隆突性皮肤纤维肉瘤是一种较为少见的起源于皮肤真皮层并可扩展至皮下软组织的局限性低度恶性的纤维肉瘤,临床以缓慢局部浸润性生长、手术切除后易复发为特征。目前发现其有10多个亚型。由于其形态比较复杂,极易与多种良恶性软组织肿瘤相混淆,但它们治疗方式、预后相差很大,因此需要准确的病理诊断。本文着重就隆突性皮肤纤维肉瘤各个类型的临床病理特点、分子遗传学及治疗方面研究进展作一概述。 Subcutaneous dermatofibrosarcoma is a relatively rare low-grade fibrosarcoma that originates in the dermis of the skin and extends to subcutaneous soft tissue. It is characterized by slow, locally invasive growth and a relapse after surgical resection. It is found that there are more than 10 subtypes. Because of its complex morphology, easily confused with a variety of benign and malignant soft tissue tumors, but their treatment, the prognosis varies widely, so the need for accurate pathological diagnosis. This article focuses on the various types of clinical manifestations, molecular genetics and treatment of tubullary fibrosarcoma in order to give an overview of the research progress.