Treatment Of Extramedullary Plasmacytoma Research Articles

Radiotherapy vs Surgery for Survival and Locoregional Control of Head and Neck Extramedullary Plasmacytoma

There are significant gaps in the literature pertaining to the locoregional control and survival rates of extramedullary plasmacytoma (EMP) with respect to various treatment approaches. To systematically evaluate the differences in radiotherapy and surgical outcomes in EMP. Databases including PubMed, Scopus, Web of Science, Embase, and ScienceDirect were systematically searched from their inception up to November 2023. Articles reporting radiotherapy and surgical outcomes of head and neck EMP were included. A random-effects model for meta-analysis was used to obtain pooled estimates and calculate hazard ratios for survival and odds ratios for recurrence and progression of EMP. Survival, tumor control, and progression rates to multiple myeloma (MM) between radiation therapy and surgery for EMP of the head and neck. Of 742 included patients from 12 studies, 527 (71.0%) were male, and the median (IQR) age was 59.1 (53-62) years. A total of 505 patients (68.1%) received radiotherapy only, while 237 (31.9%) underwent surgery-only treatment for EMP. All included patients had an initial diagnosis of EMP without MM. Comparable trends were observed in overall survival and disease-free survival (DFS) rates at 2, 3, 5, and 10 years between patients with EMP treated with radiotherapy only and surgery only. Notably, there were no significant differences in recurrence rate (odds ratio, 0.65; 95% CI, 0.20-2.06) between radiotherapy-only and surgery-only treatment. However, radiotherapy-only treatment of EMP was associated with decreased odds of progression to MM compared with surgery (odds ratio, 0.4; 95% CI, 0.1-0.9). Sensitivity analysis revealed that the radiotherapy-only population had significantly better 5-year DFS (hazard ratio, 0.55; 95% CI, 0.31-0.96) compared with surgery-only treatment. This systematic review and meta-analysis provides evidence that patients with EMP receiving radiotherapy had significantly lower chances of progression to MM compared with surgery-only therapy. Additionally, radiotherapy had better 5-year DFS outcomes compared with surgery. Comparable outcomes in terms of overall survival rates, recurrence, and mortality rates were noted between radiotherapy-only and surgery-only EMP treatment groups.

Extramedullary Intracranial Plasmacytomas: A Systematic Review of Literature.

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.

Consensus for the diagnosis and management of extramedullary plasmacytoma in China(2024)

Extramedullary plasmacytoma (EMP) is a special type of malignant plasmacytosis, which is complex and heterogeneous. Most EMP patients have poor prognosis and lack a stratified prognostic system or ideal treatment strategy supported by evidence-based medical evidence, which cannot meet clinical needs. In order to improve the understanding of this disease entity, Plasma Cell Disease Group, Chinese Society of Hematology, Chinese Medical Association and Chinese Myeloma Committee-Chinese Hematology Association developed the "Chinese Expert Consensus on the diagnosis and treatment of extramedullary plasmacytoma", which aims to standardize the clinical diagnosis and treatment of EMP and ultimately improve the overall survival of patients with plasmacytoma.

Extramedullary Plasmacytoma of The Frontal Sinus: A Case Report

We report a rare case of an extramedullary plasmacytoma (EMP) in the frontal sinus with an indolent clinical presentation. Although a history of trauma was absent, the initial diagnosis was a mucocele, based on the radiological findings. Upon surgical excision, the patient was found to have an EMP. EMP, a form of solitary plasmacytoma, has a significantly high rate of conversion to multiple myeloma. This mandates long-term follow-up, even after successful radiotherapy and/or resection. While radiation therapy is generally considered a first line treatment for EMP, surgical intervention may provide optimal treatment in complicated cases. This case presentation highlights the prognosis of patients diagnosed with EMP.

