Case report: An uncommon presentation of extramedullary plasmacytoma without a concurrent diagnosis of multiple myeloma.

Introduction: Extramedullary plasmacytoma (EMP) is an uncommon solitary tumor originating from neoplastic plasma cells located outside the bone marrow. Despite its rarity, the occurrence of EMP without a concurrent diagnosis of multiple myeloma (MM) is considered extremely rare. Approximately 80-90% of EMP cases are found in the head and neck region, with a higher incidence in men aged between 50 and 60 years. The current treatment modalities include radiotherapy (RT) as a first-line approach, with surgery or chemotherapy regarded as other therapeutic options. While RT proves effective in the majority of EMP cases, there are instances where the tumor remains refractory to radiation. In this case report, we present an unusual scenario of EMP resistant to RT without concurrent signs of multiple myeloma which was successfully treated with surgery followed by systemic therapy. Case report: A 72-year-old male was admitted to the Head and Neck Cancer Clinic with a 6-month history of swallowing difficulties. He denied experiencing weight loss or pain on swallowing. Basic laboratory tests yielded results within normal limits, except for beta-2 microglobulin. Physical examination revealed an enlarged submandibular lymph node on the right side. Fiberoptic examination identified a soft tissue polypoid mass within the right piriform fossa, slightly protruding into the vocal slit. A CT scan displayed a well-circumscribed 2 cm polypoid, homogeneously enhancing soft tissue mass adjacent to the posterior surface of the epiglottis and the right side of the tongue base. Bone marrow biopsy revealed no abnormalities, and there were no clinical or laboratory signs of multiple myeloma. Based on the tumor biopsy results and imaging studies, a diagnosis of EMP was made. Due to the lack of response to RT, surgical removal of the tumor was pursued, followed by systemic therapy. Ultimately, the patient achieved full recovery with effective disease control. Conclusion: In conclusion, EMP without concurrent multiple myeloma is an exceedingly rare condition that demands a multidisciplinary approach for both diagnosis and treatment. Moreover, although RT continues to be the primary standard treatment for EMP, in some cases other therapeutic regimens prove to be successful.

[Multiple myeloma with IgH::MYC and multiple extramedullary lesions].

A 41-year-old woman with right shoulder pain was found to have multiple tumors with osteolysis and M-proteinemia. Abnormal plasma cells (CD38+, CD138+, Igλ≫κ) were detected in 1.4% of bone marrow nucleated cells, and G-banding analysis revealed a 46,XX,t (8;14), (q24;q32) karyotype in 4 of 20 cells analyzed. A biopsy specimen from an extramedullary lesion had a packed proliferation of aberrant plasmacytoid cells with positive IgH::MYC fusion signals on fluorescence in situ hybridization. The patient was diagnosed with symptomatic multiple myeloma and treated with the BLd regimen, which significantly reduced M protein levels. Extramedullary lesions were initially reduced, but increased again after four cycles. The lesions disappeared with subsequent EPOCH chemotherapy and radiation, and complete remission was confirmed. The patient was then treated with high-dose chemotherapy with autologous peripheral blood stem cell transplantation. Complete remission was maintained for over one year with lenalidomide maintenance therapy. A solitary IgH::MYC chromosomal translocation is extremely rare in multiple myeloma and may be associated with high tumor proliferative capacity, multiple extramedullary lesions, and poor prognosis. Combined therapeutic modalities with novel and conventional chemotherapy and radiation might be a promising treatment strategy for patients with this type of multiple myeloma.

Melan-A immunolabeling in canine extramedullary plasmacytomas.

Histologic diagnosis of less well-differentiated cases of canine extramedullary plasmacytomas (CEMPs) may require immunohistochemical confirmation to discriminate these tumors from other round cells tumors including lymphoma, cutaneous histiocytoma, and amelanotic melanomas. CEMPs are characterized by widespread immunoreactivity for multiple myeloma 1 (MUM1) antigen and λ light chains, while the melanocytic marker melan-A has been reported to yield negative results. Here, 33 randomly selected CEMPs, 20 melanocytomas, and 20 malignant melanomas were immunohistochemically tested for MUM1, melan-A, and PNL2. In addition, CEMPs were examined for PAX5, E-cadherin, CD3, CD18, CD20, S100, as well as λ and κ light chain immunoreactivity. All CEMPs were characterized by labeling for MUM1 and λ light chain, as well as variable immunopositivity for the remaining antibodies. Notably, 13 cases of CEMPs (39.4%) exhibited immunolabeling for melan-A. Melanocytic tumors immunolabeled for melan-A (40/40; 100%) and PNL2 (34/40; 85%). An unexpected cytoplasmic immunoreactivity for MUM1 was observed in 2 melanocytic tumors. Summarized, MUM1 or melan-A immunomarkers alone are not sufficient to differentiate between CEMPs and amelanotic melanomas and should be part of a larger immunopanel including λ light chain, CD20, and PNL2.

