Lee Jung Yeon, Chung Haerim, Cho Hyunsoo, Jang Ji Eun, Kim Yundeok, Kim Soo-Jeong, Kim Jin Seok, Hyun Shin Young, Min Yoo Hong, Cheong June-Won
Department of Internal Medicine, Yonsei University Wonju College of Medicine, Wonju, Korea.
Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.
Blood Res. 2017 Sep;52(3):184-192. doi: 10.5045/br.2017.52.3.184. Epub 2017 Sep 25.
Isolated myeloid sarcoma (MS) is a rare extramedullary tumor mass composed of malignant myeloid precursor cells without any evidence of leukemia in the peripheral blood and bone marrow. We describe the clinical characteristics and outcomes of patients diagnosed with isolated MS at our institution.
We retrospectively reviewed 9 of 497 acute myeloid leukemia (AML) patients (1.8%) with isolated MS. Isolated MS patients were divided into 2 groups according to the first-line treatment strategy: systemic treatment only (S) or local treatment with or without systemic treatment (LS).
The most common site of MS occurrence was the head and neck area (N=4, 44.4%), followed by the anterior mediastinum (N=2, 22.2%) and the gastrointestinal tract (N=2, 22.2%). The tumors of 4 patients (44.4%) eventually evolved to AML, in a median time of 13.4 months (range, 2.4-20.1 mo). The number of patients achieving complete remission after first-line treatment was higher in the LS group (N=5, 83.3%) than in the S group (N=1, 33.3%) ( =0.226). All patients in the LS group survived, but those in the S group died (=0.012).
Accurate and rapid diagnosis using various modalities and the early initiation of intensive combined treatment may be the optimal strategies to reduce the risk of isolated MS subsequently evolving to AML. To fully understand the characteristics of isolated MS, a larger number of patients from a multinational study is necessary.
孤立性髓系肉瘤（MS）是一种罕见的髓外肿瘤块，由恶性髓系前体细胞组成，外周血和骨髓中无白血病证据。我们描述了在我们机构诊断为孤立性MS的患者的临床特征和预后。
我们回顾性分析了497例急性髓系白血病（AML）患者中的9例（1.8%）孤立性MS患者。根据一线治疗策略，将孤立性MS患者分为2组：仅全身治疗（S）组或联合或不联合全身治疗的局部治疗（LS）组。
MS最常见的发生部位是头颈部（n = 4，44.4%），其次是前纵隔（n = 2，22.2%）和胃肠道（n = 2，22.2%）。4例患者（44.4%）的肿瘤最终进展为AML，中位时间为13.4个月（范围2.4 - 20.1个月）。LS组一线治疗后达到完全缓解的患者数量（n = 5，83.3%）高于S组（n = 1，33.3%）（P = 0.226）。LS组所有患者均存活，但S组患者死亡（P = 0.012）。
采用多种方式进行准确快速诊断并尽早开始强化联合治疗可能是降低孤立性MS随后进展为AML风险的最佳策略。为全面了解孤立性MS的特征，有必要进行一项纳入更多患者的多国研究。
