目的    探讨甲磺酸伊马替尼治疗局部晚期、不可切除或远处转移隆凸性皮纤维肉瘤（DFSP）病人的疗效和安全性。 方法    回顾性分析2008年2月至2012年4月复旦大学附属肿瘤医院收治的10例DFSP病人的临床资料。10例DFSP病人中，2例原发肿瘤，4例复发肿瘤，4例转移肿瘤（2例肺转移和软组织转移，2例仅为软组织转移），所有病人甲磺酸伊马替尼治疗前均已病理确诊。甲磺酸伊马替尼初始剂量400 mg/d，耐药后加量至800 mg/d。结果    10例病人中，除1例出现原发耐药外，9例获得客观疗效，包括2例完全缓解，5例部分缓解，2例疾病稳定。全组DFSP病人1年无进展存活率为30%，1年和2年存活率分别为50%和30%。结论    甲磺酸伊马替尼是治疗局部晚期、不可切除或远处转移DFSP病人的有效治疗方式，靶向治疗可能有助于使病人获得根治切除，或降低致残的可能性。

Analysis of effect and safety of imatinib mesylate in the treatment of advanced, unresectable or metastatic dermatofibrosarcoma protuberans        WANG Chun-meng*, LUO Zhi-guo, CHEN Yong, et al. *Department of Gastric and Soft Tissue Surgery, Fudan University Shanghai Cancer Center; Department of Oncology, Shanghai Medical College, Fudan University, Shanghai 200032, China
Corresponding author: SHI Ying-qiang, E-mail: yingqiangshi@126.com
Abstract    Objective    To analyze the therapeutic effect and safety of patients with advanced, unresectable or metastatic dermatofibrosarcoma protuberans （DFSP）treated with imatinib mesylate. Methods    The clinical data of 10 cases of DFSP admitted between February 2008 and April 2012 in Fudan University Shanghai Cancer Center were analyzed retrospectively. In all 10 cases of DFSP, there were 2 cases of primary tumor, 4 cases of recurrent tumor and 4 cases of metastatic lesions (2 cases of lung metastases and soft tissue metastases, 2 cases of soft tissue metastases only). All cases had been confirmed pathologically before imatinib treatment. Imatinib was used 400 mg/d initially and escalated to 800 mg/d. Results    The overall responses were 2 cases of complete responses (20%),5 cases of partial responses (50%), 2 cases of stable diseases (20%) and 1 case of progressive diseases (10%).The 1-year progression-free survival (PFS) rate was 30%. The 1-year overall survival and 2-year overall survival (OS) rate was 50% and 30% respectively. Conclusion    Imatinib therapy is the effective treatment in advanced, unresectable or metastatic DFSP cases currently, and the targeted therapy may potentially facilitate resection or decrease possible disfigurement.