was searched without date restrictions for reports of intra-abdominal extramedullary plasmacytoma to synthesise a narrative review. Electronic records were reviewed at a high-volume, quaternary soft-tissue sarcoma centre to identify patients with histopathologically confirmed extramedullary plasmacytoma affecting the gastrointestinal tract. Results Gastrointestinal extramedullary plasmacytomas can present with mass effect or organ-specific dysfunction. Techniques for tissue diagnosis of extramedullary plasmacytoma vary dependent on location, with a formal diagnosis often being made from a resected specimen. Management can include surgery, radiotherapy, systemic chemotherapy or a combination. No high-quality evidence base exists to guide treatment. Two case studies of operated gastrointestinal extramedullary plasmacytoma are presented at different phases of disease progression, with a resultant impact on survival. Conclusion Intra-abdominal extramedullary plasmacytoma is a rare and heterogeneous condition that lacks consensus guidelines for diagnosis and management. Collaboration between international specialist centres will create better quality evidence for treatment of this cohort.
Topics: Aged; Combined Modality Therapy; Disease Progression; Fatal Outcome; Female; Gastrointestinal Neoplasms; Humans; Male; Plasmacytoma
PubMed: 29692194
DOI: 10.1308/rcsann.2018.0015 -
Journal of Neurosurgery Jul 1999Intracranial solitary plasmacytomas are extremely rare tumors and are often misdiagnosed preoperatively. The authors report the successful treatment of a patient who... (Review)
Review
Intracranial solitary plasmacytomas are extremely rare tumors and are often misdiagnosed preoperatively. The authors report the successful treatment of a patient who harbored such a tumor involving both the falx and tentorium; this is the second case reported. A 59-year-old woman suffered from a seizure disorder due to a falcotentorial lesion, which had been identified 3 years earlier and was thought at the time to be an en plaque meningioma. Most recently, the patient presented with symptoms of increased intracranial pressure and hemiparesis. Computerized tomography and magnetic resonance imaging of her head revealed progressive growth of the tumor. The patient underwent partial resection of the tumor and chemo- and radiation therapies. Intracranial plasmacytomas must always be included in a differential diagnosis because potential complete cure can be achieved using fairly conservative treatment modalities.
Topics: Brain Neoplasms; Combined Modality Therapy; Diagnosis, Differential; Female; Hemiplegia; Humans; Intracranial Hypertension; Magnetic Resonance Imaging; Middle Aged; Plasmacytoma; Seizures; Supratentorial Neoplasms
PubMed: 10389893
DOI: 10.3171/jns.1999.91.1.0132 -
International Forum of Allergy &... Feb 2014This study reviews the published literature related to extramedullary sinonasal plasmacytomas. Clinical presentation, demographics, treatment, and outcomes of this... (Review)
Review
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.
Topics: Combined Modality Therapy; Humans; Nose Neoplasms; Paranasal Sinuses; Plasmacytoma; Survival Analysis; Treatment Outcome
PubMed: 24339430
DOI: 10.1002/alr.21254 -
Journal of Medical Case Reports May 2019Primary plasmacytomas are localized proliferations of clonal plasma cells occurring in the absence of a systemic plasma cell dyscrasia such as multiple myeloma. Primary... (Review)
Review
BACKGROUND
Primary plasmacytomas are localized proliferations of clonal plasma cells occurring in the absence of a systemic plasma cell dyscrasia such as multiple myeloma. Primary plasmacytomas most commonly manifest as solitary lesions of the bone or of the upper aerodigestive tract. Presentation in a lymph node is very uncommon and can often be initially mistaken for lymphoma. Because they are local phenomena, primary plasmacytomas are managed with local therapies such as radiation or, less commonly, excision. Multifocal presentations are rare and are often not amenable to local treatment modalities, thus requiring systemic therapies. Because of their rarity, standardized treatment guidelines are not established, and treatment paradigms borrow heavily from those employed in multiple myeloma. Multifocal presentation in lymph nodes is nearly unheard of with only seven such cases reported in the existing literature, only four of which were diffuse enough to require systemic therapy. Here we describe the most diffuse and widely distributed instance of primary lymph node plasmacytoma yet reported and present a description of its successful treatment with systemic therapy.
CASE PRESENTATION
A 71-year-old Asian man presented with progressive fatigue in the setting of diffuse hypermetabolic lymphadenopathy throughout his chest, abdomen, and pelvis. A diagnosis of lymphoma was initially suspected; however, a lymph node biopsy was consistent with plasmacytoma. A bone marrow biopsy was unremarkable, and no monoclonal protein was identified, establishing a diagnosis of primary extramedullary plasmacytomas of the lymph nodes. He was treated with a myeloma-like regimen consisting of four cycles of bortezomib/dexamethasone followed by two cycles of thalidomide/prednisone with improvement in symptoms and near complete resolution of prior hypermetabolic lymphadenopathy. He remains in remission over 18 months following completion of therapy.
