【摘要】：目的探討經(jīng)典型腺泡狀橫紋肌肉瘤(alveolar rhabdomyosarcoma,ARMS)的臨床病理學特征、鑒別診斷、治療及預后。方法觀察1例成人經(jīng)典型ARMS的臨床病理學及免疫表型特征,并復習相關文獻。結果腫瘤發(fā)生于左肩部,鏡下見腫瘤邊界不清,浸潤周圍纖維及脂肪組織,瘤細胞呈片狀或巢狀排列,部分瘤細胞形成特征性的腺泡狀排列結構,腺泡之間可見纖維血管性間隔。腫瘤由未分化的原始間葉性細胞組成,呈圓形、卵圓形或小多邊形,核深染,核分裂易見,胞質少。免疫表型:瘤細胞desmin、Myo D1彌漫強陽性,vimentin、Myoglobin、CD56陽性,Syn、Cg A、CD20、CD79a、CD3、CD99、CK(AE1/AE3)、EMA均陰性,Ki-67增殖指數(shù)約70%。結論 ARMS是橫紋肌肉瘤的一種特殊類型,形態(tài)學上以瘤細胞形成腺泡狀結構為特征,需與腺泡狀軟組織肉瘤、骨外尤因肉瘤/外周原始神經(jīng)外胚層瘤、小細胞神經(jīng)內分泌癌及惡性淋巴瘤等相鑒別。
[Abstract]:Objective to investigate the clinicopathological features, differential diagnosis, treatment and prognosis of classical alveolar rhabdomyosarcoma (alveolar rhabdomyosarcoma,ARMS). Methods the clinicopathological and immunophenotypic features of a typical adult ARMS were observed and the relevant literatures were reviewed. Results the tumor occurred on the left shoulder. Under the microscope, the boundary of the tumor was unclear, the surrounding fibers and adipose tissue were infiltrated, the tumor cells were arranged in the shape of flake or nest, and some of the tumor cells formed the characteristic acinar structure. Fibrous vascular septum can be seen between the acinus. The tumor is composed of undifferentiated primitive mesenchymal cells, which are round, oval or small polygon, with deep staining of nucleus, easy mitosis and less cytoplasm. Immunophenotype: desmin,Myo D1 was strongly diffusely positive, vimentin,Myoglobin,CD56 was positive, Syn,Cg Agna CD20, CD79, CD39 and CK (AE1/AE3), EMA were negative, Ki-67 proliferative index was about 70%). Conclusion ARMS is a special type of rhabdomyosarcoma, which is characterized by acinar structure formed by tumor cells, and should be associated with acinar soft tissue sarcoma, extraosseous Ewing sarcoma / peripheral primitive neuroectodermal tumor. Small cell neuroendocrine carcinoma and malignant lymphoma are differentiated.
【作者單位】： 江蘇省常州市中醫(yī)醫(yī)院病理科;江蘇省常州市第一人民醫(yī)院病理科;江蘇省人民醫(yī)院病理科;
【分類號】：R738.7

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