骨外黏液样软骨肉瘤2例报道及文献复习

目的探讨骨外黏液样软骨肉瘤的临床病理学特征。方法对2例骨外黏液样软骨肉瘤进行光镜观察及免疫组化染色标记,并通过相关文献复习,对病理诊断及鉴别诊断等指标进行分析。结果1例发生于足底,1例发生于乳腺。光镜下肿瘤呈分叶状,边界清。细胞为圆形及短梭形,成束状排列于黏液样基质中,局部可见围血管形成玫瑰花结样结构。部分肿瘤细胞异型性明显。免疫表型:vimentin、NSE、Syn均呈阳性;例1EMA灶性阳性,例2阴性;S-100蛋白、CgA及CK均阴性。结论骨外黏液样软骨肉瘤为罕见的软组织恶性肿瘤,具特异性的组织病理学特点。主要发生于四肢,少数可发生于实质器官,至今未有乳腺原发病例报道。部分肿瘤细胞可发生间变导致诊断困难,须与脊索瘤、骨内软骨肉瘤、化生性癌及黏液性肿瘤等鉴别。

Extraskeletal myxoid chondrosarcoma: A report of two cases and review of literature

Purpose To investigate the clinicopathological characteristics of extraskeletal myxoid chondrosarcoma(EMC).Methods The histopathology and immunophenotype of 2 cases of EMC were studied in order to analyse its diagnostic and differential diagnostic factors.Results One tumor occurred in sole of foot and another in breast.Microscopically,the neoplasms were relatively well circumscribed and composed of rounded and elongated cells arranged in strands and cords in a myxoid background.The cells created nests and showed focally a perivascular rosette-like arrangement.Apparent atypical cells presented in local areas.The tumors were immunoreactive for vimentin,neuron specific enolase,synaptophysin and partly for epithelial membrane antigen,but negative for chromogranin A,S-100 protein and cytokeratin.Conclusions EMC is a rare soft-tissue sarcoma characterized by distinctive histopathological features.The tumors are located mainly in extremities and rarely in parenchymatous organs.No case of primary tumor of EMC has been reported to date arising within breast.Diagnosis is always confused when anaplasia exists and differential diagnosis includes chordoma,conventional chondrosarcoma,metaplastic carcinoma and soft tissue tumors with a myxoid stroma.