Zorn Kristin E, Cunningham Ashley M, Meyer Alison E, Carlson Karen Sue, Rao Sridhar
Department of Pediatrics, Division of Hematology/Oncology/Transplantation, Medical College of Wisconsin, Milwaukee, WI 53226, USA.
Versiti Blood Research Institute, Milwaukee, WI 53226, USA.
Cancers (Basel). 2023 Feb 24;15(5):1443. doi: 10.3390/cancers15051443.
Myeloid sarcomas (MS), commonly referred to as chloromas, are extramedullary tumors of acute myeloid leukemia (AML) with varying incidence and influence on outcomes. Pediatric MS has both a higher incidence and unique clinical presentation, cytogenetic profile, and set of risk factors compared to adult patients. Optimal treatment remains undefined, yet allogeneic hematopoietic stem cell transplantation (allo-HSCT) and epigenetic reprogramming in children are potential therapies. Importantly, the biology of MS development is poorly understood; however, cell-cell interactions, epigenetic dysregulation, cytokine signaling, and angiogenesis all appear to play key roles. This review describes pediatric-specific MS literature and the current state of knowledge about the biological determinants that drive MS development. While the significance of MS remains controversial, the pediatric experience provides an opportunity to investigate mechanisms of disease development to improve patient outcomes. This brings the hope of better understanding MS as a distinct disease entity deserving directed therapeutic approaches.
髓系肉瘤（MS），通常称为绿色瘤，是急性髓系白血病（AML）的髓外肿瘤，其发病率各异，对预后的影响也不同。与成年患者相比，儿童MS的发病率更高，临床表现、细胞遗传学特征及危险因素也独具特点。最佳治疗方案尚未明确，但异基因造血干细胞移植（allo-HSCT）和儿童的表观遗传重编程是潜在的治疗方法。重要的是，MS发生的生物学机制尚不清楚；然而，细胞间相互作用、表观遗传失调、细胞因子信号传导和血管生成似乎都起着关键作用。本综述描述了儿童特有的MS文献以及关于驱动MS发生的生物学决定因素的当前知识状态。虽然MS的重要性仍存在争议，但儿童患者的经验为研究疾病发生机制以改善患者预后提供了机会。这带来了更好地将MS理解为一种值得采用针对性治疗方法的独特疾病实体的希望。
