透明细胞肉瘤的治疗及疗效分析  

Clinical therapy for clear cell sarcoma

作　　者：邢汝维[1] 方志伟[1] 宋金纲[1] 杨蕴[1] 马育林[1] 张瑾[1] 陈勇[1] 滕胜[1] 多健[1] 

机构地区：[1]天津医科大学附属肿瘤医院骨软组织肿瘤科,300060

出　　处：《中华骨科杂志》2005年第8期485-490,共6页Chinese Journal of Orthopaedics

摘　　要：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 characteristic of clear cell sarcoma and evaluate the therapeutic effects as well as the influencing factors. Methods 17 cases of clear ceil sarcoma from June 1990 to July 2004 were analyzed. There were 8 males and 9 female with an average age of 36.8 ranging from 12 to 73 years. The tumor site were 6 in lower extremity including 5 in foot and ankle, 6 in upper extremity and 5 in trunk. Tumor size ≥5 cm in 6 cases and 〈5 cm in 11. 10 of 14 patients whose primary tumor was marginally excised had tumor relapse, at admission, 2 presented with local lymph node metastases, 1 in inguinal and popliteal fossa, the other in claviclar region, There were no lymph node metastases and distant metastases in 15. Primary tumor operations included compartment resection in 2, wide resection in 12, amputation in 1, Combined lymph node dissection in 4, and disarticulation in 1 after lymph node dissection. Two cases received radiotherapy and 13 recewed combined chemotherapy. Results After 5-89 months （average 31.5 months） of follow-up, tumor recurrence occurred in 1 of 15 who received operation, 9 of 17 had lymph nodes metastases and 7 of the 9 with marginal tumor excision previously, tumor size in 6 of the 9 were ≥5cm, the other 3 were 〈5 cm, Lymph nodes metastases rate was 52.9%, 7 with lymph node metastases developed distant metastases, including 2 with lymph node dissection and 5 without lymph node dissection.Six succumbed to the disease, including 4 of 13 who received chemotherapy and 2 of 4 without chemotherapy,tumor size were ≥5 cm in 5, 11 were alive （1 alive with disease） with a 2- and 5-year survival rate of 84% and 36% respectively. Conclusion Clear cell sarcoma is a rare and high-grade soft tissue sarcoma with poor prognosis, Correct operation as initial therapy plays the key role in treatment, The tumor size is a major factor in predicting prognosis, Tumor size and recurrence are closely related to metastases, Lymph nodes metastases predicted poor prognosis as hemat

关 键 词：肉瘤 透明细胞 临床方案 治疗结果 透明细胞肉瘤 治疗方法 疗效分析 区域淋巴结转移 5年生存率 肿瘤大小 手术治疗 

分 类 号：R738.6[医药卫生—肿瘤] R737.9[医药卫生—临床医学]