纤维肉瘤型隆突性皮肤纤维肉瘤的临床特征分析

Analysis of clinical features of fibrosarcomatous dermatofibrosarcoma protuberans

目的:探讨纤维肉瘤型隆突性皮肤纤维肉瘤(fibrosarcomatous dermatofibrosarcoma protuberans,FS-DFSP)的临床特征、治疗方法及预后因素。方法:收集天津市黄河医院自2004年6月至2014年6月收治的经病理学证实的18例FS-DFSP,分析包括年龄、性别、部位、既往非计划手术次数、肿瘤大小、深浅、末次手术切缘、化疗、复发、转移、生存时间等因素。结果:18例患者均接受手术治疗,17例R0,1例R1,局部复发2例,R1切缘患者术后放疗。12例患者(肿瘤基底深在、和/或肿瘤最长径≥5 cm)术后接受MAID方案化疗,无化疗期间病情进展病例,化疗结束后病情进展(肺转移)3例,肺转移伴局部复发1例。2例接受甲磺酸伊马替尼治疗,疗效为SD。患者的2年生存率为93%,5年生存率为79%。单因素分析结果显示性别、年龄、部位、肿瘤大小、深度、复发、手术切缘、化疗等因素与总生存时间无关,而既往非计划手术次数和转移与总生存时间相关。多因素分析显示各临床因素均不是总生存的独立预后因素。结论:手术彻底切除肿瘤降低复发率是治疗FS-DFSP的关键,肿瘤的复发及转移是影响预后的重要因素。

Objective： To investigate the clinical features, therapeutic method, and prognostic factor of fibrosarcomatous dermato- fibrosarcoma protuberans （FS-DFSP）. Methods： Data of 18 FS-DFSP cases treated in the Department of General Surgery, Tianjin Huanghe Hospital between June 2004 and June 2014 were retrospectively analyzed. Research indexes included age, sex, pathogenic site, number of previous unplanned surgeries, tumor size, depth, incisal margin of the last surgical procedure, chemotherapy, relapse and metastasis, and survival time. Univariate and multivariate analyses of the research indicators were conducted in these cases. Results： All 18 patients underwent surgery, and the incisal margins were R0 in 17 cases and R1 in 1 case, with local recurrence in 2 of the 18 cas- es. The patient with RI incisal margin underwent postoperative radiotherapy. Among all the patients, 12 with tumor base depth and/or largest tumor diameter of 〉5 cm accepted the chemotherapy of mesna, adriamycin, ifosfamide, and dacarbazine, also known as MAID regimen, after surgery. No progression of disease occurred during chemotherapy. After chemotherapy, pulmonary metastasis occurred in 3 of the 12 cases, and pulmonary metastasis with local recurrence existed in 1 case. Two of the 3 patients with pulmonary metastasis were treated with imatinib mesylate, and the therapeutic effect stabilized the disease. Two-year survival rate was 93%, and 5-year sur- vival rate was 79% in total patients. The results of mono-factorial analysis indicated that clinical factors, such as age, sex, pathogenic site, tumor size, depth, recurrence, incisal margin of surgical operation, and chemotherapy, were unrelated to the overall survival （OS） time. The number of previous unplanned surgeries and metastasis are related to OS. The results of multiple factor analysis showed that none of the clinical factors were independent prognostic factors for OS. Conclusion： A thorough removal of tumor can reduce the recur- rence rate, which is the key point in FS-DFSP treatment. Recurrence and metastasis of tumor are significant factors affecting prognosis.