Plasma cell tumors of the head and neck. Analysis of diagnostic difficulties based on literature and own data

Background. Extramedullary plasmacytoma (EP) of soft tissues, which in most cases affects the organs of the head and neck region, is a relatively rare malignant tumor. Until now, there are no consensus approaches to the diagnosis and treatment of EP. Differentiating EP from other types of non-Hodgkin’s lymphomas is difficult. There are difficulties in the differential diagnosis of EP and carcinomas in the head and neck region. Given the rare occurrence of this nosological form, the frequency of diagnostic errors is quite high, which dictates the need for a thorough description of each head and neck EP case.Objective of the study: analysis of possible difficulties and reasons for incorrect interpretation of diagnostic data, and treatment for head and neck EP.Materials and methods. Clinical and morphoimmunological data of 97 primary patients with B-cell non-Hodgkin’s lymphomas (B-NHLs) of the head-neck region were analyzed.Results. In our cohort we identified 2 tumor cases of a plasma cell nature, which amounted to 2 % among all B-NHLs. In one case, the process was located in the nasal cavity and clinically manifested itself with nosebleeds. The second case is a lesion of the mouth floor, primarily with the ulcer formation. In the first cases, at diagnosis, the immunohistochemistry (IHC) test was performed after patient’s chemotherapy and radiation treatment, which distorted the tumor immunophenotype. In the second cases with extensive process in maxillary sinuses, a complete and very detailed IHC test was carried out; however the data did not allow for a definitive diagnosis. Difficulties apparently arose in the interpretation of CD38 expression – main marker of plasmacytic line cells, as well as due to the unusual morphology.Conclusion. The described diagnostic situations dictate the need for a comprehensive algorithm in the diagnosis of head and neck tumors. It is advisable to perform an extended morpho-immunophenotypic study of the tumor (IHC, immunocytology, flow cytometry, etc.), if a tumor of a plasma cell nature is suspected, a morpho-immunological study of the bone marrow is indicated.

Impact of radiation dose on local control and survival in extramedullary head and neck plasmacytoma

BackgroundPatients 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 materialsBetween 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.ResultsA 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).ConclusionRT 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.

Extramedullary plasmacytoma of the larynx: Literature review and report of a case who subsequently developed acute myeloid leukemia.

Extramedullary plasmacytoma (EMP) of the larynx is an extremely rare plasma cell neoplasm outside of the bone marrow, which has not been previously well characterized. A case of laryngeal EMP who developed acute myeloid leukemia (AML) following treatment is described in the present study, as well as an extensive review of the relevant literature. An electronic literature search was performed in PubMed and all pertinent case reports and series in the English language from 1948-October 2017 were identified. A total of 99 cases including the present case were available for review. The mean age of the included patients was 53 years. Supraglottis was the most frequently involved site. The most common treatment modality was radiotherapy alone (n=41; 43%), followed by a combination of surgery and radiotherapy, then surgery alone. However, for cases published in recent years, the most common treatment modality was surgically based treatment. Overall the treatment outcome was favorable, as a total of 84% of patients were alive after a mean follow-up of 60 months. However, EMP outcomes for patients with cervical lymphadenopathy or multiple sites involvement were unfavorable with >40% of patients relapsing or developing metastasis during the limited follow-up period. A total of 6 subjects developed multiple myeloma and 1 patient converted to AML. The present study provides important insights on the treatment of EMP, which is a rare disease. To the best of our knowledge, this is the first case report of a patient with laryngeal EMP who developed AML following treatment. It is recommended that secondary myeloid neoplasm should be considered besides multiple myeloma during the follow-up period.

Extramedullary Plasmacytoma of the Larynx Treated by a Surgical Endoscopic Approach and Radiotherapy

Extramedullary plasmacytoma (EMP) is a rare variant of plasma cell myeloma that affects soft tissues. The head and neck region are the most affected sites, although others have also been described. Herein we report an uncommon case of EMP of the larynx in a 65-year-old male who presented with a history of progressive dysphonia and hoarseness. Laryngeal fiberscopy evidenced a reddish pedicled voluminous mass in the left false cords and ventricle. Microscopic suspension laryngoscopy was performed under general anaesthesia and a 4 W Acublade CO2 Laser was used for transoral resection of the lesion. This was followed by adjuvant radiotherapy, with the widely recommended doses on the supraglottic region, to achieve better local control. Diagnosis of EMP is based on immunohistochemistry and the exclusion of systemic plasma cell proliferative disorders. Diagnosis of solitary EMP can be made only if studies for disseminated disease and X-ray and/or magnetic resonance imaging of the spine, pelvis, femurs, and humerus and bone marrow biopsy are negative. As there are no internationally established guidelines, treatment of EMP is mainly based on consensus of expert opinion.

