6例髓系肉瘤的临床病理分析

Clinicopathologic study of 6 patients with myeloid sarcoma

目的探讨髓系肉瘤（MS）的临床病理学特征、免疫学表型、鉴别诊断、治疗及预后。方法收集6例MS患者临床病理资料,对标本采用常规石蜡切片行HE染色,En Vision法免疫组化染色,骨髓涂片检查,并对生存进行随访。结果6例MS中男女各3例,年龄范围23~66岁;发生部位为小肠1例、上腭1例、手掌1例、结肠1例、肺1例、宫颈1例。细胞形态学特点为肿瘤细胞弥漫浸润,散在分布,互不粘附;肿瘤细胞中等大小,形态较单一,细胞核大,呈圆形或卵圆形,胞浆少,染色质细腻而均匀分布,2例核仁清晰,大小不一,部分可见嗜酸性粒细胞。免疫组化标记不同程度表达MPO、CD43、CD117、CD68-KP1、CD13、CD15、Lysozyme,结合病理组织学形态及免疫组化,6例均符合粒细胞肉瘤。随访期内3例伴急性髓系白血病确诊后分别于5、6、11个月死亡,其余3例仍存活。结论 MS为一种罕见以髓系细胞形态学和免疫表型特征的恶性肿瘤,需在形态学的基础上辅以免疫组织化学进行诊断。

Objective To investigate the clinicopathologic features,immunophenotyping,differential diagnoses,treatment and prognosis of myeloid sarcoma（ MS）. Methods The clinical and pathologic data of 6 cases of MS were reviewed. HE stain,immunohistochemistry stain by En Vision method and bone marrow slides examination were carried out. The followed up information was available in all patients. Results There were 3 males and 3 females. The age ranged from 23 to 66 years. The sites of involvement included small intestine,palate,palm of the hand,colon,lung and cervix. The tumor cells were widespread infiltration,diffused distribution with no adhesion each other; tumor cells were middle size,little cytoplasm and large nucleolus. Nucleolus were round and orbicular-ovate. Chromatin was exquisite and uniform distribution; nucleolus was clear with different sizes in 2 cases. Some eosinophilic granulocytes were visible. Immunohistochemical study showed that cases were positive for MPO,CD43,CD117,CD68-KP1,CD13,CD15 and lysozyme. According to histopathology feature and immunohistochemical result,the 6 patients were granulocytic sarcoma.During the follow-up,3 cases with AML died at 5,6,11 months after diagnosis. Other three patients survived. Conclusion MS is a scarce malignant tumor that is myeloid cells morphology and immunophenotype characters. The diagnosis should be based on tissue morphology and immunohistochemistry observation.