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， with optimal treatment through surgery， radiotherapy and chemotherapy， recurrence and distant metastasis rate could be effectively reduced. This article reviews the recent progress of DFSP.

Key words：dermatofibrosarcoma protuberan;fibrosarcomatous dermatofibrosarcoma protuberans; Mohs microgrsphic surgery;dermoscopy; imatinib

隆突性皮膚纤维肉瘤是一种生长速度缓慢、起源于皮肤，并可累及皮下组织的低度恶性肿瘤，于1924年由Darier及Ferrandh首次报道[1-2]，DFSP病理改变主要呈“蟹足样”浸润生长，男性发病率略高于女性。除典型的DFSP外，还有纤维肉瘤型、粘液型、颗粒细胞型、色素型、硬化血管瘤样型、肌样型及萎缩型等十余种组织变异亚型，早期DFSP的临床症状不明显、皮损特异性低，容易被患者所忽视，加之部分医师经验不足时，临床上易误诊、漏诊。

既往文献指出，DFSP术后仍有较高的局部复发率，约为10%～60%，局部淋巴结转移及远处转移的几率分别为1%和4%～5%，晚期可转移至淋巴结、肺、肝、大脑及骨等。且有5%～15%的DFSP可出现纤维肉瘤（FS）转化，FS-DFSP的恶性程度更高，转移风险较典型DFSP增加10%～15%，预后不佳。此外，术中对切除范围及深度把握多有偏差时，其复发率可达30%～70%[3]，这也是DFSP的治疗难点。……