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目的探讨透明细胞肉瘤的临床特点及治疗方法,分析治疗效果及影响因素。方法对1990年6月至2004年7月收治的17例透明细胞肉瘤进行回顾性分析。男8例,女9例;年龄12￣73岁,平均36.8岁。14例曾于外院行局部切除术,10例复发,2例有区域淋巴结转移。发病部位:足5例,上肢6例,下肢1例,躯干5例。肿瘤大小在5cm以上者6例,小于5cm者11例。17例中15例行手术治疗,2例放疗;联合使用化疗13例。手术方式包括间室切除2例,广泛切除12例,截趾1例。联合淋巴结清扫术4例,清扫后截肢1例。结果所有患者随访5￣89个月,平均31.5个月。15例行手术治疗者中1例局部复发。17例中9例出现区域淋巴结转移,转移率为52.9%;7例远隔转移,其中肿瘤大小在5cm以上者6例。死亡6例,存活11例,其中1例为带瘤存活。2年生存率为84%,5年生存率为36%。结论透明细胞肉瘤比较罕见,恶性度高,预后差。首次规范的手术治疗是治疗成败的关键,肿瘤大小是影响预后的重要因素。肿瘤大小、是否复发与是否发生转移关系密切,淋巴结转移与血行转移者预后差。放、化疗的疗效尚需进一步研究。
Objective To investigate the clinical features and treatment of clear cell sarcoma and to analyze the therapeutic effect and influencing factors. Methods 17 cases of clear cell sarcoma admitted from June 1990 to July 2004 were analyzed retrospectively. 8 males and 9 females; aged 12 to 73 years, an average of 36.8 years old. Fourteen cases had undergone local excision in the external hospital, 10 cases had recurrent disease, and 2 cases had regional lymph node metastasis. The incidence of parts: 5 cases of foot, upper limb in 6 cases, lower limb in 1 case, trunk in 5 cases. Tumor size in 5cm above 6 cases, less than 5cm in 11 cases. Among the 17 cases, 15 cases were treated by surgery and 2 cases were treated by radiotherapy. Thirteen cases were treated with chemotherapy. Surgical procedures included 2 cases of subtotal resection, extensive resection of 12 cases and 1 case of truncal toe. 4 cases of lymph node dissection, amputation in 1 case. Results All patients were followed up for 5 ~ 89 months with an average of 31.5 months. One of 15 patients underwent surgical treatment of local recurrence. In 17 cases, 9 cases showed regional lymph node metastasis, with a metastasis rate of 52.9%. Seven cases were distantly metastasized, of which 6 cases had a tumor size of 5cm or more. 6 died and 11 survived, of which 1 survived with tumor. The 2-year survival rate was 84% and the 5-year survival rate was 36%. Conclusions Clear cell sarcoma is relatively rare, with high malignancy and poor prognosis. The first standardized surgical treatment is the key to the success of treatment, tumor size is an important factor affecting the prognosis. Tumor size, recurrence and metastasis are closely related, lymph node metastasis and hematogenous metastasis poor prognosis. Radiotherapy, chemotherapy efficacy needs further study.