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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400mg/d,耐药后加量至800mg/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。
Objective To investigate the efficacy and safety of imatinib mesylate in the treatment of locally advanced, unresectable or distantly metastatic proton fibromuscular sarcoma (DFSP) patients. Methods The clinical data of 10 DFSP patients admitted from Cancer Hospital of Fudan University from February 2008 to April 2012 were retrospectively analyzed. Of the 10 DFSP patients, 2 had primary tumor, 4 had recurrent tumor, 4 had metastatic tumor (2 lung metastases and soft tissue metastases, 2 had soft tissue metastases only), and all patients had imatinib mesylate before treatment Pathological diagnosis. The initial dose of imatinib mesylate 400mg / d, the dose increased to 800mg / d. Results Of the 10 patients, except for 1 case of primary drug resistance, 9 patients achieved objective curative effect, including 2 complete remission, 5 partial remission and 2 stable disease. The 1-year progression-free survival rate for all DFSP patients was 30%, with 1- and 2-year rates of 50% and 30%, respectively. Conclusion Imatinib mesylate is an effective treatment for patients with locally advanced, unresectable or distant metastatic DFSP. Targeted therapies may help patients achieve radical resection or reduce the likelihood of disability.