Plasmocytoma

浆细胞瘤
  • 文章类型: Case Reports
    背景:浆细胞瘤是一种罕见的血液系统恶性肿瘤,其预后比多发性骨髓瘤更好。该病例报告集中在颅骨交界处(CVJ)的独特孤立性骨浆细胞瘤(SBP),通过手术和放疗管理。
    方法:一名50岁患者表现为4个月的颈部疼痛和颈臂神经痛恶化。尽管有治疗和物理治疗,症状持续存在。临床检查显示颈部僵硬,没有运动或感觉缺陷。X线片和MRI显示浸润轴病变,无不稳定征象。CT引导下的活检结果不确定。为了解决不稳定性并建立诊断,对C2后弓进行了刮宫活检,然后使用a骨移植物进行枕骨C4融合。组织学检查证实SBP。给予辅助放疗和化疗。在四年的随访中,没有多发性骨髓瘤的进展,但是据报道颈部活动受限,在成像上观察到稳定的融合。
    结论:孤立性骨浆细胞瘤主要影响轴向骨骼,罕见的上颈椎受累。诊断标准包括组织学确认,正常骨髓分析,无异常成像(原发病灶除外),并且没有与淋巴浆细胞增生性疾病相关的终末器官损伤。临床表现是非特异性的,MRI对软组织评估有价值。放射治疗是主要的治疗方法,保留特定适应症的手术。
    结论:孤立性骨浆细胞瘤是一种罕见的疾病,如果及时治疗,预后良好。该病例强调了早期诊断和治疗以预防复发或多发性骨髓瘤进展的重要性。多学科方法,包括必要时的手术,对于最佳结果至关重要。
    BACKGROUND: Plasmacytoma is a rare hematological malignancy with a more favorable prognosis than multiple myeloma. This case report focuses on a unique solitary bone plasmacytoma (SBP) at the craniovertebral junction (CVJ), managed through surgery and radiotherapy.
    METHODS: A 50-year-old patient presented with four months of worsening neck pain and cervicobrachial neuralgia. Despite treatment and physiotherapy, symptoms persisted. Clinical examination revealed neck stiffness, with no motor or sensory deficits. Radiographs and MRI showed an infiltrating axis lesion without instability signs. A CT-guided biopsy yielded inconclusive results. To address instability and establish a diagnosis, a curettage biopsy of the C2 posterior arch was performed, followed by occipito-C4 fusion using an iliac crest graft. Histological examination confirmed SBP. Adjuvant radiotherapy and chemotherapy were administered. At four-year follow-up, there was no multiple myeloma progression, but limited neck mobility were reported, with stable fusion observed on imaging.
    CONCLUSIONS: Solitary bone plasmacytoma primarily affects the axial skeleton, with rare upper cervical spine involvement. Diagnostic criteria include histological confirmation, normal bone marrow analysis, unremarkable imaging (except for the primary lesion), and absence of end-organ damage related to lymphoplasmacytic proliferative disorders. Clinical presentation is nonspecific, and MRI is valuable for soft tissue assessment. Radiotherapy is the primary treatment, with surgery reserved for specific indications.
    CONCLUSIONS: Solitary bone plasmacytoma is a rare condition with a favorable prognosis when promptly managed. This case underscores the importance of early diagnosis and treatment to prevent recurrence or multiple myeloma progression. A multidisciplinary approach, including surgery when necessary, is crucial for optimal outcomes.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: English Abstract
    未经证实:由于缺乏有关单克隆丙种球蛋白的信息,我们的主要研究结果是描述流行病学,Souss-Massa地区单克隆丙种球蛋白的临床和生化特征,在摩洛哥南部。
    未经评估:我们进行了一项回顾性研究,只选择完整的医疗记录。我们使用了在当地肿瘤中心诊断为单克隆丙种球蛋白病的患者超过10年的记录。
