目的　了解透明细胞肉瘤的病理与临床特点。方法　复习中国医学科学院肿瘤医院1973 年至1998 年收治的6 个病例及复习文献,对其病理及临床特点、治疗方法及疗效、预后因素等做一总结。结果　透明细胞肉瘤来源于原始神经外胚层细胞,由在胚胎形成过程中迷走于肌腱和肌筋膜中的神经脊细胞发展而来,具有特征性的染色体改变,即t(12;22)(q1314;q1213) 等;病变与肌腱及肌筋膜关系密切;易出现区域淋巴结转移;手术切除后易局部复发。治疗原则应为手术加术后大野放射治疗并采用多次缩野技术,区域淋巴结应给予手术清扫或放射治疗。5 年生存率为37 % ～67% 。影响预后的因素有肿瘤大小及是否伴有坏死。结论　透明细胞肉瘤是一类有自身特征的肿瘤,治疗上应有别于其他软组织肉瘤 Objective To understand the pathological and clinical features of clear cell sarcoma. Methods Review 6 cases from the Cancer Hospital of the Chinese Academy of Medical Sciences from 1973 to 1998 and review the literature. We summarized the pathology and clinical features, treatment methods, curative effects and prognostic factors. RESULTS Clear cell sarcoma originated from primitive neuroectodermal cells and developed from neural crest cells that are lost in tendons and myofascial muscles during embryonic development and have characteristic chromosomal alterations, ie t(12;22)(q13). 14; q12  13), etc.; lesions are closely related to tendon and myofascial; regional lymph node metastasis is easy to occur; local recurrence after surgery is easy. The principle of treatment should be surgery plus postoperative Ohno radiation therapy and multiple shrinkage techniques. Regional lymph nodes should be given surgery or radiotherapy. The 5-year survival rate was 37% to 67%. Factors affecting prognosis are tumor size and whether it is associated with necrosis. Conclusion Clear cell sarcoma is a type of tumor with its own characteristics. It should be treated differently than other soft tissue sarcomas.