Multiple primary cutaneous plasmacytoma a decade after a nasal solitary extramedullary plasmacytoma: a puzzling case.
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ABSTRACT:
SUBMITTER: Gonzalez-Calle V
PROVIDER: S-EPMC5134131 | biostudies-other | 2016 Dec
REPOSITORIES: biostudies-other
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Project description:Solitary plasmacytoma (SP) is an extremely rare form of hematologic malignancy that can be classified as solitary bone plasmacytoma or solitary extramedullary plasmacytoma. Here, we report a patient who presented with progressive shortness of breath and foreign body sensation in his throat. Fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG-PET/CT) demonstrated an abnormal FDG-avid soft tissue mass arising from the larynx involving the cricoid cartilage without additional suspicious lesions. Histology revealed an abundance of plasma cells; immunohistochemistry was positive for CD138 expression and lambda chains, and negative for CD20. Comprehensive imaging studies and panendoscopy of the ENT tract confirmed solitary disease involvement. Following additional systemic work-up, a diagnosis of extramedullary plasmacytoma was rendered. The patient underwent definitive radiotherapy using intensity-modulated radiation therapy (total dose of 46?Gy, divided in 23 fractions of 200?cGy). Serial PET/CT showed the stepwise resolution of abnormal FDG uptake and resolution of the cricoid cartilage lesion. With 22?months of follow-up, the patient remains free of disease. We describe the rare case of SP presenting as a FDG-avid hypermetabolic soft tissue mass in the cricoid cartilage, which should be considered in the differential diagnosis of laryngeal tumors. Of note, SP is radiosensitive; favorable outcome can be expected once treated with doses of 40-50?Gy. FDG PET/CT is helpful in diagnosis and response assessment for this disease.
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Project description:INTRODUCTION: Extramedullary plasmacytoma is a plasma cell tumor that grows within any of the soft tissues of the organism. Similar to all plasmacytic dyscrasia, the disease is extremely rare. AIM: This study reports a case of an extramedullary plasmacytoma of the nasal cavity and provides a literature review on the topic. CASE REPORT: A 51-year-old woman presented at our tertiary university hospital with a 6-month history of progressive nasal obstruction, predominantly to the right side, and self-limiting epistaxis. Examination revealed a large pale-reddish tumor within the right nasal cavity. Anatomopathological analysis showed features consistent with a plasmacytoma diagnosis, which was subsequently confirmed by immunohistochemical techniques. Further assessment revealed the solitary nature of the condition, consistent with extramedullary plasmacytoma. Radiotherapy was initiated, which led to partial regression of the symptoms. The tumor was surgically removed by using a mid-facial degloving approach. The patient evolved with a naso-oral fistula and underwent 3 corrective surgeries. No evidence of associated systemic disease was found after 5 years of follow-up. CONCLUSIONS: This case report, which describes a rare tumor of the nasal cavity, is expected to improve the recognition and referral of this condition by ear, nose, and throat (ENT) specialists for multidisciplinary management and long-term follow-up.
| S-EPMC4423260 | biostudies-other
Project description:Extramedullary plasmacytoma is a rare neoplasm characterized by monoclonal proliferation of plasma cells outside bone marrow. It accounts for 4% of all non-epithelial sinonasal tumors. According to the literature, radiotherapy is the standard therapy for extramedullary plasmacytoma. However, the conversion rate of extramedullary plasmacytoma to multiple myeloma is reported to be between 11 and 33% over 10 years. The highest risk of conversion is reported during the first 2 years after diagnosis, but conversion has been noted up to 15 years after diagnosis. Once conversion to multiple myeloma is complete, less than 10% of patients will survive 10 years.We present three cases of sinonasal extramedullary plasmacytoma who underwent radiotherapy: a 61-year-old white man, a 60-year-old white man, and a 37-year-old white woman. We found long-term survival with stable disease in all three cases.The management of solitary extramedullary plasmacytomas of the sinonasal tract is not well established yet. However, the possibility of recurrence and progression to multiple myeloma requires a thorough follow-up protocol. Due to the absence of a standardized protocol for these tumors, we tried to design a tailored long-term follow-up scheme.
| S-EPMC5550939 | biostudies-other
Project description:Extramedullary plasmacytomas (EMPs) are uncommon plasma cell tumors that develop in soft tissue as isolated tumors without osseous involvement while secondary lesions are associated with systemic multiple myeloma (MM). Primary extramedullary lesions are most commonly found in upper respiratory tract, gastrointestinal tract and lymph nodes. They can be found either in patients with history of MM or preceding the manifestation of a systemic disease. Orbital manifestation of the lesion is rare but conjunctival involvement is very unusual. The reported cases in the English-written literature are only five cases. Herein, we report the sixth case of primary EMP in a middle-aged adult who presented with a lesion confined to the conjunctiva, unremarkable present and past medical history, and confirmed tissue diagnosis. In addition, a review and summary of the previously reported cases in the literature is presented. We aim to attract the attention of ophthalmic surgeons to consider plasmacytoma within the differential diagnosis of a conjunctival lesion.