Extramedullary relapse of multiple myeloma presenting as massive upper gastrointestinal bleeding: a rare complication

To the Editor, Multiple myeloma (MM) is a malignant disease of terminally differentiated B cells presenting with bone lesions, renal failure, infections, anemia, hypercalcemia, and hyperviscosity syndrome. The malignant plasma cells are usually confined to the bone marrow in MM. Extramedullary plasmacytoma (EMP) accounts for 4% of all plasma cell malignancies. EMP mainly occurs in the respiratory tract (82.2%), with gastrointestinal (GI) tract involvement in less than 5% of all EMP cases. The small bowel is the most common site of GI involvement, followed by the stomach, colon, and esophagus [1]. Here, we report a 69-year-old patient, admitted to hospital with hematemesis and melena, and finally diagnosed with massive upper GI bleeding caused by gastric plasmacytoma. A 69-year-old male presented at the Emergency Unit with hematemesis and melena. He was known to have a history of MM and had previously been treated with vincristine, doxorubicin, and dexamethasone therapy with a very good partial response. The patient underwent emergent esophagogastroduodenoscopy. There was fresh blood in the lumen, oozing from an ulcerated, multilobulated mass 3 × 3 cm in diameter occupying the greater curvature of the proximal corpus (Forrest 1B). In addition, there were many separated polypoid, ulcerated masses about 1 cm in diameter in the body of the stomach (Fig. 1). The bleeding was successfully ablated with adrenaline injection and heater probe cautery. The patient was treated with intravenous fluids, proton pump inhibitors, erythrocytes, and fresh frozen plasma suspensions in the intensive care unit. A repeat endoscopy was performed 48 hours later, which revealed the previously identified gastric masses without evidence of bleeding. Biopsies were obtained from the lesions. The pathology specimens showed diffuse atypical plasma cell infiltration with prominent staining for CD38 and lambda immunostains leading to a pathological diagnosis of plasmacytoma (Figs. 2 and ​and33). Figure 1 Endoscopic view of the gastric plasmacytomas. Figure 2 Gastric mucosa with diffuse atypical plasma cell infiltration (H&E, ×40). Figure 3 Immunostained section shows diffuse and strong reactivity for CD38 (×200). Extramedullary involvement, particularly involvement of the GI tract, is rare in MM. Most patients with gastric plasmacytomas are elderly and often present with nonspecific GI symptoms, including anorexia, weight loss, abdominal pain, vomiting, and rarely GI bleeding, usually from an ulcerated lesion. Intestinal obstruction or malabsorption may be the presenting symptom in patients with colonic or small bowel involvement [2]. On endoscopy, gastric plasmacytomas present as nodular ulcerated masses and occasionally irregular thickened gastric folds that can mimic gastrointestinal stromal tumor, lymphoma, or carcinoma [3]. The diagnosis is based on morphology and immunohistochemical demonstration of monoclonal kappa and lambda light chains or heavy chains found in plasma cells [4]. Although the treatment of EMP has not been standardized, managing mass-related local complications such as bleeding or bowel obstruction is critical in these patients. Surgery and irradiation with or without chemotherapy might be other treatment options [5]. Once GI involvement is present, the prognosis is very poor despite aggressive therapy. Consequently, although very rare, gastric plasmacytoma should be kept in mind in a patient with GI bleeding and atypical endoscopic lesions, especially those with a history of hematologic malignancy.

