透明细胞肉瘤的临床治疗与预后分析

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-2009年连续治疗的38例病理证实透明细胞肉瘤的患者进行回顾性分析.结果 全部病例随访时间4-242个月（平均34个月）.发生转移26例,局部复发9例.全部病例的5年复发率23.7%,5年和10年转移率分别为61.0%和68.4%,转移风险累积明显大于局部复发（P=0.004）.转移、局部复发、死亡的中位时间为分别为13个月,19个月和20个月.全部病例的5年、10年生存率分别为47%、36%.单因素分析结果显示性N（P=0.018）,肿瘤大小（P=0.001）,肿瘤深度（P=0.002）,TNM分期性（P=0.001）及手术切缘（P=0.042）可能成为预后因素.而化疗（P=0.18）、放疗（P=0.06）与肿瘤预后无明显相关.在多变量分析中,只有肿瘤大小（P=0.021）成为独立的预后因素.结论 早期诊断和首次广泛切除是改善透明细胞肉瘤预后的关键.肿瘤大小与转移关系密切,是影响预后的重要因素.

Treatment and prognosis analysis of clear cell sarcoma

Objective To investigate the clinical features of and the treatment for clear cell sarcoma （CCS）, and to analyse the efficacy and and prognosis factors. Methods A total of 38 consecutive patients who were confirmed to have CCS with histological test were treated between 1990 and 2009. They were studied retrospectively. Results All patients were followed up for 4 though 242 months, 34 months on average. 26 patients were confirmed to have metastasis. 9 had residual recurrence. The overall 5-year recurrence rate was 23.7%. The 5-year and 10-year metastasis rate was 61.0% and 68.4%, respectively. The accumulated risk of metastasis was significantly higher than that of residual recurrence （P=0.004）. The median time of metastasis, recurrence, and death was 13 months, 19 months and 20 months, respectively. The 5-year and 10-years overall survival rates were 47% and 36%, respectively. According to uni- variant analysis, sex （P=-0.018）, tumor size （P=0.001）, tumor invasion depth （P=0.002）, TNM classification （P=-0.001） and positive surgical margin （P=0.042） were identified as significant prognostic factors. Chemotherapy （P=O.18）, radiotherapy （P=0.06） were not significantly related to prognosis. Multi-variant analysis indicated the tumor size was the only independent prognosis factor （P=-0.021）. Conclusions Early diagnosis and extensive excision were crucial to improving the prognosis of clear cell sarcoma. The tumor size was closely related to metastasis, and was a key factor for prognosis.