Cutaneous sarcomas

Summary Cutaneous sarcomas comprise a heterogeneous group of mesenchymal spindle cell tumors of the dermis and subcutis, one of the best‐known entities being dermatofibrosarcoma protuberans. Other sarcomas addressed in this review include atypical fibroxanthoma, cutaneous undifferentiated pleomorphic sarcoma, leiomyosarcoma, liposarcoma, and angiosarcoma. With the exception of dermatofibrosarcoma protuberans, which has its peak incidence in middle‐aged adults, cutaneous sarcomas usually occur in elderly individuals starting in the sixth or seventh decade of life. The pathogenesis of the various disease entities is not uniform and has not been definitively ascertained. Histology is the key to arriving at a correct diagnosis, and should always include ruling out other dermal neoplasms such as melanoma. In recent years, molecular genetic methods have provided greater insight into the pathogenesis, thus paving the way for new targeted therapies. Treatment of choice for cutaneous sarcomas is excision with sufficient surgical margins. Adjuvant and neoadjuvant therapeutic concepts include radiation therapy and the use of targeted therapies or chemotherapies. Local recurrences have frequently been reported in cutaneous sarcomas. Unlike soft tissue sarcomas, the prognosis in terms of survival – with the exception of angiosarcoma – is very good if treated adequately, a fact that should be emphasized to patients.