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Thoracic Cancer Mar 2021Extramedullary plasmacytoma (EMP) is a rare plasma cell tumor involving the organs but without bone marrow involvement or the characteristics of multiple myeloma....
Extramedullary plasmacytoma (EMP) is a rare plasma cell tumor involving the organs but without bone marrow involvement or the characteristics of multiple myeloma. Primary solitary endobronchial plasmacytoma is extremely rare. Here we present the case of an 86-year-old male ex-smoker who visited our outpatient clinic for an endobronchial mass in the left upper lobe of the lung. Fiberoptic bronchoscopy revealed a protruding mass in the left upper lobar bronchus; based on the bronchoscopic biopsy findings, a primary solitary endobronchial plasmacytoma was diagnosed. After radiation therapy the patient was well and 6 months after treatment showed no evidence of disease recurrence. Extramedullary plasmacytoma should be considered in the differential diagnosis of an endobronchial mass.
Topics: Aged, 80 and over; Bronchial Neoplasms; Humans; Male; Plasmacytoma; Rare Diseases
PubMed: 33501775
DOI: 10.1111/1759-7714.13853 -
American Journal of Hematology Oct 2002Plasmacytomas of the testis are rare neoplasias; they may occur as isolated tumors or in concomitance with generalized multiple myeloma. We report the case of a... (Review)
Review
Plasmacytomas of the testis are rare neoplasias; they may occur as isolated tumors or in concomitance with generalized multiple myeloma. We report the case of a 77-year-old man with previous clinical evidence of multiple myeloma involving skin, ribs, and lungs, and initially treated with surgery, radiotherapy, and chemotherapy attaining partial response. Fourteen months after the onset, the patient presented with left testicular enlargement due to plasmacytoma. Immunohistochemical stains showed monoclonal cytoplasmic IgA-lambda in tumour cells; serum M component showed the same immunoglobulin. Following radiotherapy the tumour mass disappeared. Nonetheless, 2 months later while on chemotherapy, disease recurred with progressive increase of skeletal lesions. The patient is currently alive with disease progression 22 months after onset. On the basis of a review of the literature, the clinical significance of testicular myeloma localization is discussed.
Topics: Aged; Combined Modality Therapy; Disease Progression; Humans; Immunoglobulin A; Immunoglobulin lambda-Chains; Male; Multiple Myeloma; Plasmacytoma; Testicular Neoplasms
PubMed: 12353308
DOI: 10.1002/ajh.10174 -
Impact of radiation dose on local control and survival in extramedullary head and neck plasmacytoma.Radiation Oncology (London, England) Apr 2019Patients with plasma-cell neoplasia usually suffer from systemic disease, although a minority (< 5%) may present with solitary involvement of bone or soft tissue...
BACKGROUND
Patients with plasma-cell neoplasia usually suffer from systemic disease, although a minority (< 5%) may present with solitary involvement of bone or soft tissue (extramedullary plasmacytoma (EMP)). Radiotherapy (RT) is a state-of-the-art treatment for these tumors offering long term curation.
METHODS AND MATERIALS
Between January 2005 and January 2017, twenty-seven patients underwent RT at our institution. The aim of this study was to analyse the effectiveness of various RT doses for different forms of EMP.
RESULTS
A total of 33 radiation courses were administered to 27 patients with a median age of 56 years. The median RT dose was 45 Gy (range: 12-55.8). The local control rate was 76% (93% for primary EMP vs. 61% for the secondary EMP lesions; P < 0.05). A complete response (CR) rate to local RT was achieved for 42% lesions (67% for primary EMP vs. 22% for the secondary EMP lesions; P < 0.01). The overall response rate (ORR) for the EMP lesions treated with high-dose regimens (> 45 Gy) versus low-dose regimens (≤ 45 Gy) was 87% versus 67%, respectively (P = 0.2). The median survival with high-dose RT group was significantly longer (P = 0.02). In subgroups analysis, primary EMP patients treated with high-dose RT had a non-significant higher ORR (100% vs. 80%, respectively; P = 0.3,) longer duration of LC (P = 0.3) with a longer survival (P = 0.05) than patients in low-dose group. No significant difference has been detected in secondary EMP patients treated with high-dose RT regarding ORR (60% vs. 62%, respectively; P = 1), and survival (P = 0.4).
