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目的观察骨和软组织间叶性软骨肉瘤的临床病理特点,分析其鉴别诊断方法。方法复习6例间叶性软骨肉瘤(包括活检误诊的2例)的临床、影像学及病理学资料,进行免疫组化染色,并复习文献进行讨论。结果 6例间叶性软骨肉瘤,男女各3例;年龄21～74岁,平均38.8岁。其中3例为骨原发,2例为软组织原发,1例位于后纵隔及椎管内难以确定骨还是软组织原发。临床表现为疼痛、肿胀、局部肿块及压迫症状。影像学可见溶骨性破坏或软组织肿块伴斑点状钙化。组织学表现呈双向分化的特点,由未分化小细胞和不同分化程度的软骨以不同比例构成,可见逐渐成熟的现象。免疫组化:小细胞CD99(6/6)、vimentin(5/6)和NSE(3/6)(+);软骨S-100(6/6)(+);小细胞间质及软骨基质呈不同程度collagenⅡ(6/6)(+)。结论间叶性软骨肉瘤是一种少见的恶性肿瘤,活检标本容易误诊,须结合组织学特征、影像学表现及免疫组化表型与其他肿瘤相鉴别。collagenⅡ可以作为鉴别诊断的有用标记。
Objective To observe the clinicopathological features of mesenchymal and soft tissue mesenchymal chondrosarcoma and to analyze the differential diagnosis methods. Methods The clinical, radiographic and pathological data of 6 cases of mesenchymal melanoma (including 2 cases misdiagnosed by biopsy) were reviewed, immunohistochemically stained and reviewed. Results 6 cases of mesenchymal chondrosarcoma, male and female in 3 cases; aged 21 to 74 years, mean 38.8 years. Three of them were primary bone, two were primary soft tissue, and one was located in the posterior mediastinum and spinal canal to determine the origin of bone or soft tissue. Clinical manifestations of pain, swelling, local mass and compression symptoms. Radiographic evidence of osteolytic destruction or soft tissue mass with plaque calcification. Histological manifestations of the characteristics of a two-way differentiation, from undifferentiated small cells and different degrees of differentiation of cartilage in different proportions, we can see the gradual maturation of the phenomenon. Immunohistochemistry: Small cell CD99 (6/6), vimentin (5/6) and NSE (3/6) (+); Cartilage S-100 (6/6) The collagen Ⅱ (6/6) (+) in varying degrees. Conclusion Mesenchymal chondrosarcoma is a rare malignant tumor. Biopsy specimens are easy to be misdiagnosed. The histological features, imaging findings and immunohistochemical phenotypes should be differentiated from other tumors. Collagen Ⅱ can be used as a useful marker for differential diagnosis.