成人经典型腺泡状横纹肌肉瘤1例并文献复习
发布时间：2018-11-05 08:25
【摘要】：目的探讨经典型腺泡状横纹肌肉瘤(alveolar rhabdomyosarcoma,ARMS)的临床病理学特征、鉴别诊断、治疗及预后。方法观察1例成人经典型ARMS的临床病理学及免疫表型特征,并复习相关文献。结果肿瘤发生于左肩部,镜下见肿瘤边界不清,浸润周围纤维及脂肪组织,瘤细胞呈片状或巢状排列,部分瘤细胞形成特征性的腺泡状排列结构,腺泡之间可见纤维血管性间隔。肿瘤由未分化的原始间叶性细胞组成,呈圆形、卵圆形或小多边形,核深染,核分裂易见,胞质少。免疫表型:瘤细胞desmin、Myo D1弥漫强阳性,vimentin、Myoglobin、CD56阳性,Syn、Cg A、CD20、CD79a、CD3、CD99、CK(AE1/AE3)、EMA均阴性,Ki-67增殖指数约70%。结论 ARMS是横纹肌肉瘤的一种特殊类型,形态学上以瘤细胞形成腺泡状结构为特征,需与腺泡状软组织肉瘤、骨外尤因肉瘤/外周原始神经外胚层瘤、小细胞神经内分泌癌及恶性淋巴瘤等相鉴别。
[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.
【作者单位】： 江苏省常州市中医医院病理科;江苏省常州市第一人民医院病理科;江苏省人民医院病理科;
【分类号】：R738.7
本文编号：2311498
[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.
【作者单位】： 江苏省常州市中医医院病理科;江苏省常州市第一人民医院病理科;江苏省人民医院病理科;
【分类号】：R738.7
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