透明细胞肉瘤的治疗和预后

Clinical treatment and prognosis for dear cell sarcoma： analysis of 19 cases

目的探讨透明细胞肉瘤的临床特点、治疗方法和预后。方法对1973年3月至2007年3月收治的19例透明细胞瘤患者进行回顾性分析。其中男12例，女7例，平均年龄30．9岁。发病部位：上肢5例，手2例，下肢6例，足2例，躯干3例，头部1例。肿瘤大小在5cm以上者3例，〈5cm者16例。19例均在外院行肿物局部切除术，10例局部复发，2例有区域淋巴结转移，1例肺转移，1例骨转移。局部复发的10例再行手术治疗。联合使用化疗8例，放疗8例，1例术后联合放化疗。结果所有患者随访3～144个月，平均51．4个月。10例再手术的患者中，1例复发，3例淋巴结转移，2例肺转移。放疗或化疗而未再手术患者9例，7例淋巴结转移，6例肺转移，6例死亡。总的5年生存率为75．2％，10年生存率为37．5％。结论透明细胞肉瘤恶性程度高，容易复发和转移，预后差；局部复发后再次手术是最佳选择。透明细胞肉瘤的放化疗疗效有待进一步研究。

Objective To explore the clinical features, prognosis, and optimal treatment strategy of clear cell sarcoma （CCS）. Methods Nineteen patients, 12 males and 7 females, aged 30. 9, with histologically confirmed CCS, 3 with tumors t〉5 cm and 16 with tumors 〈5 cm, 5 tumors being located in the upper extremity, 2 in the hand, 6 in the lower extremity, 2 in the foot, 3 in the trunk, and 1 in the head, were hospitalized from March 1973 to March 2007. The primary tumors of all the cases were marginally excised before admission and 10 cases had local relapse at admission. Two presented local lymph node metastasis and 2 presented distant metastasis. Ten patients with tumor relapse underwent re-operation, Eight patients received chemotherapy, 8 radiotherapy and 1 combined chemotherapy and radiotherapy. The patients were followed up for 51.4 months （3 -144 months ）. Results Tumor recurrence occurred in 1 of the 10 patients who received re-operation. 3 patients developed lymph node metastasis and 2 developed pulmonary metastasis. Of the 9 patients who did not undergo re-operation 7 developed lymph node metastasis, 6 developed pulmonary metastasis and 6 died. The overall 5-year survival rate was 75.2% and the 10-yeat survival rate was 37.5%. Conclusion CCS is a rare and high grade soft tissue sarcoma with high incidence rates of local recurrence and metastasis, and poor prognosis. The role of chemotherapy and radiotherapy for CCS should be investigated further. The best choice after local recurrence is re-operation.