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目的总结转化型隆凸性皮肤纤维肉瘤(DFSP)的临床病理特征,探讨规范化的手术方法和综合治疗途径。方法对复旦大学上海医学院附属肿瘤医院1985年1月至2006年12月收治的34例转化型DFSP病例,进行临床和病理资料的回顾性分析。结果34例转化型DFSP中,伴有纤维肉瘤样转化者(DFSP-FS)32例,伴有恶性纤维组织细胞瘤样转化者(DFSP-MFH)2例,占同期收治手术治疗的260例DFSP的13.1%。经中位随访49个月后,发现其广泛切除手术后局部复发率较传统型DFSP的复发率明显增高(P<0.0001),且复发灶较大(P=0.009),复发时间较短(P=0.012),淋巴结和血道等远处转移的发生率高(P<0.0001),5年和10年存活率明显比传统型DFSP差(P<0.0001)。结论转化型DFSP的恶性程度较高,预后较差,临床医师必须引起重视并采用更积极的治疗方案。及时和规范化的广泛切除手术非常重要。术后辅助放疗等综合治疗的应用也常需考虑。
Objective To summarize the clinical and pathological features of transformed proton -ofacial fibrosarcoma (DFSP) and discuss the standardized surgical methods and comprehensive treatment approaches. Methods A retrospective analysis of clinical and pathological data was performed on 34 cases of transformed DFSP cases admitted to the Tumor Hospital Affiliated to Shanghai Medical College, Fudan University from January 1985 to December 2006. Results Thirty-two cases of DFSP-transformed DFSP had DFSP-FS with 32 cases and DFSP-MFH with 2 cases, which accounted for 260 cases of DFSP Of the 13.1%. After a median follow-up of 49 months, it was found that the local recurrence rate after extensive resection was significantly higher than that of the traditional DFSP (P <0.0001), with a larger recurrence (P = 0.009) and shorter recurrence (P = 0.012). The incidence of distant metastasis such as lymph nodes and bloodstream was high (P <0.0001). The 5-year and 10-year survival rates were significantly lower than those of conventional DFSP (P <0.0001). Conclusions Transforming DFSP is more malignant and has a worse prognosis. Clinicians must pay attention to and adopt a more aggressive treatment regimen. Timely and standardized extensive resection is very important. Postoperative adjuvant radiotherapy and other comprehensive treatment applications often need to be considered.