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Solitary Plasmacytoma

机译:孤立性浆细胞瘤

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摘要

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.
机译:孤立性浆细胞瘤是一种罕见的疾病,特征在于肿瘤性单克隆浆细胞的局部增殖,而没有全身性疾病的迹象。如果病变起源于骨,则可细分为孤立的骨浆细胞瘤;如果病变累及软组织,则可细分为孤立的髓外浆细胞瘤。孤立性骨浆细胞瘤的发生率高于孤立性髓外浆细胞瘤。同样,预后也不同:即使两种形式对治疗的反应都很好,但由于多发性骨髓瘤的发展速度较快,孤立骨浆细胞瘤的总体生存率和无进展生存期比孤立髓外浆细胞瘤差。然而,多发性骨髓瘤的诊断的最新进展可以更好地完善浆细胞瘤的诊断。流式细胞术研究和分子分析可揭示骨髓中的克隆浆细胞。磁共振成像或18氟脱氧葡萄糖正电子发射断层扫描可以更好地定义溶骨性病变。更明确地排除可能的隐匿性系统性累及可以避免误诊为多发性骨髓瘤患者的病例,这些患者先前被认为是孤立性浆细胞瘤且治疗较少,不可避免的不良预后。由于疾病的罕见性,关于预后因素尚无统一共识和治疗。放疗是一种选择。但是,一些作者对放疗剂量及其与缓解率的关系进行了辩论。而且,手术和化学疗法的作用仍在争论中。尽管如此,我们必须考虑到大多数研究只包括了少数患者,并分析了常规化学疗法的疗效。关于新药功效的报道很少。

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