A Multidisciplinary Approach to Complex Dermal Sarcomas Ensures an Optimal Clinical Outcome

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.

Oncological Safety and Recurrence in the Surgical Treatment of Atypical Fibroxanthoma and Pleomorphic Dermal Sarcoma of the Scalp

“…The resection was planned based on the clinical appearance, comorbidities, imaging, and pathology reports. The ultimate goal, however, must be clear margins [ 23 ], as incomplete initial resection causes local recurrence [ 24 , 25 ].…”

“…In the past, excision with 2 cm margins was recommended for both entities. However, to date, no guidelines for surgical treatment and follow-up have been established [ 23 ]. A recently published probability model based on a systemic review by Jibbe et al found peripheral excision margins of at least 2 cm for AFX and 3 cm for PDS, respectively, to reduce the recurrence rate [ 26 ].…”

“…This patient, later on, developed metastasis. Recent studies have reported that radiotherapy should not be used in cases of incomplete resection margins as a treatment modality to bypass large defects [ 23 ]. For patients treated at our department, the decision for adjuvant radiotherapy was made based on the interdisciplinary tumor board decision.…”

The Usefulness of Ultrasonography for Supporting the Differentiation, Diagnosis, and Treatment of Atypical Fibroxanthoma and Pleomorphic Dermal Sarcoma

“…Depending on the tissue of origin, these tumors can be classified into dermal, smooth muscle, or vascular lesions, having a vast spectrum regarding their malignant potential. Dermal sarcomas can therefore be divided into dermatofibrosarcoma protuberans (DFSP), atypical fibroxanthoma (AFX), pleomorphic dermal sarcoma (PDS), leiomyosarcoma (LMS), and vascular sarcomas (Kaposi's sarcoma, angiosarcoma) 1 …”

Computer analysis of genes associated with Kaposi's sarcoma

“…Treatment for Kaposi's sarcoma can be local or systemic. Local therapy includes applications of 30 % prospidin ointment, radiation methods, cryotherapy, injections of chemotherapeutic drugs into the tumor, applications with dinitrochlorobenzene, injections of interferon-alfa into the tumor, and some other methods [7]. Radiation therapy, local chemotherapy, surgical method, cryotherapy with liquid nitrogen, photodynamic therapy, systemic polychemotherapy or palliative monochemotherapy approaches also used [6].…”