CONCLUSION
RT is an efficacious treatment modality in the treatment of EMP. A radiation dose ≤45 Gy confer a comparable CR rate to high-dose regimens and appears to be an effective treatment for controlling local EMP progression. Radiation dose-escalation may be beneficial for particular subgroups of patients.
Topics: Adult; Aged; Aged, 80 and over; Cohort Studies; Female; Head and Neck Neoplasms; Humans; Male; Middle Aged; Organs at Risk; Plasmacytoma; Prognosis; Radiotherapy Dosage; Radiotherapy Planning, Computer-Assisted; Radiotherapy, Intensity-Modulated; Survival Rate
PubMed: 30987659
DOI: 10.1186/s13014-019-1265-5 -
Journal of Neurology, Neurosurgery, and... Feb 1978Three patients with peripheral neuropathy and a solitary plasmacytoma are presented, and the literature is reviewed. It is suggested that middle-aged men with an obscure...
Three patients with peripheral neuropathy and a solitary plasmacytoma are presented, and the literature is reviewed. It is suggested that middle-aged men with an obscure progressive sensorimotor neuropathy, a raised CSF protein, and otherwise negative investigations should have a full skeletal survey since irradiation of a plasmacytoma may lead to a considerable improvement in the associated neurological disability.
Topics: Adult; Bone Neoplasms; Cerebrospinal Fluid Proteins; Clavicle; Demyelinating Diseases; Humans; Ilium; Male; Middle Aged; Nerve Degeneration; Paraproteins; Peripheral Nervous System Diseases; Plasmacytoma; Ribs
PubMed: 204745
DOI: 10.1136/jnnp.41.2.177 -
BMC Cancer Jan 2017Solitary plasmacytoma (SP) is a localized neoplastic plasma cell disorder with an annual incidence of less than 450 cases. Given the rarity of this disorder, it is...
BACKGROUND
Solitary plasmacytoma (SP) is a localized neoplastic plasma cell disorder with an annual incidence of less than 450 cases. Given the rarity of this disorder, it is difficult to conduct large-scale population studies. Consequently, very limited information on the disorder is available, making it difficult to estimate the incidence and survival rates. Furthermore, limited information is available on the efficacy of various treatment modalities in relation to primary tumor sites.
METHODS
The data for this retrospective study were drawn from the Surveillance, Epidemiology and End Results (SEER) database, which comprises 18 registries; patient demographics, treatment modalities and survival rates were obtained for those diagnosed with SP from 1998 to 2007. Various prognostic factors were analyzed via Kaplan-Meier analysis and log-rank test, with 5-year relative survival rate defined as the primary outcome of interest. Cox regression analysis was employed in the multivariate analysis.
RESULTS
The SEER search from 1998 to 2007 yielded records for 1691 SP patients. The median age at diagnosis was 63 years. The patient cohort was 62.4% male, 37.6% female, 80% Caucasian, 14.6% African American and 5.4% other races. Additionally, 57.8% had osseous plasmacytoma, and 31.9% had extraosseous involvement. Unspecified plasmacytoma was noted in 10.2% of patients. The most common treatment modalities were radiotherapy (RT) (48.8%), followed by combination surgery with RT (21.2%) and surgery alone (11.6%). Univariate analysis of prognostic factors revealed that the survival outcomes were better for younger male patients who received RT with surgery (p < 0.05). Additionally, patients who received neoadjuvant RT had increased survival rates compared to those receiving adjuvant RT (86% vs 73%, p < 0.05). Furthermore, the analyses revealed that 5-year survival rates for patients with axial plasmacytoma were superior when RT was combined with surgery (p < 0.05). In the multivariate analysis, age <60 years and treatment with either RT or surgery showed superior survival rates. Progression to multiple myeloma (MM) was noted in 551 patients. Age >60 years was associated with a lower 5-year survival in patients who progressed to MM compared to those who were diagnosed initially with MM (15.1 vs 16.6%). Finally, those who received RT and progressed to MM still had a higher chance of survival than those who were diagnosed with MM initially and treated with RT/surgery (21.8% vs 15.9%, p < 0.05).