A Plasmacytoma of the Lateral Pharyngeal Wall: A Diagnostic Enigma.

Plasmacytoma is a rare plasma cell dyscrasia that grows in bones or soft tissues such as the pharynx. Soft tissue plasmacytomas are rare, and a higher burden has been reported in the upper aero-digestive tract, often manifesting as hoarseness, dysphagia, or odynophagia. Due to their rarity, extramedullary plasmacytomas (EMPs) have unknown prognostic factors, and guidelines for optimal management are still lacking. However, radiation therapy and surgery have been used with positive outcomes. Herein, we describe a unique case of plasmacytoma of the pharyngeal tissue in a male patient with a history of HIV disease. The patient completed 28 sessions of radiation therapy, resulting in an improvement in his throat pain and hoarseness. Given the patient's age and lack of traditional risk factors for head and neck cancers, his hoarseness and odynophagia proved to be a diagnostic conundrum. Although infrequent, soft tissue plasmacytomas should be considered in the differential diagnosis of head and neck tumors.

Superior Oblique Muscle Extramedullary Plasmacytoma in a Patient with Multiple Myeloma and a Review of Literature.

Introduction: Multiple myeloma (MM), a plasma cell malignancy, is a systemic disease affecting various body organs. Plasmacytoma of bone and extramedullary disease (EMD) are presentations of MM. EMD is usually the sign of a more aggressive form of the disease. Herein, we report a patient with refractory MM presenting with extramedullary plasmacytoma in the superior oblique (SO) muscle. Case Presentation: A 51-year-old female presented complaining of gradual protrusion of the left eye and ocular pain from 20 days prior. She received bone marrow transplantation 1 year prior and was on a chemotherapy regimen for MM for the past 1 year. Ocular examination revealed proptosis of the left eye and mild limitations of adduction and elevation. Orbital magnetic resonance imaging demonstrated remarkable enlargement of the left SO muscle with focal contrast enhancement. The patient underwent a biopsy and mass debulking. The histopathologic exam revealed fibromuscular tissue containing a neoplasm composed of sheets of plasmacytoid cells in a varying degree of differentiation with intervening scantly vascularized stromal components. The plasmacytoid cells were diffusely positive for a cluster of differentiation 138 (CD138), leading to a diagnosis of EMD involving the EOM and soft tissue of the orbit. The patient underwent palliative radiotherapy and a systemic workup. The PET-CT scan revealed involvement of the pelvic bone and left calf. Accordingly, the chemotherapy regimen was upgraded to reflect the aggressive nature of the disease. In the last follow-up, there was no sign of tumor reactivation in the orbital soft tissues. Unfortunately, the patient succumbed to her illness 7 months following her most recent presentation. Conclusion: Early recognition of disease recurrence is lifesaving in MM patients; ophthalmic manifestations should be seriously considered as a sign of MM activity.

Recurrence of solitary plasmacytoma in the liver 10 years after the onset of multiple bone lesions.

A 79-year-old man presented with a history of solitary plasmacytoma in the bone 10 years ago. Chemoradiotherapy was effective, and remission was maintained with intermittent treatment at relapse of the bone lesions. One year after the last treatment, a follow-up computed tomography (CT) scan revealed multiple liver masses, and a liver biopsy revealed plasmacytoma. There was no clonal plasma cell infiltration in the bone marrow, and the final diagnosis was solitary plasmacytomas of the liver. Although liver involvement is known in relapsed refractory multiple myeloma, solitary plasmacytoma in the relapsed stage confined to the liver is rare, and all previous reports have been from the initial presentation. To the best of our knowledge, this is the first recurrent case of solitary plasmacytoma of the liver.

Spinal Solitary Plasmacytoma With Minimal Marrow Involvement Presenting With Epidural Spinal Cord Compression.

