Purpose To explore the clinical, imaging, pathologic features and diagnosis, treatment and prognosis of dedifferentiated chondrosarcoma (DDCS). Methods Forty cases of DDCS confirmed by pathologists were collected from Shanghai Jiaotong University Affiliated Sixth People's Hospital from January 2005 to August 2017, including clinical, imaging, pathologic and follow-up data. HE, immunohistochemistry and statistical methods were used with review of the related literature. Results There were 21 males and 19 females in 40 cases of DDCS, with an average age of 51 years. The tumors were located in hip, proximal femur, humerus, sternum, tibia, shoulder, finger, and thoracic cavity. The main clinical manifestations were local pain, swelling, limited mobility and so on. Typical radiographic manifestations are "bimorphic features", which showed two manifestations of dot or circular like calcification of chondrosarcoma, and invasive soft tissue masses. Histologically, clearly defined well-differentiated chondrosarcoma components juxtaposed highly malignant dedifferentiation components, which can display as follows: osteosarcoma, fibrosarcoma, malignant fibrous histiocytoma, and spindle cell sarcoma that could not be clearly classified. The dedifferentiated components might also be low-grade tumors such as giant cell tumor of bone or low grade osteosarcoma. The proportions of these two ingredients were uncertain. Preoperative biopsy was performed in 34 cases, of whom only 9 (26.5％) were correctly diagnosed as DDCS. The average survival time of patients with metastasis at first visit was significantly shorter than the patient without metastasis. Conclusion Diagnosis of DDCS should be combined with clinical features, imaging and histological morphology. As their histological morphology complex, and preoperative puncture biopsy limitations, its diagnosis is very difficult. Patients with metastases at the time of initial diagnosis have a worse prognosis.%目的 探讨去分化软骨肉瘤(dedifferentiated chondrosarcoma,DDCS)的临床、影像、病理学特征及诊断、治疗和预后等.方法 收集2005年1月～2017年8月上海交通大学附属第六人民医院经病理确诊的40例DDCS,包括临床、影像、病理和随访资料,采用HE、免疫组化对其进行分析并复习相关文献.结果 40例DDCS中男性21例,女性19例,平均年龄51岁.发病部位分别为髋部、股骨、肱骨、胸骨、胫骨、肩部、手指、胸椎.临床主要表现为疼痛、肿胀、活动受限等.典型的影像学表现为双相征,即呈现出软骨肉瘤的点状、环状钙化和具有侵袭性特征的软组织肿块两种表现.镜下可见界限清楚的高分化软骨肉瘤成分和高度恶性成分(包括骨肉瘤、恶性纤维组织细胞瘤、纤维肉瘤、不能明确分类的梭形细胞肉瘤),或去分化为低级别肿瘤(包括骨巨细胞瘤或低级别骨肉瘤),两种成分比例不定.40例中有34例术前行穿刺活检,仅9例正确诊断为DDCS,穿刺诊断正确率为26.5％.在初诊时已发生转移患者的平均生存时间明显短于未发生转移患者.结论 DDCS的诊断需临床、影像和病理三结合.其组织学形态表现复杂多样、术前穿刺活检存在局限性,其诊断比较困难.初次诊断时伴有转移的患者预后更差.
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