CONCLUSIONS
A review of the pertinent literature indicates that we provided the most comprehensive population-based analysis of SP to date. Moreover, our study contributes to the establishment of the optimal SP treatment modality, as RT is the favored option in frontline settings. Consensus is currently lacking regarding the benefits of combined treatment including surgery. Thus, the findings reported here elucidate the role of primary treatment modalities while also demonstrating the quantifiable benefits of combining RT with surgery in relation to different primary tumor sites. While our results are promising, they should be confirmed through further large-scale randomized studies.
Topics: Adult; Aged; Female; Humans; Kaplan-Meier Estimate; Male; Middle Aged; Plasmacytoma; Proportional Hazards Models; Retrospective Studies; SEER Program; United States
PubMed: 28056880
DOI: 10.1186/s12885-016-3015-5 -
Journal of Clinical and Experimental... 2013A 56-year-old man developed epistaxis, hoarseness, and swelling of a finger in March 2010. On the basis of biopsies of the masses in the pharynx and finger, he was... (Review)
Review
A 56-year-old man developed epistaxis, hoarseness, and swelling of a finger in March 2010. On the basis of biopsies of the masses in the pharynx and finger, he was diagnosed with extramedullary plasmablastic plasmacytoma, with somewhat immature CD45(+), MPC-1(-), and CD49e(-). CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisolone) and VCAP (vincristine, cyclophosphamide, doxorubicin, and prednisolone) therapy was ineffective, but combination therapy with thalidomide and dexamethasone was highly effective. Thalidomide monotherapy successfully maintained partial remission for approximately 7 months. A mass appeared in the right neck in February 2011, and a biopsy confirmed recurrence. Changes to CD45 negativity and MPC-1 partial positivity were seen, while CD49e negativity persisted, suggesting that the plasmablastic plasmacytoma had reverted to a more immature state. Bortezomib therapy was started in March 2011 and was effective. However, during the second round of treatment, the patient developed acute lung injury, which was improved by steroid pulse therapy. After the discontinuation of bortezomib, the extramedullary mass increased rapidly, and the patient died of multiple organ failure. An autopsy showed that plasmablastic plasmacytomas had infiltrated into multiple organs. Extramedullary plasmacytomas and those that revert to an immature state are associated with a poor prognosis, so further treatment improvements are needed in the future.
Topics: Antineoplastic Combined Chemotherapy Protocols; Dexamethasone; Humans; Male; Middle Aged; Multiple Myeloma; Plasmacytoma; Thalidomide
PubMed: 23801130
DOI: 10.3960/jslrt.53.21 -
International Journal of Clinical and... 2015A paucity of data exists concerning the presentation, natural course and outcome of extramedullary plasmcytoma (EMP). It is difficult to determine the optimal treatment... (Review)
Review
A paucity of data exists concerning the presentation, natural course and outcome of extramedullary plasmcytoma (EMP). It is difficult to determine the optimal treatment strategy and prognostic factors for EMP. We present an additional case of laryngeal EMP and systemic review relevant reports in the English and Chinese literature. We found, to our knowledge, 147 cases in larynx in the English-language literature and Chinese-literature. The most common treatment modality was radiotherapy alone. The mean survival duration was ~184 months, and the 5- and 10- year survival rates were 76.1% and 67.4%, respectively. The univariate analysis suggested that progression to multiple myeloma and amyloid deposits may be poor prognostic factors. The multivariate analysis suggested that only progression to multiple myeloma may be a poor prognostic factor. Laryngeal EMP is uncommon. Progression to multiple myeloma may be a poor prognostic factor.
