盆腹腔上皮样炎性肌纤维母细胞肉瘤的临床病理研究

目的:为了探讨盆腹腔上皮样炎性肌纤维母细胞肉瘤的临床特点、诊断、治疗和预后，对其病理学特点进行研究分析。方法:选取2018年06月我院接诊且病理科病理诊断为盆腹腔上皮样炎性肌纤维母细胞肉瘤的1例患者作为研究对象，分别通过巨检、镜检、免疫表型以及免疫组织化学的方法对病理标本进行分析，并通过查阅相关文献，复习相关文献，对盆腹腔上皮样炎性肌纤维母细胞肉瘤的临床特点做进一步的研究。结果:镜下见肿瘤细胞排列紊乱，连成片状，大小不一，形态多样。主要组成为上皮样细胞，肿瘤细胞形状呈圆形、短梭形、多边形、椭圆形及不规则形。细胞排列较为松散，弥散分布。肿瘤细胞核仁较大，镜下可见核分裂象和瘤巨细胞;间质中见明显的大量炎症细胞浸润，炎性浸润以中性粒细胞为主，伴有少量浆细胞、淋巴细胞及嗜酸性粒细胞;胞质丰富，肿瘤细胞有明显水肿和黏液样变性。部分癌变区域排列较为密集，细胞形态不清。肿瘤细胞免疫组化结果vimentin、desmin、核膜ALK、CD30弥漫性表达强阳性，而ALK特异性的在细胞核膜阳性表达。部分肿瘤细胞EMA、INI-1、AAT、SMA、CK部分、p53部分阳性。FISH检测结果显示肿瘤细胞多有单一红色信号及红绿分离信号，多为阳性。结论:盆腹腔上皮样炎性肌纤维母细胞肉瘤发病较为罕见，是高度恶性肿瘤，预后较差。但是其病理学和免疫组化特点较为突出，根据病理学结果更有利于临床诊断。

Clinicopathological study of pelvic and peritoneal inflammatory myofibroblastic sarcoma

Objective:To investigate the clinical features,diagnosis,treatment and prognosis of pelvic and intraperitoneal inflammatory myofibroblastic sarcoma,and to analyze its pathological features.Methods:A patient with pathological diagnosis of pelvic and abdominal epithelial inflammatory myofibroblastic sarcoma was enrolled in our hospital in June 2018.The subjects were examined by macroscopic examination,microscopic examination,immunophenotype and immunohistochemistry.The clinical characteristics of pelvic and abdominal epithelial inflammatory myofibroblastic sarcoma were further studied by analyzing the pathological specimens and reviewing the relevant literature.Results:Under the microscope,the tumor cells were arranged in disorder,and they were connected into pieces,varying in size and in various forms.The main composition was epithelioid cells,and the shape of the tumor cells was circular,short fusiform,polygonal,elliptical and irregular.The cells were arranged loosely and diffusely.The nucleoli of tumor cells were large,and mitotic figures and giant cells of the tumor were seen under the microscope.A large number of inflammatory cells infiltrate in the interstitial,and inflammatory infiltrates were mainly neutrophils,accompanied by a small amount of plasma cells,lymphocytes and eosinophils.Cells,cytoplasm rich,tumor cells have obvious edema and mucoid degeneration.Some cancerous regions were densely arranged and the cell morphology was unclear.The immunohistochemical results of tumor cells were strongly positive for vimentin,desmin,nuclear membrane ALK and CD30,while ALK-specific expression was positive in nuclear membrane.Some tumor cells were positive for part of p53 in EMA,INI-1,AAT,SMA and CK.The results of FISH test showed that the tumor cells had a single red signal and red and green separation signals,mostly positive.Conclusion:The incidence of pelvic and peritoneal inflammatory myofibroblastic sarcoma is rare.And it is a highly malignant tumor with a poor prognosis.However,its pathological and immunohistochemical features are more prominent,and the pathological results are more conducive to clinical diagnosis.