Kheirkhah Pouyan, Avila-Rodriguez Ana M, Radzik Bartlomiej, Murga-Zamalloa Carlos
Department of Pathology, University of Illinois at Chicago, USA.
Department of Internal Medicine, University of Illinois at Chicago, USA.
Mediterr J Hematol Infect Dis. 2021 Nov 1;13(1):e2021067. doi: 10.4084/MJHID.2021.067. eCollection 2021.
Myeloid sarcomas can be detected in up to 30% of acute myeloid leukemia cases or occur de-novo without bone marrow involvement. The most frequent localization of myeloid sarcomas in the abdominal cavity is the small intestine, and gastric presentations are infrequent, frequently misdiagnosed, and a high level of suspicion should exist when the characteristic histomorphology features are present. The current review features a case report with gastric presentation of myeloid sarcoma in a patient with a diagnosis of acute myeloid leukemia with trisomy 8. In addition, a review of the literature of intestinal-type myeloid sarcomas shows that less than 15% of these cases have been reported in the stomach. The most common molecular aberrancy detected in intestinal myeloid sarcomas is the fusion protein CBFB-MYH11. A review of several large studies demonstrates that the presence of myeloid sarcoma does not constitute an independent prognostic factor. The therapeutic approach will be tailored to the specific genetic abnormalities present, and systemic chemotherapy with hematopoietic stem cell transplant is the most efficient strategy.
在高达30%的急性髓系白血病病例中可检测到髓系肉瘤，或在无骨髓受累的情况下新发。腹腔内髓系肉瘤最常见的部位是小肠，胃部表现罕见，常被误诊，当出现特征性组织形态学特征时应高度怀疑。本综述报道了一例诊断为急性髓系白血病伴8号染色体三体的患者出现胃部髓系肉瘤的病例。此外，对肠道型髓系肉瘤文献的回顾表明，这些病例中不到15%发生在胃部。在肠道髓系肉瘤中检测到的最常见分子异常是融合蛋白CBFB-MYH11。几项大型研究的综述表明，髓系肉瘤的存在并不构成独立的预后因素。治疗方法将根据存在的特定基因异常进行调整，全身化疗联合造血干细胞移植是最有效的策略。
