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Extramedullary plasmacytoma (EMP) is an uncommon disorder characterized by the development of abnormal plasma cell tumors outside the bone marrow. These tumors are typically observed in various locations, including the upper respiratory tract, gastrointestinal tract, and other soft tissues. Among the less explored manifestations of EMP is intracranial EMP, which remains poorly understood due to the limited literature available on the subject. The objective was to comprehend the population characteristics, localization, type, treatment, and outcomes of intracranial EMP. A systematic review of the literature for EMPs was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines. The strategy "extramedullary plasmacytoma AND multiple myeloma" was used for the search. The search terms were queried using PubMed, Embase, Scopus, Cochrane, and Web of Science databases. We included only those studies that presented clinical studies with patients diagnosed with intracranial plasmacytomas. In this study, a total of 84 patients from 25 studies were analyzed. The average age of diagnosis was 57.25 years, with a slightly higher proportion of females (57%) compared to male patients (43%). The most common locations of intracranial plasmacytomas were the clivus (29.7%), frontal lobe (18.9%), parietal lobe (8.1%), occipital lobe (6.7%), temporal lobe (6.7%), and sphenoid (4%). Chordoma and meningioma were the most common differential diagnoses encountered during clinical investigations. Treatment modalities included radiotherapy (RT), chemotherapy (QT), surgical resection (SR), and conservative approaches. The most frequent treatment combinations were SR + RT (19%) and RT only (17.8%). Mortality was reported in 48% of the cases, with complete resolution observed in 10 cases and partial resolution in 3 cases. The average follow-up duration was 37.5 months. The clivus is the most frequently reported site of extramedullary intracranial plasmacytoma (EMIP) occurrence, representing 29.7% of cases. Chordomas were commonly observed alongside EMIPs and emerged as the primary differential diagnosis. RT was the predominant treatment modality, with SR considered when feasible. RT alone demonstrated the highest effectiveness in managing EMIPs (30%), while QT as a sole intervention showed lower efficacy. However, a combination of dexamethasone, lenalidomide, and targeted RT displayed promising results, offering improved tumor response and increased safety. [ABSTRACT FROM AUTHOR]

A plasmacytoma is a cancerous growth of abnormal plasma cells that arise within osseous or soft tissue structures. In soft tissue structures, plasmacytomas can present as solitary or multiple masses in the absence of systemic involvement. Solitary plasmacytomas can be subcategorized as extramedullary plasmacytoma (derived from plasma cells located in soft tissues) or osseous plasmacytoma (derived from plasma cells located in the bone marrow). Infrequently, these tumors can arise as extramedullary lesions from the pancreas and present similarly to other tumors, such as pancreatic neuroendocrine tumors (PNETs). In this article, we report the case of a 62-year-old male with a diagnosis of solitary plasmacytoma of the pancreas. We focus on optimizing diagnosis and management through the application of radiological imaging modalities, specifically computed tomography (CT) scans and positron emission tomography-computed tomography (PET-CT) scans.

Extramedullary plasmacytomas (EMPs) are solitary plasma cell neoplasms that involve sites other than bone or bone marrow. It constitutes only 0.4% of tumors of head-and-neck region. Surgery and radiotherapy are standard treatment, depending on the site. Radiotherapy promises 90–100% local control rate and prolonged survival. Here, we are reporting a case of plasmacytoma nasopharynx treated with volumetric-modulated arc technique (VMAT) radiotherapy, with contouring details, dose to target, and nearby structures. Patient had complete response and is relapse free for the past 3 years with minimal toxicity. In our case, the lesion is in nasopharynx and is about 1cm, as such it required precise radiotherapy technique like VMAT, to give targeted dose delivery and sparing the nearby critical organs.

Solitary Plasmacytoma (SP) is a rare disease characterised by a localised proliferation of neoplastic monoclonal plasma cells, without evidence of systemic disease. The aetiology of SP remains unknown, but chronic stimulation, overdose irradiation, viruses, and gene interactions in the reticuloendothelial system have been suggested as the aetiologic factor. While pain is the most common symptom of osseous mandible SP, it is usually painless in the maxilla and may manifest orally with paresthesia, swelling, soft tissue masses, mobility and migration of teeth, haemorrhage, or pathologic fracture due to cortical destruction of bone. Because the amount of haemopoitic bone marrow in the maxilla is decreased, oral involvement of the maxilla is less common than that of the mandible. Here, authors reported a case of 46-year-old male patient presented with the chief complaint of mobility of his upper left back teeth and swelling over his left-side palatal area. Clinical examination was suggestive of benign odontogenic tumors and salivary gland tumors, while two-Dimensional (2D) and three-Dimensional (3D) imaging were suggestive of malignant tumors arising from either alveolar bone or maxillary sinus. The laboratory investigations and biopsy revealed solitary plasmacytoma of maxilla. There were clinical symptoms of paresthesia and mobility, but no other signs of malignancy were observed. As the case report concludes, all necessary investigations need to be conducted to exclude all other pathologies, so that treatment can begin as soon as possible.

