Cutaneous mesenchymal sarcomas are rare malignancies that include dermatofibrosarcoma protuberans, atypical fibroxanthoma, pleomorphic dermal sarcoma, cutaneous angiosarcoma, myofibrosarcoma, and leiomyosarcoma. These tumors lack consensus guidelines on staging and management. Treatment of local disease involves complete surgical removal but recurrence rates are higher compared with more common forms of nonmelanoma skin cancer. Cutaneous angiosarcoma, pleomorphic dermal sarcoma, and subcutaneous leiomyosarcoma have increased risk of metastatic spread and lower survival rate. Further research is needed on targeted therapies for these more aggressive sarcomas.
