Ugurel S
Klinik und Poliklinik für Dermatologie, Venerologie und Allergologie, Universität Würzburg, Josef-Schneider-Strasse 2, 97080 Würzburg, Deutschland.
Hautarzt. 2008 Nov;59(11):933-9; quiz 940. doi: 10.1007/s00105-008-1501-7.
Dermatofibrosarcoma protuberans (DFSP) is a rare tumor but still the most common cutaneous sarcoma. DFSP is a tumor of fibroblastic origin, characterized by a slow, undermining and locally destructive growth pattern, which only rarely metastasizes. The clinical appearance, especially of smaller lesions, is often not characteristic, so that diagnosis is often made late and only on biopsy findings. The standard treatment of DFSP is excision with safety margins of 2 to 3 cm. If smaller margins are employed, the risk of local relapse is high. Surgically incurable or metastatic DFSP can be irradiated; the cells are generally radiation-sensitive. 90% of DFSP carry a chromosome translocation of 17 and 22, harboring a gene fusion, which results in a continuous activation of the PDGFbeta signal transduction pathway. This finding led the way to a new molecular targeted therapy of DFSP using inhibitors of the PDGFbeta pathway. The first drug to be registered for targeted treatment of locally advanced or metastasized DFSP is the multikinase inhibitor imatinib, showing a response of about 70% in clinical trials.
隆突性皮肤纤维肉瘤（DFSP）是一种罕见肿瘤，但仍是最常见的皮肤肉瘤。DFSP是一种成纤维细胞起源的肿瘤，其特征为生长缓慢、侵袭性且具有局部破坏性，很少发生转移。临床表现，尤其是较小病变的表现，通常不具有特征性，因此诊断往往较晚，且通常仅基于活检结果。DFSP的标准治疗方法是切除，切缘安全距离为2至3厘米。如果切缘较小，局部复发风险较高。无法通过手术治愈或发生转移的DFSP可进行放疗；其细胞通常对放疗敏感。90%的DFSP存在17号和22号染色体易位，伴有基因融合，这导致血小板衍生生长因子β（PDGFbeta）信号转导通路持续激活。这一发现为使用PDGFbeta通路抑制剂对DFSP进行新的分子靶向治疗开辟了道路。首个获批用于局部晚期或转移性DFSP靶向治疗的药物是多激酶抑制剂伊马替尼，在临床试验中显示出约70%的缓解率。