Topics: Adult; Aged; Aged, 80 and over; Biomarkers, Tumor; Biopsy; Diffusion Magnetic Resonance Imaging; Disease Progression; Dose Fractionation, Radiation; Female; Humans; Immunohistochemistry; Laryngeal Neoplasms; Laryngoscopy; Male; Middle Aged; Multiple Myeloma; Multivariate Analysis; Plasmacytoma; Radiotherapy, Adjuvant; Risk Factors; Stroboscopy; Time Factors; Tracheostomy; Treatment Outcome
PubMed: 26045749
DOI: No ID Found -
Canadian Respiratory Journal 2013
Topics: Bone Neoplasms; Female; Humans; Middle Aged; Plasmacytoma; Ribs
PubMed: 23457668
DOI: 10.1155/2013/469876 -
BMC Medical Imaging Jun 2018Malignant plasma cell proliferation may present as a disseminated disease (multiple myeloma), a solitary plasmacytoma of bone, or an extramedullary plasmacytoma of soft...
BACKGROUND
Malignant plasma cell proliferation may present as a disseminated disease (multiple myeloma), a solitary plasmacytoma of bone, or an extramedullary plasmacytoma of soft tissue. The latter plasmacytomas represent approximately 3% of all plasma cell proliferations, and 80% develop in the head-and-neck region. The unexpected clinical presentation of such masses may be present.
CASE PRESENTATION
Here, we report a rare case of primary tonsillar plasmacytoma in a 42-year-old female. The patient presented with asymmetric tonsillar hypertrophy that was resistant to antibiotherapy. Upon further workup, we found no evidence of multiple myeloma or light-chain disease. The patient underwent surgery and, at the last follow-up, exhibited no evidence of such disease.
CONCLUSIONS
In adults presenting with asymptomatic tonsillar enlargement, the possibility of submucosal masses should be considered, thus encouraging the radiologist to evaluate crypts within the palatine tonsil on a postcontrast MRI, besides enlargement and signal change.
Topics: Adult; Female; Humans; Magnetic Resonance Imaging; Palatine Tonsil; Plasmacytoma; Tonsillar Neoplasms; Treatment Outcome
PubMed: 29914385
DOI: 10.1186/s12880-018-0261-9 -
The Korean Journal of Gastroenterology... May 2014Solitary extramedullary plasmacytoma (EMP) is a plasma cell neoplasm without bone marrow involvement. EMPs are rare in the gastrointestinal (GI) tract. We report two... (Review)
Review
Solitary extramedullary plasmacytoma (EMP) is a plasma cell neoplasm without bone marrow involvement. EMPs are rare in the gastrointestinal (GI) tract. We report two cases of primary EMP, one in the transverse colon and the other in the stomach. In the first case, a mass on the transverse colon was found on colonoscopy. The patient underwent left hemicolectomy and has been followed-up for 3 years without recurrence postoperatively. The latter case had several masses in the stomach. The patient underwent bypass surgery and has received supportive care for 1 month. Histopathologic specimens of both the cases showed a monoclonal lambda chain EMP. Subsequent investigations included a bone marrow biopsy, serum IgA, IgG, IgM and serum protein electrophoresis, and the results were negative for multiple myeloma in both the cases. Solitary EMP in the GI tract can be mistaken for colon cancer or stomach cancer on endoscopy; therefore, a sufficient number of biopsy specimens can help diagnose solitary EMPs. Surgical resection alone or with radiation therapy in cases with positive surgical margin is currently the only treatment for solitary EMP in the GI tract. Further study is necessary to determine disease prognosis and to investigate other treatment methods.
Topics: Colonic Neoplasms; Endoscopy, Gastrointestinal; Humans; Immunohistochemistry; Male; Middle Aged; Plasmacytoma; Positron-Emission Tomography; Stomach Neoplasms; Tomography, X-Ray Computed
PubMed: 24870305
DOI: 10.4166/kjg.2014.63.5.316