隆突性皮肤纤维肉瘤的诊断及治疗进展

Progress in the diagnosis and treatment of dermatofi brosarcoma protuberans

隆突性皮肤纤维肉瘤(DFSP)是一种罕见且具有侵袭性的软组织肉瘤,起源于皮肤的真皮层。生长缓慢,转移潜能低,常侵袭皮下组织,甚至可达肌肉。尽管DFSP罕见,其高复发率仍是对临床的重大挑战。目前组织病理学诊断仍是DFSP的重要确诊手段。首选治疗方式为外科手术治疗,慢Mohs显微外科手术(sMMS)通过精确切除肿瘤组织而保留健康组织,改变了DFSP的手术方式。新兴疗法如靶向治疗、免疫治疗和基因治疗也显示出巨大潜力。现对DFSP规范化诊疗方案及最新治疗研究进行阐述,以期待为患者带来更好的治疗方案。

Dermatofi brosarcoma protuberans(DFSP)is a rare and aggressive soft tissue sarcoma that originates in the dermis of the skin.Characterized by slow growth and low metastatic potential,DFSP often invades subcutaneous tissues,and can even reach the muscles.Although DFSP is rare,its high recurrence rate remains a major challenge for clinical practice.Histopathological diagnosis is still an important diagnostic method for DFSP.The fi rst treatment method chosen is surgical treatment.Slow Mohs micrographic surgery(sMMS)changes the surgical approach of DFSP by accurately excising tumor tissue while conserving healthy tissue.Emerging treatments such as targeted therapy,immunotherapy,and gene therapy have also shown great potential.This review elaborates on the standardized diagnosis and treatment plan for DFSP and the latest treatment research,in order to provide better treatment options for the patients.