CONCLUSION
This case report and accompanying literature review highlight the exceedingly rare and easily misclassified entity of primary plasmacytoma of diffuse lymph nodes. Importantly, we demonstrate that this entity may be treated with, and demonstrate excellent response to, systemic therapies often employed in multiple myeloma.
Topics: Aged; Bortezomib; Dexamethasone; Fatigue; Humans; Lymph Nodes; Lymphadenopathy; Male; Plasma Cells; Plasmacytoma; Prednisone; Remission Induction; Thalidomide
PubMed: 31113466
DOI: 10.1186/s13256-019-2087-7 -
Journal of the American Academy of... Feb 1996Primary plasmacytoma of the skin without evidence of bone marrow plasmacytosis is a rare disorder belonging to the heterogenous spectrum of plasma cell neoplasms. With... (Review)
Review
Primary plasmacytoma of the skin without evidence of bone marrow plasmacytosis is a rare disorder belonging to the heterogenous spectrum of plasma cell neoplasms. With immunohistochemical techniques, differentiation from benign plasma cell aggregates can be accomplished by demonstrating the monoclonality of tumor cells. We describe a patient in whom a solitary primary cutaneous plasmacytoma developed on the left thigh. Immunohistochemically, plasma cells showed restriction of immunoglobulin lambda-chain expression. Underlying multiple myeloma was excluded by serum protein and immunoglobulin electrophoresis, roentgenographic skeletal survey, and bone marrow biopsy. The tumor responded well to local electron-beam radiotherapy. Whereas multiple primary cutaneous plasmacytomas seem to carry a significant early mortality rate, the prognosis of solitary lesions is likely to be more favorable.
Topics: Female; Humans; Immunoglobulin lambda-Chains; Middle Aged; Plasmacytoma; Skin; Skin Neoplasms
PubMed: 8655732
DOI: 10.1016/s0190-9622(07)80014-4 -
American Journal of Otolaryngology 2003To define the optimal treatment and outcomes for patients with solitary plasmacytoma of bone and soft tissue. (Review)
Review
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.
Topics: Bone Neoplasms; Female; Humans; Male; Plasmacytoma; Prognosis; Soft Tissue Neoplasms; Survival Rate; Treatment Outcome
PubMed: 14608572
DOI: 10.1016/s0196-0709(03)00092-9 -
Journal of Medical Imaging and... Feb 2020Extramedullary plasmacytomas are rare and can occur at any site. The imaging results are often nonspecific. This report highlights the radiographic, sonographic and...
Extramedullary plasmacytomas are rare and can occur at any site. The imaging results are often nonspecific. This report highlights the radiographic, sonographic and cross-sectional imaging features of extramedullary plasmacytomas.
Topics: Adult; Aged; Aged, 80 and over; Diagnostic Imaging; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Plasmacytoma; Radiography; Retrospective Studies; Tomography, X-Ray Computed; Ultrasonography
PubMed: 31785037
DOI: 10.1111/1754-9485.12975 -
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 -
Ear, Nose, & Throat Journal Feb 2009Extramedullary plasmacytoma is uncommon, accounting for only 3% of all plasma cell neoplasms. Most lesions occur in the head and neck, primarily in the upper... (Review)
Review
Extramedullary plasmacytoma is uncommon, accounting for only 3% of all plasma cell neoplasms. Most lesions occur in the head and neck, primarily in the upper aerodigestive tract; only a minority occur in the larynx. Most cases of extramedullary plasmacytoma are seen in older men; presentation in an adolescent is rare. We report the case of a 13-year-old girl who presented with a 2- to 3-month history of progressive dysphonia. Physical examination identified two lesions--a nasopharyngeal mass and a laryngeal mass. Biopsy indicated that the masses were extramedullary plasmacytomas with amyloid deposition. The patient was treated with radiation therapy, and the tumors were eradicated. We also review the literature with respect to the epidemiology and presentation of extramedullary plasmacytoma of the head and neck, specifically those that affect the larynx.
Topics: Adolescent; Biopsy; Dysphonia; Female; Hoarseness; Humans; Laryngeal Neoplasms; Nasopharyngeal Neoplasms; Plasmacytoma; Radiotherapy, Adjuvant
PubMed: 19224468
DOI: No ID Found