Solitary plasmacytoma (SPC) is a rare type of plasma cell dyscrasia characterized by the proliferation of neoplastic monoclonal plasma cells. It can involve bone or soft tissue without signs of systemic disease. The solitary bone plasmacytoma typically involves the axial skeleton, most commonly the vertebrae. This article presents a 58-year-old male with a history of Parkinson's disease, hypertension, and cervical spine degenerative joint disease. He arrived at the emergency department with severe thoracic and lumbar back pain, accompanied by numbness and weakness in both legs, which worsened with movement and deep breathing. Magnetic resonance imaging (MRI) findings revealed a sizable mass in the T11 vertebra, leading to thoracic spinal cord compression. Treatment included high-dose dexamethasone, and surgical intervention was undertaken. Subsequent pathology confirmed plasma cell dyscrasia. Radiotherapy and chemotherapy (lenalidomide and dexamethasone) were administered, resulting in no recurrence or new masses after two years. Solitary plasmacytoma is a rare disease with limited clinical trials due to the inability to accrue larger cohorts. Prompt diagnosis and staging of plasmacytomas, involving robust histopathological and radiographic methods, are needed to prevent further complications and possible progression to multiple myeloma. Radiation therapy is the primary treatment, with some studies showing the benefits of lenalidomide and dexamethasone. Further studies are needed to improve treatment options for these patients. This case report adds to the current literature the importance of a multidisciplinary approach to the treatment of SPC.

Solitary Extramedullary Plasmacytoma of the Left Lower Eyelid: A Case Report and Review of Literature.

Solitary extramedullary plasmacytoma (SEP) is a collection of plasma cells in soft tissue tumors characterized by monoclonal plasma cells without systemic symptoms or evidence of bone disease. We present a case of SEP in a 49-year-old African American patient who presented with a slowly enlarging eyelid mass and underwent an excisional biopsy with ophthalmology before the diagnosis was confirmed by pathology in the absence of systemic symptoms or bone disease. Our review found only six confirmed cases of SEP of the eyelid described in the literature. In such cases, treatment is typically surgical excision or radiotherapy. Our patient was treated with radiation after the excision was incomplete. This case report adds another rare case of SEP of the eyelid to the literature.

Extramedullary Plasmacytoma of the Penis as a First Manifestation of Multiple Myeloma: A Case Report.

INTRODUCTION: Plasmacytoma is a rare plasma-cell neoplasm, which includes bone and extramedullary types. While most cases occur in the head and neck, our report presents an unusual case of extramedullary plasmacytoma (EMP) in the penis, emphasizing the diverse locations of this condition. CASE PRESENTATION: An 88-year-old man, post-hydrocelectomy, presented with a palpable penile mass causing urinary symptoms. CT scans revealed a tumor with extracapsular spread and potential urethral involvement. Biopsy confirmed lymphoma, later identified as extramedullary plasmacytoma. A follow-up whole-body CT scan was performed, revealing multiple areas of bone rarefaction of the dens of the axis. His diagnosis has been further specified as multiple myeloma. Treatment with lenalidomide, bortezomib, and dexamethasone led to significant penile tumor reduction and improved voiding symptoms after three cycles. CONCLUSION: A rare case of primary EMP in the penis is reported, with only two documented cases of EMP in this location. The etiology of EMP remains unclear, possibly linked to chronic infection, irritation, or inflammation. EMP typically occurs in soft tissues, commonly in the head and neck, presenting as submucosal masses with symptoms in individuals aged 50-70. Diagnosis requires demonstrating monoclonal plasma cell infiltration and excluding multiple myeloma. While EMPs are often treated with radiotherapy, a patient with bone rarefaction suggestive of multiple myeloma requires first-line chemotherapy. This case highlights the importance of recognizing myeloma-defining events for appropriate treatment.

A prognostic nomogram and risk classification system of elderly patients with extraosseous plasmacytoma: a SEER database analysis.

