刘珍如 周园 刘梦茜 王晓晴

    [摘要]隆突性皮肤纤维肉瘤(Dermatofibrosarcoma protuberans，DFSP)是一种低度恶性肿瘤，早期DFSP临床症状不典型、特异性低，导致临床医师容易漏诊、误诊，此外，DFSP经手术治疗后仍有高复发率，部分病变更会出现纤维肉瘤化改变，增加其转移风险、并提示预后不佳。近年来，许多研究开始关注DFSP的诊断及治疗，透过皮肤镜、病理活检帮助医师早期诊断，提高疾病检出率，并通过手术、放疗及化疗等治疗，有效降低病变复发率、避免远处转移，本文就DFSP的最新治疗进展作一综述。

    [关键词]隆突性皮肤纤维肉瘤;纤维肉瘤样隆突性皮肤纤维肉瘤;莫氏手术;皮肤镜;伊马替尼

    [中图分类号]R739.5? ? [文献标志码]A? ? [文章编号]1008-6455(2021)03-0171-04

    Research Progress in Diagnosis and Treatment of Dermatofibrosarcoma Protuberans

    LIU Zhen-ru，ZHOU Yuan，LIU Meng-xi，WANG Xiao-qing，WANG Da-guang

    (Department of Dermatology， the First Affiliated Hospital of Nanjing Medical University， Nanjing 210029，Jiangsu，China)

    Abstract： Due to the atypical and low specificity clinical symptoms in early stage，Dermatofibrosarcoma protuberans (DFSP) is a low grade malignant tumor which might likely be misdiagnosis. In addition， even after surgical treatment DFSP still has a high recurrence rate. Lesions with fibrosarcomatous changes were associated with increased the risk of metastasis and poor prognosis. In recent years， many studies have begun to focus on the diagnosis and treatment of DFSP. Recent study showed with dermoscopy and pathological biopsy can help with early diagnosis and signicfanctly improve the detection rate. Also ......