Cutaneous sarcoma: a review and practical approach to management

Abstract

Sarcomas arising in the skin are rare but potentially fatal. These tumours originate from mesenchymal cells and can be divided between those that arise in soft tissue and those arising from bone. General guidelines exist for the management of soft tissue sarcomas however there are no specific guidelines for cutaneous sarcomas. Current literature was reviewed for management of seven cutaneous sarcomas including atypical fibroxanthoma (AFX), pleomorphic dermal sarcoma (PDS), dermal and subcutaneous leiomyosarcoma (LMS), dermatofibroma sarcoma protuberans (DFDP), Kaposi’s sarcoma (KS), cutaneous angiosarcoma (CAS) and malignant peripheral nerve sheath tumour (MPNST). All suspected sarcomas should be discussed in a sarcoma multidisciplinary team (MDT) meeting. This article is not a clinical guideline but should serve as a practical summary of how these tumours present, how they are recognised histologically, and how best to manage and follow up patients. The aim is to support clinicians and facilitate the best and most evidence-based standard of care available.

The Neutrophil-to-Lymphocyte Ratio as a Biomarker in Cutaneous Oncology: A Systematic Review of Evidence beyond Malignant Melanoma

“…Cutaneous sarcomas are the subset of the soft tissue sarcomas (STSs) that originate from the connective tissue skeleton of the skin, the dermis, and the subcutis [ 55 , 56 , 57 ]. They comprise a clinically and morphologically highly heterogeneous family of mesenchymal spindle cell tumors of varying pathogenesis, that includes clinical entities as variables in their clinical behavior as dermatofibrosarcoma protuberans, atypical fibroxanthoma, cutaneous undifferentiated pleomorphic sarcoma, leiomyosarcoma, liposarcoma, Kaposi’s sarcoma, and malignant peripheral nerve sheath tumor.…”

“…Unlike the overall poor prognosis of most non-cutaneous STSs, with adequate treatment the prognosis in terms of disease-specific mortality of most dermal sarcomas is excellent with the main therapeutic challenge being their trend to exhibit local recurrences. However, biologically aggressive entities do also exist, with the most characteristic being the angiosarcomas [ 55 , 56 , 57 ]. Apart from cutaneous angiosarcomas, no studies were found in the literature that report on the association of NLR or similar hematologic biomarkers and the clinical features of cutaneous sarcomas.…”