He Tao, Guo Yun, Wang Chao, Yan Jun, Zhang Meiying, Xu Wei, He Xuan, Zheng Shufang
Department of Pathology, The Affiliated Hospital of Logistics University of Chinese People's Armed Police Force Tianjin 300162, China.
Department of Endocrinology and Hematology, The Affiliated Hospital of Logistics University of Chinese People's Armed Police Force Tianjin 300162, China.
Int J Clin Exp Pathol. 2018 Aug 1;11(8):4158-4162. eCollection 2018.
Myeloid sarcoma (MS) is a rare, extramedullary hematological malignant tumor. MS, which is shown to precede acute myeloid leukemia (AML), and in which bone marrow aspiration or biopsy finds no hematological disorder, is classified as primary or isolated MS. Primary MS with no evidence of the cancer in the blood is rare. Herein, we report a case of a primary MS involved the small intestine and mesentery. A 40-year-old man with intermittent upper vague abdominal pain for 1 month was admitted to the hospital on October 20th, 2017. The pain was obviously aggravated after food ingestion, but he had no nausea or vomiting. An abdominal computed tomography (CT) showed a soft tissue density mass in the mesenteric region and a wall thickening of the jejunum. Positron emission tomography (PET)-CT imaging with 18F-fluorodeoxyglucose (18F-FDG) showed a significant uptake inmesenteric regional mass (7.6 cm × 4.1 cm) and jejunum wall. The patient underwent a laparotomy, and the involved part of the small intestine along with the mesentery was resected. Histological examination and immunohistochemical (IHC) staining determined the pathological diagnosis was MS. Clinical laboratory tests and a bone marrow biopsy were used to rule out systemic AML. The patient had been treated with a combination of pirarubicin and cytarabine. A follow-up CT scan and necessary clinical laboratory tests were performed after the surgery and no abnormalities were found. To date, the patient continues to be in complete remission. In conclusion, primary MS is a rare disease, yet the diagnosis of MS should be considered when any mass with diffusely infiltrating tumor cells is observed.
髓系肉瘤（MS）是一种罕见的髓外血液系统恶性肿瘤。MS被证明先于急性髓系白血病（AML）出现，且骨髓穿刺或活检未发现血液系统疾病，被归类为原发性或孤立性MS。无血液系统癌症证据的原发性MS很罕见。在此，我们报告一例累及小肠和肠系膜的原发性MS病例。一名40岁男性，间歇性上腹部隐痛1个月，于2017年10月20日入院。进食后疼痛明显加重，但无恶心或呕吐。腹部计算机断层扫描（CT）显示肠系膜区域有软组织密度肿块，空肠壁增厚。18F-氟脱氧葡萄糖（18F-FDG）正电子发射断层扫描（PET）-CT成像显示肠系膜区域肿块（7.6 cm×4.1 cm）和空肠壁有明显摄取。患者接受了剖腹手术，切除了受累的小肠部分及肠系膜。组织学检查和免疫组织化学（IHC）染色确定病理诊断为MS。临床实验室检查和骨髓活检用于排除全身性AML。患者接受了吡柔比星和阿糖胞苷联合治疗。术后进行了随访CT扫描和必要的临床实验室检查，未发现异常。迄今为止，患者仍处于完全缓解状态。总之，原发性MS是一种罕见疾病，但当观察到任何有弥漫性浸润肿瘤细胞的肿块时，应考虑MS的诊断。
