腹腔上皮样炎性肌纤维母细胞肉瘤临床病理分析并文献复习

Clinicopathologic analyses of epithelioid inflammatory myofibroblastic sarcoma and review of the literature

目的:通过对上皮样炎性肌纤维母细胞肉瘤(epithelioid inflammatory myofibroblastic sarcoma,E I M S)的临床病理学特征的分析,提高对其诊断认识能力,减少误诊。方法:分析3例E I M S的临床病理特征并复习相关文献。结果:术后镜下示:肿瘤细胞弥漫分布,呈圆形、上皮样,胞浆丰富、嗜酸性、核偏位,核仁明显,部分可见梭形细胞,背景显著黏液变性伴突出的炎症细胞浸润,以中性粒细胞为主。免疫表型:肿瘤细胞表达ALK、Desmin、CD30、EMA、INI-1;部分表达:CK和SMA;不表达LCA、CD15、Myo D1、S-100、HMB-45、CD20、CD3、CD34、CD117和DOG-1。FISH检测显示3例均有ALK基因相关易位。结论:EIMS是高度恶性肿瘤,发生率比较低,细胞学形态特征、免疫组织化学特点及分子特征对本病的诊断具有重要价值。

Objective：To explore the characteristics of clinicopathology and diagnostic criteria about epithelioid inlfammatory myoifbroblastic sarcoma （EIMS）. Methods：Analyzed the clinicopathological features of 3 cases of EIMS and review the relevant literature. Results：Postoperative mirror are shown below：the tumor cells were diffusely distributed, rounded and epithelioid, abundant cytoplasm, addicted to acidic, eccentric nucleus and nucleoli, partially visible spindle cells, background significantly mucoid degeneration with prominent inlfammatory cell inifltration, in neutrophils. Immune phenotype：expression of tumor cells ALK, Desmin, CD30, EMA, INI-1;partial expression：CK and SMA;no expression：LCA, CD15, MyoD1, S-100, HMB-45, CD20, CD3, CD34, CD117 and DOG-1, FISH detection showed that 3 cases have ALK gene related translocation. Conclusion：EIMS is a highly malignant tumor, which has a low incidence rate. It has important value in the diagnosis of the disease by morphological features, immunohistochemical characteristics and molecular characteristics.