隆突性皮肤纤维肉瘤发病机制及治疗进展

Advances in pathogenesis and treatment of dermatofibrosarcoma protuberans

隆突性皮肤纤维肉瘤(DFSP)是一种起源于成纤维细胞的局部侵袭性皮肤软组织肉瘤, 局部复发率较高, 转移率低。目前确诊经典型DFSP主要依靠组织病理学检查。治疗上仍以肿瘤局部广泛切除的方式为主, 但效果更好的Mohs显微外科手术已经开始被越来越多的临床医师采用。辅助治疗包括分子靶向治疗、放疗、化疗、冷冻消融术等, 维甲酸类药物及抗血管生成药物阿帕替尼有可能为治疗DFSP的潜在药物。该文主要对DFSP的发病机制、临床表现、诊断及治疗等进行了综述, 为临床诊治DFSP提供参考。

Dermatofibrosarcoma protuberans(DFSP)is a kind of locally invasive skin soft tissue sarcoma originating from fibroblasts.DFSP has a high local recurrence rate and a low metastatic rate.The diagnosis of typical DFSP is mainly based on histopathological examination.The treatment of DFSP is still dominated by extensive local resection of the tumor,but Mohs microsurgery,which is more effective,has been adopted by more and more clinicians.Adjunctive therapy for DFSP includes molecular targeted therapy,radiotherapy,chemotherapy and cryoablation etc.Retinoic acid drugs and antiangiogenic drug Apatinib have been reported as potential therapeutic agents for DFSP.In this review,the pathogenesis,clinical manifestations,diagnosis and treatment of DFSP are reviewed to provide reference for clinical diagnosis and treatment of DFSP.