Wollina Uwe, Langner Dana, Schönlebe Jacqueline, França Katlein, Lotti Torello, Tchernev Georgi
Städtisches Klinikum Dresden - Department of Dermatology and Allergology, Dresden, Sachsen, Germany.
Städtisches Klinikum Dresden - Department Dermatology and Allergology, Dresden, Germany.
Open Access Maced J Med Sci. 2018 Jan 10;6(1):35-37. doi: 10.3889/oamjms.2018.030. eCollection 2018 Jan 25.
Dermatofibrosarcoma protuberans (DFSP) is rare mesenchymal neoplasia with a high risk of local recurrence but a low risk of metastatic spread. Tumor cells express CD34 and show a characteristic translocation t(17;22)(q22;q13). We analysed the documented cases at the Department of Dermatology and Allergology between 08/2001 and 08/2017. The diagnosis had been confirmed by histology and immunohistology in all cases. We identified four adults and a pediatric patient with DFSP. All patients were treated by wide surgical excision and controlled by three-dimensional histologic margin control. We observed no recurrence and no metastatic spread. We discuss prognostic factors and emerging treatments.
隆突性皮肤纤维肉瘤(DFSP)是一种罕见的间叶性肿瘤,局部复发风险高但转移扩散风险低。肿瘤细胞表达CD34,并显示特征性易位t(17;22)(q22;q13)。我们分析了2001年8月至2017年8月期间皮肤科和变态反应科记录的病例。所有病例均经组织学和免疫组织学确诊。我们确定了4名成年患者和1名儿童DFSP患者。所有患者均接受了广泛手术切除,并通过三维组织学切缘控制进行监测。我们未观察到复发和转移扩散情况。我们讨论了预后因素和新出现的治疗方法。