摘要隆突性皮肤纤维肉瘤是一种发生于皮肤和皮下的间叶细胞恶性肿瘤,以局部复发率高和远处转移率低为特点.近年研究发现,除了组织病理和免疫组化可诊断隆突性皮肤纤维肉瘤外,细胞遗传学、分子生物学、影像学及皮肤镜和皮肤CT(反射式的激光共聚焦显微镜)均对隆突性皮肤纤维肉瘤的诊断、治疗及评估起到重要的辅助作用.目前,对于隆突性皮肤纤维肉瘤的手术治疗为扩大切除手术或Mohs显微外科手术,两种方法各有利弊,术后可辅助放疗、免疫综合治疗;冷冻治疗对复发患者有效;对不能手术或手术切缘阳性的患者需联合靶向药物伊马替尼的治疗.另外,抗雌激素(如他莫昔芬)或新型靶向药物乙酰肝素酶抑制剂roneparstat(SST0001)可能也有一定疗效.

abstractsDermatofibrosarcoma protuberans (DFSP) is a rare cutaneous and subcutaneous mesenchymal malignant tumor characterized by high rates of local recurrence and low risk of metastasis.Histopathological and immumohistochemical study can be used to diagnose DFSP.Recent studies have shown that cell genetics and molecular biology,imagiology,dermatoscopy and cutaneous computed tomography (reflectance confocal microscopy) can also play an important auxiliary role in the diagnosis,treatment and evaluation of DFSP.At present,the surgical treatment of DFSP mainly includes wide local excision (WLE) and Mohs micrographic surgery (MMS).Each of the two approaches has its advantages and disadvantages.After surgery,radiation therapy and comprehensive immunological treatment can serve as adjuvant treatment.Cryotherapy is effective in patients with recurrent DFSP,and the targeted agent imatinib can be combined for inoperable patients or patients with positive surgical margins.In addition,antiestrogenic drugs (such as tamoxifen) or the new targeted agent heparanase inhibitor roneparstat (SST0001) may have some therapeutic effects.