Extramedullary plasmacytoma presenting as a mediastinal mass.

Extramedullary plasmacytoma (EMP) is a plasma cell neoplasm without bone marrow involvement or other systemic characteristics of multiple myeloma. Few large-scale clinical studies have been conducted because of the rarity of EMP, especially when it arises from the mediastinum. Herein we report a rare case of solitary mediastinal plasmacytoma with reactive pleural effusion. A 58-year-old female presented with grade 4 dyspnea and dysphagia, with a mediastinal mass observed with on PET. CT-guided biopsy results were suggestive of IgG kappa-type EMP arising from the anterior mediastinum. The patient was treated with local radiotherapy to the mediastinum, and had clinical and radiological response s were good. Radiotherapy is an effective treatment for mediastinal EMP, but a complete workup is mandatory, including PET, as the majority of such masses coexist with multiple myeloma.

Extramedullary Plasmacytoma of the Tonsil

Plasma cell tumors are a diverse group of neoplasms characterized by monoclonal proliferation of plasma cells. Extramedullary plasmacytoma (EMP) is a rare form of localized plasma cell tumor that arises most often in the head and neck region. We present an unusual case of EMP of the palatine tonsil from a tertiary care university hospital. We discuss the histopathologic and radiologic evaluation as well as treatment of EMP.

Extramedullary Plasmacytoma of the Nasopharynx Treated with Surgery and Adjuvant Radiation: Case Report and Review of the Literature

Purpose: Extramedullary plasmacytoma (EMP) is a rare neoplasm of plasma cell origin. Although the head and neck is the most common location of EMP, there are only a handful of cases reported in the nasopharynx. Design: Case report and review of the literature Results: A 50 year-old healthy male presented with a year-long history of increasing bilateral nasal congestion unresponsive to medical therapy. Nasal endoscopy revealed a large exophytic mass filling both posterior nasal cavities. Imaging demonstrated a 3X3 cm mass arising from the nasopharynx and extending into the posterior nasal cavities bilaterally. A biopsy was performed, demonstrating extramedullary plasmacytoma. The patient underwent surgical debulking followed by radiation therapy to the primary site and bilateral necks. He has not recurred to date. Conclusions: Although surgery is usually considered second line treatment for EMP of the head and neck, this case demonstrates the utility of surgical debulking in symptom palliation before definitive treatment with radiation.

Extramedullar plasmacytoma: clinicopathology, immunohistochemistry and therapeutic approach to a case with a tonsillar site

Hematol Oncol Stem Cell Ther 1(4) October 2008 hemoncstem.edmgr.com 241 Monoclonal extramedullary plasmacytoma (EMP) is a rare plasma cell tumor. The dia agnosis is based on a mass of clonal plasma cells separate from bone or bone marrow without evia dence of occult disease elsewhere. It occurs in the upper aerodigestive tract in more than 80% of patients, most often in the nasal cavity, paranasal sinuses, nasopharynx, oropharynx and larynx.1,2 Regional nodes are invaded in less than 10% of patients.1 The disease is more coma mon in males with a peak incidence in the sixth decade. Primary EMP often presents with a mass and produces local compressive symptoms. The differential diagnosis includes benign reactive processes, carcinoma and lyma phoma. Radiotherapy (RT) is an effective modality of treatment for EMP with high local control rates (85% to 100%).1,3a6 Currently moderate dose RT (40a50 Gy) using involved fields is recommended.

Extramedullary plasmacytomas in the head and neck region

Extramedullary plasmacytoma (EMP) arises outside the bone marrow, particularly in the head and neck region (nasopharynx, nose cavity, sinuses, and tonsils), and can be associated with multiple myeloma (MM). Three cases of EMP in the head and neck region are described: a first case describes an EMP of the subglottis 3 years after treatment of MM, a second case of an EMP solitary in the middle ear presenting as a jugular foramen syndrome, and a third case of an EMP localised at the epiglottis, recurring at the floor of the nose cavity. Treatment of each EMP was surgical. We reviewed literature about aetiology, clinical course, diagnostics, treatment and prognosis. Important presenting symptoms vary from epistaxis, rhinorrhoea, a sore throat, dysphonia to haemoptoea. Association with MM must be confirmed or excluded. Histopathological examination, with immunological staining or flow cytometry confirms the diagnosis. CT and MRI are useful in staging EMP. The treatment of EMP is surgery and/or radiotherapy. The prognosis depends on tumour size (>5 cm) and nodal involvement. The 10-year survival rate is 50-80%.

