【摘要】 目的:分析软组织间叶性软骨肉瘤的临床病理学和免疫组织化学特点。探讨其诊断、鉴别诊断依据和预后。方法:复习北京积水潭医院1995至2005年确诊的2例软组织间叶性软骨肉瘤的病理学及临床特点,应用免疫组织化学二步法检测肿瘤细胞对白细胞共同抗原(leukocyte common antigen,LCA),CD3,CD20,CD45RO,CD79a,CD99,神经元特异性烯醇化酶(neuron specific enolase,NSE),S-100,突触素(synatpophysin,Syn),嗜铬素(chromogra-nin A,CgA),CK7,CK19,上皮膜抗原(epithelial membrane antigen,EMA),二型胶原(collagen typeⅡ,Coll-Ⅱ),肌动蛋白(sarcomeric-actin),结蛋白(desmin),Ki-67,P53的表达,并进行随访。结果:2例软组织间叶性软骨肉瘤均发生于下肢横纹肌组织内。其主要影像学特点为肿瘤内钙化和肿瘤边缘硬化;病理组织学表现为未分化小细胞与肿瘤性软骨岛并存,小细胞区均见血管外皮瘤样结构。CD99,NSE,CgA和Syn在肿瘤小细胞呈阳性表达;S-100在软骨细胞呈阳性表达;Coll-Ⅱ在软骨基质呈阳性表达;Ki-67,P53在51%-75%的肿瘤细胞核呈阳性表达;LCA,CD3,CD20,CD45RO,CD79a,Sarcomeric-Actin,Desmin,CK7,CK19和EMA阴性。Ki-67的表达程度可能和预后有关。结论:软组织间叶性软骨肉瘤是小细胞原始间叶组织向幼稚软骨组织的分化,主要病理学特征是未分化小细胞与肿瘤性软骨并存的双态性结构和小细胞区形成血管外皮瘤样结构。此肿瘤预后差。更多还原

【Abstract】 Objective:To investigate the clinicopathological and immunohistochemical characteristics of soft tissue mesenchymal chondrosarcoma. Methods:The clinical material,pathological and immunohistochemical characteristics(reactiontoLCA,CD3,CD20,CD45RO,CD79a,CD99,NSE,S-100,Syn,CgA,CK7,CK19,EMA,Coll-Ⅱ,Sarcomeric-Actin,Desmin,Ki-67,P53) of 2 cases of soft tissue mesenchymal chondrosarcoma in Jishuitan Hospital between 1995 and 2005 were reviewed and followed up. Results:The two patients were both females. The tumors were located in the low extremity muscles. The main roentgenographical appearance was stippled calcification in tumor and calcification at the edge of the tumor. The histological characteristic features showed undifferentiated small cells together with islands of chondrosarcoma;there was hemangiopericytoma-like arrangement of small cells.The tumor cells were positive for CD99,NSE,Syn,CgA;The cells in chondroid matrix were positive for S-100;chondroid matrix was positive for Coll-Ⅱ. All tumor cells were negative for LCA,CD3,CD20,CD45RO,CD79a,Sarcomeric-actin,Desmin and CK7,CK19, and EMA. The patient with followed radiotherapy was alive. and the other without radiotherapy dead. Conclusion: Mesenchymal chondrosarcoma of soft tissue has the characteristics of primary mesenchyme which differentiates to congenital cartilage. The pathological characteristics of bimophic pattern and roentgenographical appearance of tumor are useful for diagnosis.The prognosis is poor.更多还原