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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 -
Frontiers in Endocrinology 2022Extramedullary plasmacytoma (EMP) can occur in various parts of the body. It is generally accepted that the highest site of occurrence is the head and neck region (80%...
RATIONALE
Extramedullary plasmacytoma (EMP) can occur in various parts of the body. It is generally accepted that the highest site of occurrence is the head and neck region (80% to 90%), followed by the gastrointestinal tract and the skin. It is worth mentioning that the site of disease, in this case, was the urethral meatus, which is extremely rare in clinical practice.
PATIENT CONCERNS
A 50-year-old female complained of an episode of painless gross hematuria without symptoms of frequent urination, urgency, abdominal pain, abdominal distension, fever, or oliguria. The patient has no history of smoking or drinking and denied any family history of solid malignancy or hematological disease.
DIAGNOSIS AND INTERVENTIONS
Urethrocystoscopy revealed urethral polypoid hyperplasia, which we initially thought could be a urethral caruncle. The patient was asked to undergo caruncle resection after 1 week of potassium permanganate sitz bath, and postoperative pathology revealed plasmacytoma. After that, a whole-body MRI showed no other lesions. She received postoperative radiotherapy.
OUTCOMES
During radiotherapy, the patient's condition and appetite were fair, and the skin mucositis was I°. Pelvic contrast-enhanced MRI and whole-body PET-CT 6 months after urethral meatus lumpectomy and radiotherapy showed changes without obvious abnormal signs. No recurrence or metastasis was detected after one year of follow-up.
LESSONS
Urinary EMP is a rare disease. It is not easy to diagnose due to its rare site of occurrence and non-specific symptoms. The diagnosis of EMP requires a combination of imaging studies and pathological findings. Currently, surgery combined with radiotherapy or radiotherapy alone is the mainstay treatment and usually results in an acceptable local control rate. At the same time, chemotherapy cannot be ignored.
Topics: Female; Humans; Magnetic Resonance Imaging; Middle Aged; Plasmacytoma; Positron Emission Tomography Computed Tomography
PubMed: 35250855
DOI: 10.3389/fendo.2022.783855 -
Radiologia 2014Extramedullary plasmacytomas are uncommon malignant plasma cell tumors that present outside the bone marrow; 80% of extramedullary plasmacytomas are located in the upper...
Extramedullary plasmacytomas are uncommon malignant plasma cell tumors that present outside the bone marrow; 80% of extramedullary plasmacytomas are located in the upper respiratory tract, and gastrointestinal plasmacytomas are rare. We present the case of an asymptomatic 65-year-old man in whom a pancreatic mass was found incidentally. The lesion was determined to be a pancreatic plasmacytoma after fine-needle aspiration cytology and surgical resection. No clinical, laboratory, or imaging findings indicative of multiple myeloma or association with other plasmacytomas were found, so the tumor was considered to be a primary pancreatic plasmacytoma.
Topics: Aged; Humans; Male; Pancreatic Neoplasms; Plasmacytoma
PubMed: 22738942
DOI: 10.1016/j.rx.2011.10.010 -
Cancer Medicine Jan 2021Solitary plasmacytoma of bone (SPB) is a single, isolated plasmacytoma originated from the bone. The survival trends of patients with SPB in recent years remain unknown....
Solitary plasmacytoma of bone (SPB) is a single, isolated plasmacytoma originated from the bone. The survival trends of patients with SPB in recent years remain unknown. And the prognostic system of SPB may also need to be refined. The 18 Surveillance, Epidemiology, and End Results (SEER) databases of the National Cancer Institute in the United States were used to extract data for this study. The third edition of the International Classification of Disease for Oncology (ICD-O-3) code 9731 was used to identify cases of SPB. For each case, factors including age at the time of diagnosis, sex, race, marital status, insurance status, primary sites of tumors, and the use of surgery were collected. The outcomes of patients with SPB were compared between two groups. And the prognostic impacts of baseline characteristics and use of surgery was studied. A total of 4103 (from 1976 to 2016) cases of SPB were identified. The median age was 65 years old. Patients in time period-2 (2008-2016) show better survival as compared to those in time period-1(1976-2007) (median overall survival: 88 months vs. 73 months, p = 0.0332). Age ≤ 65 years and being male were associated with better outcomes. The widowed individuals had significantly inferior survival and myeloma-specific survival than the single, married, or divorced individuals (p values all <0.0001). Patients with lesions in bones of skull and face and associated joints had longer survival as compared with those with bone lesions in other sites (median overall survival: 107 months vs. 79 months, p = 0.0694). The use of surgery was significantly associated with improved survival (median survival: surgery performed 98 months vs. not performed 73 months, hazards ratio [HR]: 0.7623, 95% CI: 0.7009-0.8472; p < 0.0001) and myeloma-specific survival (median myeloma-specific survival: surgery 160.0 months vs. no surgery 143.0 months, HR: 0.8469, 95% CI: 0.7493-0.9572; p = 0.0078). Multivariable analysis revealed that surgery, marital status, and age were independent prognostic factors for overall survival in patients with SPB. The improvement in the survival of patients with SPB has been observed in recent years. And several potential prognostic factors were identified. Future prospective studies are warranted to explore the roles of novel agents, surgery, and systemic chemotherapy in the treatment of SPB.