    未经评估:这项研究纳入了117名患者,男性占比很高(65%),男女性别比为1.85。我们研究人群的平均年龄为61.44(ET14.54)岁。诊断(基于频率)包括:多发性骨髓瘤82.0%(n=96),孤立性浆细胞瘤8.5%(n=10),意义不明的单克隆丙种球蛋白2.6%(n=3),淋巴瘤2.5%(n=3),继发性浆细胞白血病1.7%(n=2),Waldenström病1.7%(n=2)和慢性淋巴样白血病(n=1)。同种型分布如下:IgGκ33.7%(n=28),IgGλ21.7%(n=18),IgAκ12.0%(n=10),IgAλ7.2%(n=6),IgMκ3.6%(n=3),和IgDλ2.4%(n=2)。在两个案例中达到了双峰,比例为2.4%。
    UNASSIGNED:与国际研究相比,由于护理结构中无法使用电泳,因此观察到诊断延迟。
    UNASSIGNED: given the lack of information about monoclonal gammopathies, our primary study outcome was to describe the epidemiological, clinical and biochemical profiles of monoclonal gammopathies in the Souss-Massa region, in southern Morocco.
    UNASSIGNED: we conducted a retrospective study, by selecting only complete medical records. We used records of patients diagnosed with monoclonal gammopathy at the local oncology center during a period of over 10 years.
    UNASSIGNED: one hundred and seventeen patients were included in the study, with a high male predominance (65%) and a male/female sex-ratio of 1.85. The average age of our study population was 61.44 (ET 14.54) years. Diagnoses (based on frequency) included: multiple myeloma 82.0% (n=96), solitary plasmacytoma 8.5% (n=10), monoclonal gammopathies of undetermined significance 2.6% (n=3), lymphoma 2.5% (n=3), secondary plasma cell leukaemia 1.7% (n=2), Waldenström´s disease 1.7%(n=2) and chronic lymphoid leukemia (n=1). The isotype distribution was as follows: IgG Kappa 33.7% (n=28), IgG lambda 21.7% (n=18), IgA Kappa 12.0% (n=10), IgA lambda 7.2% (n=6), IgM kappa 3.6% (n=3), and IgD lambda 2.4% (n=2). Biconal peak was reached in two cases, with a percentage of 2.4%.
    UNASSIGNED: diagnostic delay was observed compared to international studies due to the unavailability of electrophoresis in the care structures.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Case Reports
    POEMS综合征是一种罕见的副肿瘤起源疾病,其特征是存在与血浆单克隆细胞增殖性疾病和血管内皮生长因子过度产生相关的感觉运动多神经病。首字母缩写“POEMS”代表多系统发现,包括多发性神经病,器官肿大,内分泌病,单克隆浆细胞疾病和皮肤变化;尽管如此,临床表现是异质的。我们描述了一个临床病例,诊断和治疗方法在患有感觉运动多神经病的患者中被诊断为POEMS综合征;了解这种病理,其临床和临床旁表现,以便做出诊断或避免延迟诊断并提供适当的治疗。
    POEMS syndrome is a rare condition of paraneoplasic origin characterized by the presence of a sensorimotor polyneuropathy associated with the presence of a proliferative disorder of plasmatic monoclonal cells and overproduction of vascular endothelial growth factor. The acronym \"POEMS\" represents multisystem findings including polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder and skin changes; nevertheless, clinical presentation is heterogeneous. We describe a clinical case, the diagnostic and therapeutic approach in a patient with sensorimotor polyneuropathy in whom POEMS syndrome was diagnosed; to understand this pathology, its clinical and paraclinical manifestations in order to make a diagnosis or to avoid a delayed one and to provide an adequate treatment.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

