间叶性软骨肉瘤23例临床病理和免疫表型分析

Clinicopathologic and immunohistochemical study of 23 cases of mesenchymal chondrosarcoma

目的探讨间叶性软骨肉瘤的临床病理和免疫表型特点。方法复习23例骨及软组织间叶性软骨肉瘤的临床和病理学资料，对其中14例进行免疫组织化学（EnVision法）染色。结果男性14例，女性9例，年龄范围12-47岁，其中原发于骨的病变13例，原发于软组织5例，早期症状以局部肿块和疼痛为主。镜下组织学特点为含有原始间叶性小细胞和分化良好的软骨成分，二者有移行过渡，可见“骺板样软骨”。原始间叶性小细胞免疫组织化学染色阳性表达Sox9（14/14）、CD99（12/14）、波形蛋白（6/14）、CD56（4/14）、CD57（4/14）、神经元特异性烯醇化酶（3/14）、结蛋白（1／14）；II型胶原、S-100蛋白、上皮细胞膜抗原、PCK、突触素、嗜铬粒素A、CD34、c-erbB2均阴性。结论间叶性软骨肉瘤少见，结合组织病理形态特征及免疫组织化学染色对病理鉴别诊断有较大帮助。

Objective To study the clinicopathologic and immunohistochemieal features of mesenchymal ehondrosarcoma. Methods The clinical and histologic features of 23 cases of mesenchymal chondrosarcoma were analyzed. Immunohistochemieal study was also performed in 14 of the cases. Results The age of patients ranged from 12 to 47 years. Fourteen of them occurred in males. Thirteen cases involved the bony skeleton and 5 cases affected the soft tissue. The patients presented with pain and/or swelling. Histologically, the tumor consisted of a mixture of undifferentiated small round cells and hyaline cartilage. Transition between the two components was demonstrated and growth plate-like cartilage was observed. lmmunohistochemical study showed that the small round cells were positive for Sox9 （14/14）, CD99 （ 12/14 ）, vimentin （6/14）, CD56 （4/14）, CD57 （4/14）, neuron-specific enolase （ 3/14 ） and desmin （1/14）. They were negative for Coll-Ⅱ, S-100 protein, epithelial membrane antigen, pan-cytokeratin, synaptophysin, chromogranin A, CD34 and c-erbB2. Conclusions Mesenchymal chondrosarcoma is a rare malignant tumor. Thorough histologic examination, when coupled with immunohistochemical findings, is helpful in arriving at a correct diagnosis.