36例隆突性皮肤纤维肉瘤临床诊疗分析

Clinical analysis and treatment for 36 patients with dermatofibrosarcoma protuberans

目的:分析隆突性皮肤纤维肉瘤误诊率、复发率高的原因,探讨提高治愈率的策略。方法:回顾性分析36例隆突性皮肤纤维肉瘤患者的临床和病理资料。全组病例均行肿瘤局部扩大切除术,切缘距离肿瘤缘2.5～3.0cm,术中快速冷冻切片病理检查至边缘和基底阴性。创面行皮片或皮瓣移植修复,3例多次复发患者手术切除同时行125I放射性粒子组织间植入治疗。结果:临床首诊误诊率高达63.8%(23/36)。36例扩大切除后创面一期愈合率95%。中位随访时间3.4(1～6)年,共4例(11.1%)复发,3例辅以125I粒子植入治疗者,随访1～2年均未复发。全组病例未发现远处转移和死亡。结论:隆突性皮肤纤维肉瘤临床症状不典型是误诊率高的主要原因,病理检查是明确诊断的方法,手术扩大切除是提高治愈率的关键,放射性粒子组织间近距离治疗是较好的辅助疗法。

Objective：To study the reasons of misdiagnosis and postoperative recurrence of derrnatofibrosarcoma protuberan（DFSP） and find out effective ways to improve its cure rate. Methods：The clinical and pathological data of 36 patients with DFSP was submitted to a retrospective study. All 36 patients underwent wide excisions with intraoperative frozen sections. The surgical margins were between 2.5 and 3.0 era. Three patients with relapse history were treated with ^125I radioactive seeds implantation. Results：The clinical misdiagnosis rate at first diagnosis was high to 63.8%（23/36）. The operative wounds of all 36 extensive resections were covered with skin grafts or flap transplantations,and the primary healing rate was 95%. The median follow-up period was 3.4 （1-6）years and 4 （11.1%）cases recurred. Three patients with ^125I radioactive seeds implantation were followed up for 1 year to 2 years and none of them had recurrence. There were neither distant metastasis nor death in all cases. Conclusion：The high misdiagnosis rate of DFSP is mainly attributed to untypical early symptoms. The diagnosis of DFSP depends on pathological examination. Wide excision is the crucial measure to improve cure rate and interstitial brachytherapy is an effective adjunctive therapy.