BACKGROUND: The survival trends and prognostic factors of patients with extraosseous plasmacytoma (EOP) or extramedullary plasmacytoma (EMP) have not been reported in recent years. The objective of this study was to develop a novel nomogram and risk stratification system for predicting the overall survival (OS) of elderly patients with EOP based on the Surveillance, Epidemiology, and End Results (SEER) database. METHODS: The demographic characteristics of 900 patients aged 60 years and above, diagnosed with EOP between 2000 and 2019, were extracted from the SEER database. The patient population was randomly divided into a training cohort and an internal validation cohort in a ratio of 7:3. Univariate and multivariate Cox regression analyses were conducted to identify independent predictors of prognosis in elderly EOP patients, followed by developing a nomogram for prognostic assessment. The performance of the model was evaluated through receiver-operating characteristic (ROC) curves, C-index, calibration curves for calibration accuracy assessment, and decision curve analysis (DCA) to assess its clinical utility. All elderly EOP patients were stratified into three risk subgroups by cutoff value utilizing X-tile software based on their total OS scores for comparative analysis purposes. Kaplan-Meier (K-M) survival curve analysis was employed to validate any observed differences in OS among these three risk groups. RESULTS: Six factors including age, year of diagnosis, marital status, primary site, surgery, and prior tumor history were identified to be independently predictive of the OS of elderly patients with EOP, and these predictors were included in the construction of the nomogram. The 1-, 3-, and 5-year area under the curves (AUCs) for OS were 0.717, 0.754, and 0.734 in the training cohort and 0.740, 0.730, and 0.765 in the validation cohort, respectively. The C-index values in the two cohorts were 0.695 and 0.690. The calibration curves and DCA exhibit commendable consistency and validity, respectively, thereby demonstrating their robust performance. The training set was stratified into low-, medium-, and high-risk subgroups based on the optimal cutoff points (167.8 and 264.8) identified. The K-M curve and cumulative risk curve exhibited statistically significant disparities in survival rates among the groups. CONCLUSIONS: We developed a nomogram and risk classification system, which can serve as an intuitive and effective tool for clinicians to enhance the prediction of OS in elderly EOP patients, thereby facilitating the formulation of more rational and personalized treatment strategies.

An Unusual Presentation of Extramedullary Plasmacytoma in the Orbit in a Middle-Aged Woman.

Orbital location for an extramedullary plasmacytoma is very rare. We report a case of 48 years old woman with swelling in the upper medial aspect of Right eye for 1 year. Histopathological evaluation was consistent with Plasmacytoma and IHC was strongly positive for CD 138. Her thorough evaluation for Multiple myeloma was negative.

Soft Tissue Mass Extramedullary Plasmacytoma Following Radiation Therapy for Solitary Bone Plasmacytoma.

Soft tissue involvement in extramedullary plasmacytoma (EMP) is an exceptionally rare occurrence within the spectrum of plasma cell neoplasms. This case report presents the unique scenario of a patient who developed a soft tissue mass EMP subsequent to receiving radiation therapy for a solitary bone plasmacytoma at a distinct anatomical site. The primary objective of this report is to elucidate the clinical characteristics, diagnostic complexities, and management considerations associated with this uncommon presentation. Through a comprehensive review of existing literature, we aim to provide valuable insights and expertise to healthcare providers involved in the assessment and treatment of similar cases.

A Case of Recurrent Multiple Myeloma as Testicular Plasmacytoma Without Systemic Disease.

Plasma cell neoplasms include various conditions ranging from indolent conditions such as monoclonal gammopathy of undetermined significance (MGUS) to more aggressive forms such as multiple myeloma (MM). The World Health Organization classifies plasmacytomas into two types: solitary osseous plasmacytoma (SOP) and extramedullary plasmacytoma (EMP). Most primary EMPs occur in the upper gastrointestinal tract, head and neck, upper respiratory system, central nervous system, lungs, liver, spleen, and kidneys. However, the occurrence of EMP involving the testis site is quite rare. Given the rarity of testicular plasmacytoma, there is no consensus on the standard of treatment for this diagnosis. Most EMP is radiosensitive, with few localized types responding to surgical intervention. Tumor recurrence and disseminated infiltration are treated with adjuvant chemotherapy after radiation or surgery. Our patient has a unique presentation of an individual who developed recurrent myeloma of the testis 12 years after his initial diagnosis of myeloma.

Solitary Extramedullary Plasmacytoma of the Head and Neck: A Report of Three Cases Treated With Curative Radiotherapy and a Review of the Dose-Control Relationship.

Solitary plasmacytoma is an uncommon hematologic malignancy characterized by the monoclonal proliferation of abnormal plasma cells in the bone or extramedullary tissues and the absence of other multiple myeloma-defining clinical characteristics. Mostly, solitary extramedullary plasmacytoma (SEP) occurs in the head and neck region, also called solitary extramedullary plasmacytoma of the head and neck (SEPHN). Although the standard of care for SEPHN is not well established, either a surgical approach or localized external beam radiotherapy (EBRT) can be used as a definitive treatment. Due to the high radiosensitivity of SEPHN, EBRT has been associated with adequate therapeutic effects in the management of SEP, with the advantage of being a noninvasive modality that yields high rates of local control with a reasonable toxicity profile. We present a case series of three patients with SEPHN treated with EBRT at our institution with clinical outcomes.

Plasmacytoma of the Head and Neck: Case Series and Review of the Literature.

