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伪装成阻塞性十二指肠癌的原发性髓系肉瘤。

Primary myeloid sarcoma masquerading as an obstructing duodenal carcinoma.

作者信息

Narayan Preeti, Murthy Vijayashree, Su Mu, Woel Rosemonde, Grossman I Robert, Chamberlain Ronald S

机构信息

School of Medicine, St. George's University, Grenada, West Indies.

出版信息

Case Rep Hematol. 2012;2012:490438. doi: 10.1155/2012/490438. Epub 2012 Nov 29.

Abstract

Myeloid Sarcoma (MS), a rare extra hematopoietic carcinoma composed of blast cells, is located primarily in extramedullary sites such as skin, soft tissue, lymph nodes, and bone. MS usually presents in the setting of coexisting acute myeloid leukemia (AML) and myeloproliferative disorders. Gastrointestinal involvement (GI) is extremely rare from nonspecific abdominal symptoms to obstruction. Eight cases of myeloid sarcoma involving the duodenum including the current case have been reported, overall mean age being 40 years (range 17-71) and M : F ratio 7 : 1. The prognosis of patients with de novo MS cases has been reported to be better than those who have a coexisting leukemia. MS is a rare extramedullary tumor, which should be considered in the differential diagnosis of a soft tissue mass involving the duodenum, especially if there is a coexisting hematological disorder. De novo cases often progress to AML, and current therapy involves Daunorubicin- and Cytarabine-based chemotherapy. The wide cytogenetic and molecular heterogeneity of MS implies a potential role for more targeted MS therapies, which may offer a curative strategy.

摘要

髓系肉瘤(MS)是一种罕见的由原始细胞组成的髓外造血性肿瘤,主要位于皮肤、软组织、淋巴结和骨骼等髓外部位。MS通常在同时存在急性髓系白血病(AML)和骨髓增殖性疾病的情况下出现。胃肠道受累(GI)极为罕见,可出现非特异性腹部症状至肠梗阻。包括本例在内,已有8例累及十二指肠的髓系肉瘤病例报告,总体平均年龄为40岁(范围17 - 71岁),男女比例为7∶1。据报道,初发MS病例患者的预后优于合并白血病的患者。MS是一种罕见的髓外肿瘤,在涉及十二指肠的软组织肿块的鉴别诊断中应予以考虑,特别是在存在血液系统疾病的情况下。初发病例常进展为AML,目前的治疗包括基于柔红霉素和阿糖胞苷的化疗。MS广泛的细胞遗传学和分子异质性意味着更具针对性的MS治疗可能发挥作用,这可能提供一种治愈策略。

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