Cutaneous angiosarcoma

Abstract

Cutaneous angiosarcoma (cAS) is a rare but highly aggressive malignant vascular neoplasm originating from endothelial cells and accounting for approximately 1–2% of all soft tissue sarcomas. The disease predominantly affects elderly individuals and most commonly involves the skin of the head and neck region. Cutaneous angiosarcoma may occur sporadically (Wilson–Jones type) or in association with chronic lymphedema (Stewart–Treves syndrome) and previous radiation exposure. Early diagnosis remains challenging because its clinical manifestations are highly variable and often mimic benign inflammatory, vascular, or traumatic conditions, leading to delayed recognition and treatment. This review summarizes the current understanding of cutaneous angiosarcoma, including its epidemiology, risk factors, pathogenesis, clinical manifestations, diagnostic evaluation, management, prognosis, and follow-up. Diagnostic approaches rely on a combination of clinical assessment, dermoscopy, histopathological examination, immunohistochemistry, and imaging studies to establish the diagnosis and determine disease extent. Surgical resection remains the mainstay of treatment and is frequently combined with radiotherapy and systemic therapies for advanced disease. Novel therapeutic modalities, including targeted therapy, immunotherapy, and electrochemotherapy, have emerged as promising options for selected patients. Despite advances in treatment, the prognosis of cutaneous angiosarcoma remains poor because of its aggressive behavior, high recurrence rate, and metastatic potential. Increased awareness and early recognition are essential to facilitate timely diagnosis and optimize patient outcomes.

Keywords: angiosarcoma, cutaneous angiosarcoma, soft tissue sarcoma