Shallis Rory M, Gale Robert P, Lazarus Hillard M, Roberts Kenneth B, Xu Mina L, Seropian Stuart E, Gore Steven D, Podoltsev Nikolai A
Section of Hematology, Department of Medicine, Yale University School of Medicine and Yale Cancer Center, New Haven, USA.
Haematology Section, Division of Experimental Medicine, Department of Medicine, Imperial College London, London, UK.
Blood Rev. 2021 May;47:100773. doi: 10.1016/j.blre.2020.100773. Epub 2020 Oct 30.
The World Health Organization classification and definition of "myeloid sarcoma" is imprecise and misleading. A more accurate term is "extramedullary acute myeloid leukemia tumor (eAML)." The pathogenesis of eAML has been associated with aberrancy of cellular adhesion molecules, chemokine receptors/ligands and RAS-MAPK/ERK signaling. eAML can present with or without synchronous or metachronous intramedullary acute myeloid leukemia (AML) so a bone marrow evaluation is always recommended. Accurate diagnosis of eAML requires tissue biopsy. eAML confined to one or a few sites is frequently treated with local therapy such as radiotherapy. About 75-90% of patients with isolated eAML will develop metachronous intramedullary AML with a median latency period ranging from 4 to 12 months; thus, patients with isolated eAML may also be treated with systemic anti-leukemia therapy. eAML does not appear to have an independent prognostic impact; selection of post-remission therapy including allogeneic hematopoietic cell transplant (alloHCT) is typically guided by intramedullary disease risk. Management of isolated eAML should be individualized based on patient characteristics as well as eAML location and cytogenetic/molecular features. The role of PET/CT in eAML is also currently being elucidated. Improving outcomes of patients with eAML requires further knowledge of its etiology and mechanism(s) as well as therapeutic approaches beyond conventional chemotherapy, ideally in the context of controlled trials.
世界卫生组织对“髓系肉瘤”的分类和定义不精确且具有误导性。一个更准确的术语是“髓外急性髓系白血病肿瘤（eAML）”。eAML的发病机制与细胞粘附分子、趋化因子受体/配体以及RAS-MAPK/ERK信号传导异常有关。eAML可伴有或不伴有同步或异时性髓内急性髓系白血病（AML），因此始终建议进行骨髓评估。eAML的准确诊断需要组织活检。局限于一个或几个部位的eAML通常采用局部治疗，如放疗。约75-90%的孤立性eAML患者会发生异时性髓内AML，中位潜伏期为4至12个月；因此，孤立性eAML患者也可采用全身抗白血病治疗。eAML似乎没有独立的预后影响；缓解后治疗的选择，包括异基因造血细胞移植（alloHCT），通常由髓内疾病风险指导。孤立性eAML的管理应根据患者特征以及eAML的位置和细胞遗传学/分子特征进行个体化。PET/CT在eAML中的作用目前也正在阐明。改善eAML患者的预后需要进一步了解其病因和机制，以及超越传统化疗的治疗方法，理想情况下是在对照试验的背景下。