    求助全文

  • 文章类型: Case Reports
    BACKGROUND: Multiple myeloma is a malignant hematological disease characterized by proliferation of monoclonal plasma cells mainly in the bone marrow. Extraosseous epidural plasmacytoma associated with myeloma arises from lymphoid tissue in the epidural space without focal vertebral involvement, and is rare.
    METHODS: A 52-year-old woman was diagnosed with kappa subtype nonsecretory multiple myeloma and presented with bilateral arm weakness 11 mo after completing multiple courses of chemotherapy. Spinal magnetic resonance imaging (MRI) showed a posterior C7-T3 epidural mass with spinal cord compression. After five courses of chemotherapy, follow-up MRI showed resolution of cord compression. A 54-year-old man presented with paraplegia 15 mo after a diagnosis of IgD kappa subtype multiple myeloma and completing multiple courses of chemotherapy. He underwent Th11 and L1 laminectomies for tumor resection because MRI showed an epidural mass causing cord compression. His-topathologic examination was consistent with IgD multiple myeloma. The patients have currently survived for 33 mo and 19 mo, respectively.
    CONCLUSIONS: Isolated extraosseous epidural plasmacytoma associated with multiple myeloma without bony involvement is difficult to diagnose by imaging. Definitive diagnosis requires pathological and immunohistochemical examination.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Journal Article
    BACKGROUND: Patients 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: Between 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.
    RESULTS: A 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).
    CONCLUSIONS: RT 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.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Sci-hub)

       PDF(Pubmed)

  • 文章类型: Journal Article
    Bone marrow angiogenesis plays an important role in the pathogenesis and progression of hematological malignancies. It is well known that tumor microenvironment promotes tumor angiogenesis, proliferation, invasion, and metastasis, and also mediates mechanisms of therapeutic resistance. An increased number of mast cells has been demonstrated in angiogenesis associated with hematological tumors. In this review we focused on the role of mast cells in angiogenesis in human plasma cell malignancies. In this context, mast cells might act as a new target for the adjuvant treatment of these tumors through the selective inhibition of angiogenesis, tissue remodeling and tumor-promoting molecules, permitting the secretion of cytotoxic cytokines and preventing mast cell-mediated immune suppression.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Sci-hub)

       PDF(Pubmed)

  • 文章类型: Case Reports
    Plasmacytoma of the skull-base is a rare entity. Differential diagnosis includes chordoma, osteosarcoma, carcinoma nasopharynx, meningioma, metastatic carcinoma, lymphoma, and multiple myeloma. Accurate and precise diagnosis is extremely important for plasmacytoma of the skull-base as its treatment and prognosis is different from other skull-base lesions. A 41-year-old man presented with concerns of headache, diplopia, and left eye strabismus. A magnetic resonance image (MRI) of his brain showed a large expansile mass measuring 51 mm involving the clivus and central skull-base. Trans-sphenoidal tumor decompression was done. A biopsy confirmed the plasmacytoma. A positron emission tomography-computed tomography (PET-CT) scan showed a single 2-(18F) fluoro-D-glucose (FDG) avid lesion at the skull-base. The results of all other relevant investigations such as hemoglobin, renal function test, serum calcium, serum protein immunoelectrophoresis, serum quantitative immunoglobulin, bone marrow biopsy, serum lactate dehydrogenase, and beta-2 microglobulin levels were within normal limits. He was treated with radical radiotherapy. He developed complete clinical response after radiotherapy.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Sci-hub)

       PDF(Pubmed)

  • 文章类型: Journal Article
    暂无摘要。
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Sci-hub)

  • 文章类型: Case Reports
    Presentation of multiple myeloma with bone lesions is common. It is vital that differentiation between nonsecretory multiple myeloma and plasmocytoma be done and that each disorder be treated accordingly. In this paper, we present a patient with nonsecretory multiple myeloma, who suffered from distal humerus fracture with severe bone destruction, renal failure, hypercalcemia and anemia.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Sci-hub)

  • 文章类型: Case Reports
    Multiple myeloma is a malignant tumour characterized by proliferation of a single clone of plasma cells, this cell line will produce large amount of ineffective immunoglobulins that are ineffective at fighting infection resulting in immunosuppression. These are medullary tumours most of the time; however, in rare cases they may arise extra medullary. The incidence of extramedullary plasmocytoma is about 5% and they arise in the chest most of the time; but they can also arise in other body systems like gastrointestinal system, which is involved in 10% of the time. We present a very rare case of primary plasmocytoma involving the pancreas. According to our research, there are only 25 cases of primary pancreatic plasmocytoma reported in english literature.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Sci-hub)

公众号