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:Try to analyze the clinical and pathological characteristics in patients with transformed dermatofibrosarcoma protuberans (DFSP) and discuss the standard surgical treatment and combined treatments for them. Methods:Medical charts of 34 patients with histologically confirmed transformed DFSP treated between January 1985 and December 2006 in Cancer Hospital of Fudan University were submitted to a retrospective study. Results:Among the 34 cases,32 had high grade fibrosarcomatous change (DFSP-FS) and 2 had malignant fibrous histiocytoma change (DFSPMFH).Compared with classical DFSPs,these transformed DFSPs had significantly higher recurrence rate after wide excision (P<0.0001),and the sizes of the recurrent tumors were larger (P=0.009),the durations of recurrence were shorter (P=0.012).Also they had higher metastatic rate (P<0.0001) and worse survival rates (P<0.0001). Conclusion:Transformed DFSPs are more aggressive tumors which need more active treatments.In order to reduce the local recurrence rate and decrease the degeneration into more malignancy caused by multiple local recurrences of the tumor,adequate initial resection and standard wide excision are more important for patients with transformed DFSPs.Combined treatments including adjuvant radiotherapy may be an optimal therapeutic strategy.