LAUSR

Simple Summary Knowledge of cutaneous or dermal sarcomas is crucial due to the initially peaceful appearance of skin tumours, causing delays in accurate diagnoses. They can easily be mistaken for benign skin appendages or moles. This clinical entity spans from low-grade tumours with practically no metastatic potential to highly aggressive neoplasms with substantial morbidity. Initial adequate resection is the absolute mainstay of successful therapy. Extensive tissue defects after resection necessitate a multidisciplinary approach involving reconstructive surgical planning from onset. Clinical and morphological types of dermal sarcomas are presented together with examples of cases undergoing interdisciplinary treatment and advanced microsurgical reconstructive therapy. Clinicians should be aware of the types of dermal sarcomas; their specific clinical presentations and growth patterns; as well as the necessity of clear surgical margins and the significant progress in reconstructive surgery, which gives new hope for future management of complex dermal sarcomas. Abstract Primary dermal sarcomas (PDS) belong to a highly clinically, genetically and pathologically heterogeneous group of rare malignant mesenchymal tumours primarily involving the dermis or the subcutaneous tissue. The tumours are classified according to the mesenchymal tissue from which they originate: dermal connective tissue, smooth muscle or vessels. Clinically, PDS may mimic benign soft tissue lesions such as dermatofibromas, hypertrophic scarring, etc. This may cause substantial diagnostic delay. As a group, PDS most commonly comprises the following clinicopathological forms of dermal sarcomas: dermatofibrosarcoma protuberans (DFSP), atypical fibroxanthoma (AFX), dermal undifferentiated pleomorphic sarcoma (DUPS), leiomyosarcoma (LMS), and vascular sarcomas (Kaposi’s sarcoma, primary angiosarcoma, and radiation-induced angiosarcoma). This clinical entity has a broad spectrum regarding malignant potential; however, local aggressive behaviour in some forms causes surgical challenges. Preoperative, individualised surgical planning with complete free margins is pivotal along with a multidisciplinary approach and collaboration across highly specialised surgical and medical specialties. The present review gives a structured overview of the most common forms of dermal sarcomas including surgical recommendations and examples for advanced reconstructions as well as the current adjunctive medical treatment strategies. Optimal aesthetic and functional outcomes with low recurrence rates can be achieved by using a multidisciplinary approach to complex dermal sarcomas. In cases of extended local tumour invasion in dermal sarcomas, advanced reconstructive techniques can be applied, and the interdisciplinary microsurgeon should be an integral part of the sarcoma board.