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目的:探讨透明细胞软骨肉瘤（clear cell chondrosarcoma，CCCS）的临床表现、影像学特点、病理组织学特征、诊断陷阱、治疗以及预后。方法:回顾性分析2010年1月至2020年1月23例收治并手术治疗的透明细胞软骨肉瘤患者资料，其中男21例，女2例；年龄（45.78±16.19）岁（范围：27~72岁）。21~40岁8例（35%），41~60岁10例（43%），61~80岁者5例（23%）。发病部位股骨8例，骨盆7例，胸腰椎4例，骶骨3例，胫骨1例。术前穿刺及术后大体标本用10%中性福尔马林固定，5%硝酸脱钙处理，石蜡包埋，行苏木素-伊红（hematoxylin and eosin，HE）染色及免疫组织化学染色（envision法），收集术前影像学表现及临床症状，术后显微镜下病理组织学表现及免疫表型，归纳总结透明细胞软骨肉瘤的临床、影像学及病理形态学特点。结果:23例透明细胞软骨肉瘤中影像学均显示骨质破坏，部分病例为境界相对清楚的溶骨性破坏，部分病例有硬化缘。7例术前有血清碱性磷酸酶升高。肿瘤组织大体灰白灰红色，部分病例可见瓷白色软骨样区域。镜下肿瘤细胞圆形、多角形，部分胞浆透亮，境界清楚，部分胞浆嗜酸性，细胞核圆形、居中，核分裂像罕见。常常可见结节状分布的软骨样基质及幼稚的编织骨，散在多核破骨巨细胞样巨细胞。免疫组化染色可不同程度的表达S-100、D2-40、EMA、Vimentin、p16、SATB2，表达不具备特异性。治疗主要通过外科手术整块切除，术后出现复发10例（43.5%），无远处转移病例。结论:透明细胞软骨肉瘤是软骨肉瘤的一种少见亚型，具有低度恶性生物学行为，准确诊断需要临床、影像学和病理三结合，外科整块切除预后较好。“,”Objective:To investigate the clinical manifestations, imaging features, histopathological features, diagnostic pitfalls, treatment and prognosis of clear cell chondrosarcoma (CCCS).Methods:23 cases of CCCS admitted and operated from January 2010 to January 2020 were analyzed retrospectively. Among the 23 cases, 21 were males and 2 were females. There were 8 cases (35%) aged 21-40, 10 cases (43%) aged 41-60 and 5 cases (23%) aged 61-80. There were 8 femurs, 7 pelvis, 4 thoracolumbar spine, 3 sacrum and 1 tibia. The specimens were fixed with 10% phosphate-buffered formalin, decalcified with 5% nitric acid, embedded in paraffin and stained with hematoxylin and eosin (HE) and immunohistochemistry (Envision). The preoperative imaging and clinical symptoms, and the postoperative histopathological and immunophenotype under the microscope were collected. And the relevant literature was reviewed to summarize the clinical, imaging and pathomorphological characteristics of CCCS.Results:23 cases of CCCS showed bone destruction in imaging, some cases were well-circumscribed lytic lesions, some cases had sclerotic margin. The serum alkaline phosphatase was increased in 7 patients before operation. The tumor tissue was gray-white and gray-red in general and some cases showed porcelain white cartilage-like areas. Microscopically, the tumor cells are round or polygonal, some of them have clear cytoplasm and boundary, some of them are eosinophilic, some of them have round and centrally located nuclei, and mitotic image is rare. It is often seen that there are nodular distribution of cartilage-like matrix and immature woven bone, multinucleated osteoclast-like giant cell scattered in those components. Immunohistochemical staining: S-100, D2-40, EMA, Vimentin, p16, SATB2 can be positive in varying degrees. The surgical treatment is mainly through en bloc excision. 10 patients had recurrence and no distant metastasis.Conclusion:CCCS is a rare subtype of chondrosarcoma, which has low-grade malignant biological behavior and is easy to be misdiagnosed clinically and pathologically. Pathological diagnosis needs to be careful. Careful observation of microscopic histology is necessary in order to avoid over-diagnosis of osteosarcoma leading to clinical treatment errors. Once the biopsy is confirmed, it needs en bloc excision in order to reduce the recurrence rate. Long-term follow-up is needed after the operation, the overall prognosis was good.