Successful treatment of extramedullary gastric plasmacytoma with the combination of bortezomib and dexamethasone: First reported case

We report a case of a 68-year-old man presented with upper-gastrointestinal bleeding. Endoscopy showed a large ulcerated gastric mass. Histological examination of the gastric biopsies revealed a k monoclonal extramedullary plasmacytoma (EMP). Further staging was negative for multiple myeloma. The patient was managed with bortezomib at a dose of 1.3 mg/m 2 on days 1, 4, 8 and 11 of a 21-day cycle in combination with dexamethasone 20 mg p.o. on days 1, 2, 4, 5, 8, 9 and 11, 12 of each cycle. After 4 cycles of treatment, no endoscopic or histological findings of EMP were found. Thirteen months after diagnosis the patient is in complete remission with no evidence of local relapse or evolution to multiple myeloma. This is the first reported case of EMP successfully managed with the combination of bortezomib and dexamethasone.

Extramedullary plasmacytoma

Extramedullary plasmacytoma (EMP) is a rare entity belonging to the category of non-Hodgkin lymphoma. EMPs make up 4% of all plasma cell tumors and occur mainly in the upper aerodigestive tract (UAD). Seven patients with EMP included in this evaluation were under the authors' care and have been clinically followed since 1990. Because there are no general guidelines for the treatment of patients with EMP, the authors tried to obtain detailed data about the occurrence of this disease and also reviewed the therapies that have been used. To do so, they evaluated all EMP cases published in the medical literature until now and included their own experience. Based on the clinical course and follow-up of their own EMP patients, the authors evaluated and reinvestigated all EMP cases cited in MEDLINE, Index Medicus, DIMDI (Deutsches Institut fur medizinische Dokumentation und Information, Cologne, Germany), and the reference lists of the publications found through these sources. In a detailed literature search, more than 400 publications between 1905 and 1997 were found, and these revealed that EMP mainly occurs between the fourth and seventh decades of life. Seven hundred fourteen cases (82.2%) were found in the UAD, and 155 cases (17.8%) were found in other body regions. The following therapeutic strategies were used to treat patients with EMP of the UAD: radiation therapy alone in 44.3%, combined therapy (surgery and radiation) in 26.9%, and surgery alone in 21.9%. The median overall survival or recurrence free survival was longer than 300 months for patients who underwent combined intervention (surgery and radiation). This result was statistically highly significant (P = 0.0027, log rank test) compared with the results for patients who underwent surgical intervention alone (median survival time, 156 months) or radiation therapy alone (median survival time, 144 months). In most cases of non-UAD EMP, surgery was performed (surgery alone, 55.6%; surgery and radiation combined, 19.8%; radiation alone, 11.1%), but there were no statistical differences in survival (P = 0.62). Overall, after treatment for EMP in the UAD, 61.1% of all patients had no recurrence or conversion to systemic involvement (i.e., multiple myeloma, MM); however, 22.0% had recurrence of EMP, and 16.1% had conversion to MM. After treatment for EMP in non-UAD areas, 64.7% of all patients had no recurrence or MM, 21.2% had recurrence, and 14.1% had conversion to MM. The current investigation provides evidence that surgery alone gives the best results in cases of EMP of the UAD when resectability is good. However, if complete surgical tumor resection is doubtful or impossible and/or if lymph node areas are affected, then combined therapy (surgery and radiation) is recommended. These results, which were obtained from retrospective studies, should be confirmed in randomized trials comparing surgery with combined radiation therapy and surgery.