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骨髓肉瘤:概述。

Myeloid sarcoma: An overview.

机构信息

North Bristol NHS Trust, Southmead Hospital, Pathology Sciences Building, Westbury on Trym, Bristol BS10 5NB, UK.

UT Southwestern Medical Center, Dallas, TX, USA.

出版信息

Semin Diagn Pathol. 2023 May;40(3):129-139. doi: 10.1053/j.semdp.2023.04.009. Epub 2023 Apr 17.

Abstract

Myeloid Sarcoma (MS) is a high grade, hematological malignancy defined as an extramedullary tumor mass of myeloid blasts with or without maturation that effaces tissue architecture. It is a highly heterogenous condition that represents a variety of myeloid neoplasms. This heterogeneity of MS, together with its rarity, have greatly hampered our understanding of the condition. Diagnosis requires tumor biopsy, which should be accompanied by bone marrow evaluation for medullary disease. It is presently recommended that MS be treated similar to AML. Additionally, ablative radiotherapy and novel targeted therapies may also be beneficial. Genetic profiling has identified recurrent genetic abnormalities including gene mutations associated with MS, supporting its etiology similar to AML. However, the mechanisms by which MS homes to specific organs is unclear. This review provides an overview of pathogenesis, pathological and genetic findings, treatment, and prognosis. Improving the management and outcomes of MS patients requires a better understanding of its pathogenesis and its response to various therapeutic approaches.

摘要

骨髓肉瘤(MS)是一种高级别的血液恶性肿瘤,定义为具有或不具有成熟度的髓性原始细胞的髓外肿瘤块,它会破坏组织结构。它是一种高度异质性的疾病,代表了各种髓性肿瘤。MS 的这种异质性,加上其罕见性,极大地阻碍了我们对这种疾病的理解。诊断需要肿瘤活检,并且应该伴随着骨髓中髓性疾病的评估。目前建议将 MS 治疗方法与 AML 相似。此外,消融性放疗和新型靶向治疗也可能有益。基因谱分析已经确定了复发性遗传异常,包括与 MS 相关的基因突变,这支持了其与 AML 相似的病因。然而,MS 归巢到特定器官的机制尚不清楚。这篇综述提供了发病机制、病理和遗传发现、治疗和预后的概述。改善 MS 患者的管理和结果需要更好地了解其发病机制及其对各种治疗方法的反应。

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