Solitary Plasmacytoma (SP) is a rare disease characterized by a localized proliferation of neoplastic monoclonal plasma cells, without evidence of systemic disease. The aetiology of SP remains unknown, but chronic stimulation, overdose irradiation, viruses, and gene interactions in the reticuloendothelial system have been suggested as the etiologic factor. While pain is the most common symptom of osseous mandible SP, it is usually painless in the maxilla and may manifest orally with paresthesia, swelling, soft tissue masses, mobility and migration of teeth, haemorrhage, or pathologic fracture due to cortical destruction of bone. Because the amount of haemopoitic bone marrow in the maxilla is decreased, oral involvement of the maxilla is less common than that of the mandible. A 46-year-old male patient presented with the chief complaint of mobility of his upper left back teeth and swelling over his left-side palatal area. Clinical examination was suggestive of benign odontogenic tumors and salivary gland tumors, while two-dimensional (2D) and three-dimensional (3D) imaging were suggestive of malignant tumors arising from either alveolar bone or maxillary sinus. The laboratory investigations and biopsy revealed solitary plasmacytoma of maxilla. There were clinical symptoms of paresthesia and mobility, but no other signs of malignancy were observed. As the case report concludes, all necessary investigations need to be conducted to exclude all other pathologies, so that treatment can begin as soon as possible. [ABSTRACT FROM AUTHOR]

The prognostic impact of extramedullary plasmacytomas (EMPs) on newly diagnosed symptomatic multiple myeloma (MM) was evaluated in the context of treatment approach including autologous stem cell transplantation (ASCT) and chemotherapy alone. A total of 275 consecutive patients with newly diagnosed MM were included, and 54 patients (19.6 %) had EMPs at diagnosis. Patients with initial EMPs were more likely to have myeloma bone disease but favorable laboratory parameters in hemoglobin and β2-microglobulin. Patients were treated with different schemas based on transplant eligibility (154 in ASCT-eligible vs. 121 in ASCT-ineligible). After a median follow-up of 24.6 months (range, 0.2-56.3 months) in survivors, patients with initial EMPs had significantly worse progression-free survival (PFS) ( P = 0.035) and overall survival (OS) ( P = 0.006) compared to those without initial EMPs. In the multivariate analyses, the presence of initial EMPs was an independent prognostic factor for PFS (relative risk (RR) of 2.24, P = 0.024) and OS (RR of 2.47, P = 0.027) in the transplant-ineligible patients, whereas it did not significantly influence PFS ( P = 0.341) or OS ( P = 0.499) in the transplant-eligible patients. However, the adverse impact of EMPs observed in transplant-ineligible patients was attenuated among the patients treated with bortezomib. These data suggest that ASCT can overcome the negative impact of EMPs and highlight the potential efficacy of bortezomib on EMPs in the non-transplant setting. [ABSTRACT FROM AUTHOR]

Introduction: Pancreatic involvement by plasma cell neoplasms is an extremely rare event, with only 50 cases described in the literature. They can present as a primary solitary extramedullary plasmacytoma or plasmacytoma secondary to a plasma cell myeloma. Clinical manifestations are due to the presence of a pancreatic mass usually in the pancreas head, which causes extra-biliary obstruction and abdominal pain. Methods: Abdominal imaging including CT scan or endoscopic ultrasound with fine-needle aspiration tissue sampling is essential for the initial diagnostic procedure. However, immunohistochemical analysis of the biopsy specimen or flow cytometry of the aspirated material is crucial to prove the monoclonality and the final diagnosis of a plasma cell neoplasm. Discussion: Management of these situations include radiotherapy, chemotherapy, surgery or combined therapy. Novel medications including the immunomodulatory drugs or the proteasome inhibitors followed by consolidation with intensive chemotherapy and haematopoietic stem cell transplantation are nowadays used as upfront treatment in the cases associated to a plasma cell myeloma. Conclusion: Despite the rarity, plasma cell neoplasms should be considered in the differential diagnosis of obstructive jaundice and pancreatic neoplasms since they are potentially treatable situations. [ABSTRACT FROM AUTHOR]

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We report a rare case of a gastric plasmacytoma treated with endoscopic resection and oral thalidomide therapy. A 70-year-old man was admitted to our hospital with indigestion. He had no specific medical history and unremarkable laboratory results. Gastroendoscopic findings revealed a focal, erythematous, flat elevated lesion in the anterior wall of the stomach antrum. A biopsy revealed atypical lymphocytes. Endoscopic submucosal dissection (ESD) with an insulation-tipped knife was performed 45 days after diagnosis. Radiological and hematological evaluations, including a bone marrow biopsy, were performed and showed no involvement of other organs. The patient was diagnosed with extramedullary gastric plasmacytoma. Follow-up gastroendoscopy was performed three times during a 2-year period and showed nonspecific ESD scarring. The patient's condition was found to be stable.