| S-EPMC7229271 | biostudies-literature
Project description:Solitary plasmacytoma is a rare disease characterized by a localized proliferation of neoplastic monoclonal plasma cells, without evidence of systemic disease. It can be subdivided into solitary bone plasmacytoma if the lesion originates in bone, or solitary extramedullary plasmacytoma if the lesion involves a soft tissue. The incidence of solitary bone plasmacytoma is higher than solitary extramedullary plasmacytoma. Also, the prognosis is different: even if both forms respond well to treatment, overall survival and progression-free survival of solitary bone plasmacytoma are poorer than solitary extramedullary plasmacytoma due to its higher rate of evolution in multiple myeloma. However, the recent advances in the diagnosis of multiple myeloma can better refine also the diagnosis of plasmacytoma. Flow cytometry studies and molecular analysis may reveal clonal plasma cells in the bone marrow; magnetic resonance imaging or 18 Fluorodeoxyglucose positron emission tomography could better define osteolytic bone lesions. A more explicit exclusion of possible occult systemic involvement can avoid cases of misdiagnosed multiple myeloma patients, which were previously considered solitary plasmacytoma and less treated, with an unavoidable poor prognosis. Due to the rarity of the disease, there is no uniform consensus about prognostic factors and treatment. Radiotherapy is the treatment of choice; however, some authors debate about the radiotherapy dose and the relationship with the response rate. Moreover, the role of surgery and chemotherapy is still under debate. Nevertheless, we must consider that the majority of studies include a small number of patients and analyze the efficacy of conventional chemotherapy; few cases are reported concerning the efficacy of novel agents.
| S-EPMC5584766 | biostudies-literature
Project description:Extramedullary plasmacytoma of the breast (EPB), a manifestation of multiple myeloma (MM), is very rare. It is important to recognize the imaging findings of EPB because it may be the first manifestation of relapsed MM. An 85-year-old woman presented with a lump in her right breast 4 years after the complete remission of MM. She underwent mammography and ultrasonography, which showed an oval circumscribed mass and an irregular circumscribed heterogeneous solid mass, respectively. Following ultrasound-guided vacuum-assisted breast biopsy, this lesion was confirmed to be EPB. Whole-body computed tomography showed multiple new osteolytic lesions and other multiple extramedullary lesions in addition to EPB in the right breast. The final diagnosis was relapsed MM with multiple extramedullary plasmacytoma.
| S-EPMC6406076 | biostudies-literature
Project description:INTRODUCTION: The extramedullary plasmocytoma is one of the localized forms of malignancy of the plasma cells, which has multiple myeloma main diagnosis. Its main site to the head and neck, but with a rare presentation in the larynx. OBJECTIVE: To describe a case of extramedullary plasmocytoma of the larynx, with literature review. CASE REPORT: Patient female, 49, referring to intermittent dysphonia for 01 years with progressive worsening associated with vocal fatigue and vocal effort, with reddish lesion, smooth edges fold left ventricular endoscopy. Being subjected to excisional biopsy diagnosed with extramedullary histopathological plasmocytoma. CONCLUSION: Extramedullary Plasmocytoma must be considered in the differential diagnosis of rare tumors of the larynx. It is essential after the diagnosis of multiple myeloma research and a "follow up" appropriate.
| S-EPMC4432537 | biostudies-other
Project description:BackgroundSolitary plasmacytoma of bone (SPB) is a rare plasma cell neoplasm. It arises in bone as a single locus in the absence of any plasma cell myeloma lesions. Plasma cell neoplasms intrinsically express only one immunoglobulin light chain (IgL)-kappa or lambda-and using this fact, kappa/lambda deviation is the decisive factor for diagnosis. Co-expression of both IgLs in a single tumor cell is extremely rare.Case presentationWe report a case of SPB that arose in the vertebra of a 52-year-old Japanese woman. Histologically, the resected mass showed diffuse plasma cell proliferation. Dual IgL expression was detected by flow cytometry, immunohistochemistry, and in situ hybridization (ISH) targeting IgL mRNA.ConclusionWe have presented an extremely rare case of SPB showing dual expression of kappa and lambda IgLs. This unusual case of plasma cell neoplasia might represent a possible exceptional example of failure of "IgL isotypic exclusion."
| S-EPMC8686560 | biostudies-literature
Project description:PurposeTo identify the clinical usefulness of serum M protein and to establish a rationale for regular follow-up with serum protein electrophoresis in solitary plasmacytoma.Materials and methodsSixty-nine patients with solitary plasmacytoma and solitary plasmacytoma with minimal marrow involvement according to the International Myeloma Working Group criteria were retrospectively reviewed.ResultsAt a median follow-up of 6.2 years, 5-year local control (LC), 5-year multiple myeloma-free survival (MMFS), 5-year failure-free survival (FFS), and 5-year overall survival (OS) were 82.6%, 44.1%, 41.8%, and 85.1%, respectively. Among the patients whose initial serum M protein was present or not evaluated, 37.3% of patients showed disappearance of serum M protein after various treatment. MMFS of these patients were comparable to non-secretory plasmacytoma with undetectable levels of M protein, and significantly better than patients with persistent M protein. Increase of serum M protein ≥0.1 g/dL was most predictive of treatment failure with area under the curve of 0.731.ConclusionPatients who eventually showed persistence of serum M protein after treatment showed worse MMFS and FFS compared to those whose serum M protein disappeared or who had initially non-secretory disease. The increase of serum M protein level ≥0.1 g/dL from current nadir was predictive of treatment failure. Therefore, regular follow-up with serum M protein is highly recommended especially unless the patient had initially non-secretory disease.
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Project description:Over the past 15 years, lipophobic drugs, such as bleomycin and cisplatin, have been used combined with electroporation, which promotes their uptake through the cell membrane. The present data describe general findings following electrochemotherapy and how plasmacytomas can respond to this technique. We will explain and illustrate specific outcomes during the remission process. The data presented here can be useful for researchers, veterinarians, and pet owners. Furthermore, the data could be useful for other cutaneous or oral tumors in which electrochemotherapy may be indicated. Interpretation of the data and outcomes may be found in the research article entitled "Outcome following curative-intent electrochemotherapy for extramedullary plasmocytoma in dogs - case reports ."
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