Topics: Aged; Bone Neoplasms; Female; Follow-Up Studies; Humans; Male; Middle Aged; Plasmacytoma; Prognosis; Retrospective Studies; Survival Rate
PubMed: 33145987
DOI: 10.1002/cam4.3533 -
Future Oncology (London, England) Jul 2021
Topics: Bone Neoplasms; Combined Modality Therapy; Humans; Jaw Neoplasms; Plasmacytoma
PubMed: 34047196
DOI: 10.2217/fon-2021-0316 -
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 -
Clinical Lymphoma, Myeloma & Leukemia Dec 2016Multiple myeloma/plasmacytoma-like posttransplantation lymphoproliferative disorder (PTLD-MM) is a rare complication of solid organ transplantation. Case series have...
BACKGROUND
Multiple myeloma/plasmacytoma-like posttransplantation lymphoproliferative disorder (PTLD-MM) is a rare complication of solid organ transplantation. Case series have shown variable outcomes, and survival data in the modern era are lacking.
PATIENTS AND METHODS
A cohort of 212 PTLD-MM patients was identified in the Scientific Registry of Transplant Recipients between 1999 and 2011. Overall survival (OS) was estimated by the Kaplan-Meier method, and the effects of treatment and patient characteristics on OS were evaluated by Cox proportional hazards models. OS in 185 PTLD-MM patients was compared to 4048 matched controls with multiple myeloma (SEER-MM) derived from Surveillance, Epidemiology, and End Results (SEER) data.
RESULTS
Men comprised 71% of patients; extramedullary disease was noted in 58%. Novel therapeutic agents were used in 19% of patients (more commonly during 2007-2011 vs. 1999-2006; P = .01), reduced immunosuppression in 55%, and chemotherapy in 32%. Median OS was 2.4 years and improved in the later time period (adjusted hazard ratio [aHR], 0.64, P = .05). Advanced age, creatinine > 2 g/dL, white race, and use of OKT3 were associated with inferior OS in multivariable analysis. OS of PTLD-MM patients is significantly inferior to SEER-MM patients (aHR, 1.6, P < .001). Improvements in OS over time differed between PTLD-MM and SEER-MM. Median OS of patients diagnosed from 2000 to 2005 was shorter for PTLD-MM than SEER-MM patients (18 vs. 47 months, P < .001). There was no difference among those diagnosed from 2006 to 2010 (44 months vs. median not reached, P = .5; interaction P = .08).
CONCLUSION
Age at diagnosis, elevated creatinine, white race, and OKT3 were associated with inferior survival in patients with PTLD-MM. Survival of PTLD-MM is inferior to SEER-MM, although significant improvements in survival have been documented.
Topics: Aged; Female; Humans; Immunosuppression Therapy; Kaplan-Meier Estimate; Lymphoproliferative Disorders; Male; Middle Aged; Multiple Myeloma; Organ Transplantation; Plasmacytoma; Prognosis; Proportional Hazards Models; Risk Factors; SEER Program; Survival Analysis; United States
PubMed: 27771291
DOI: 10.1016/j.clml.2016.09.002 -
In Vivo (Athens, Greece) 2020Solitary extramedullary plasmacytoma (SEP) is a rare, malignant plasma-cell tumor, which mainly occurs in the head and neck regions. Globally the disease has been rarely...
BACKGROUND/AIM
Solitary extramedullary plasmacytoma (SEP) is a rare, malignant plasma-cell tumor, which mainly occurs in the head and neck regions. Globally the disease has been rarely happening up to 2019, with only about ten papers focused on SEP cases reported in English. Thus, a literature collectively reviewing the characteristics of the patients would be valuable.
PATIENTS AND METHODS
We enrolled 10 SEP patients, and recorded their primary sites and the treatment modality, and analyzed their survival rates and outcomes. We also reviewed previous studies and compared their findings with ours.
RESULTS
No gender or age disparity has been observed, and younger patients had a better local control with RT compared to surgery among our patients.
CONCLUSION
Further investigations with more patients and long-time follow-up may provide more information for treatment determination and the recurrence and progression from SEP to MM.
Topics: Adult; Aged; Disease Progression; Female; Follow-Up Studies; Head and Neck Neoplasms; Humans; Male; Middle Aged; Neoplasm Recurrence, Local; Plasmacytoma; Prognosis; Taiwan
PubMed: 31882487
DOI: 10.21873/invivo.11769 -
Blood Sep 2000Most patients with multiple myeloma (MM) present with symptoms, have evidence of generalized disease, and require chemotherapy promptly to reduce the malignant clone.... (Review)
Review
Most patients with multiple myeloma (MM) present with symptoms, have evidence of generalized disease, and require chemotherapy promptly to reduce the malignant clone. Some patients present with a local symptom from a single plasmacytoma but no myeloma elsewhere. Such patients usually become free of symptoms after local radiotherapy. In patients with MM without symptoms, the diagnosis is made on the basis of screening laboratory tests. In patients with either solitary plasmacytoma of bone or asymptomatic MM, systemic treatment should be deferred until there is evidence of disease progression.
Topics: Bone Neoplasms; Humans; Multiple Myeloma; Plasmacytoma
PubMed: 10979944
DOI: No ID Found -
Blood Apr 2020
Topics: Adult; Humans; Immunoglobulin A; Male; Plasmacytoma; Prognosis
PubMed: 32324872
DOI: 10.1182/blood.2020004978