Solitary plasmacytoma is a rare neoplasm characterized by localized proliferation of monoclonal plasma cells and is classified as solitary bone or solitary extramedullary plasmacytoma. Here, we present two rare cases of plasmacytoma of the head and neck. The first is a 78-year-old male who presented with a 3-month history of epistaxis and progressive obstruction of the right nasal passage. Computerized tomography (CT) imaging revealed a mass in the right nasal cavity with destruction to the maxillary sinus. An excisional biopsy was performed revealing anaplastic plasmacytoma. The second is a 64-year-old male with a past medical history significant for prostate cancer who presented with a 2-month history of left ear pain and progressive non-tender temporal swelling. A PET/CT revealed a highly avid, destructive, and lytic left temporal mass with no other evidence of distant disease. A left temporal craniectomy and infratemporal fossa dissection revealed plasma cell dyscrasia with monoclonal lambda in situ hybridization. Although plasmacytomas are uncommon tumors of the head and neck, they may mimic other entities that require different treatment. Prompt and accurate diagnosis is critical for appropriate therapeutic decisions and prognosis.

A Rare Case of Primary Duodenal Plasmacytoma: An Incidental Finding.

Solitary extramedullary plasmacytoma (SEP) is a rare tumor due to the monoclonal proliferation of plasma cells without bone marrow involvement. Plasmacytomas are frequently encountered in bone or soft tissue but rarely occur in the gastrointestinal (GI) tract. They can present with a multitude of symptoms depending on their site. This report describes a case of SEP diagnosed as a duodenal ulcer (DU) during esophagogastroduodenoscopy (EGD) for iron deficiency anemia.

Ovarian plasmacytoma: a case report.

Plasmacytomas are a rare spectrum of plasma cell neoplasms that are single localized tumours, lacking the clinical features of plasma cell myeloma with no radiographical evidence of additional plasma cell tumours. Two clinical variants of plasmacytomas can be distinguished: solitary plasmacytoma of bone and extramedullary (or extraosseous) plasmacytoma. The latter is rare, representing 1% of all plasma cell neoplasms, occurring most frequently in the upper airways. Ovarian localization is exceptional, with only a few cases being reported in the literature. We herein report a case of an ovarian extramedullary plasmacytoma occurring in a 56-year-old woman who consulted for abdominal pain and abdominal mass, while highlighting the main histological and immunohistochemical features of this rare malignancy, along with a thorough review of literature gathering all cases of ovarian plasmacytomas reported to date.

Solitary Extramedullary Plasmacytoma With Development of T-cell Anaplastic Large-Cell Lymphoma: A Rare Case Report and Literature Review.

Solitary extramedullary plasmacytomas are an exceedingly rare form of malignant neoplasms characterized by a single localized mass located in any soft tissue that consists of abnormal plasma cells. This type of tumor is characterized by the absence of plasmacytosis on bone marrow biopsy, the absence of other lesions on imaging, and no clinical signs of multiple myeloma. They usually present with mass effect, so the clinical picture varies based on the location of the tumor. In cases where the tumor is located in the gastrointestinal tract, patients may experience abdominal pain, small bowel obstruction, or gastrointestinal bleeding. The diagnostic process typically involves imaging to identify the tumor and its location, followed by a biopsy of the lesion with subsequent immunohistochemical analysis, as well as fluorescence in situ hybridization, and finally, bone marrow biopsy. Treatment options vary depending on the tumor's location and may include radiation therapy, surgical resection, and chemotherapy. Currently, radiation therapy is the preferred first-line treatment, with the best outcomes reported in the literature. Surgery is also frequently used and is often followed by radiation therapy. While chemotherapy has not been shown to have significant benefits, the available data is insufficient, and further studies are required to make better conclusions. Disease progression is often associated with transformation to multiple myeloma, but due to the rarity of the disease, data is limited, and it remains unclear if other forms of progression exist.   We report a case of a 63-year-old male who presented to the hospital with symptoms of abdominal pain, nausea, and vomiting. A computed tomography scan revealed a mass causing bowel obstruction, which was subsequently resected and evaluated by pathology. The final diagnosis was determined to be a solitary extramedullary plasmacytoma. Since the margins of the resected mass were clear, the patient was managed with solely clinical observation. Approximately eight months later, the patient was diagnosed with T-cell anaplastic large-cell lymphoma, ultimately leading to his passing 15 months after the initial diagnosis of solitary extramedullary plasmacytoma. We present this case to increase awareness of the rare condition of solitary extramedullary plasmacytoma and to highlight the potential association with T-cell anaplastic large-cell lymphomas, as demonstrated in this patient's case. Given the possibility of malignant transformation, close monitoring is warranted in similar cases.