Abstract
Cutaneous angiosarcoma is a rare and aggressive malignant tumor that originates from the endothelial cells of blood vessels or lymphatic vessels. More than half of cutaneous angiosarcoma cases occur in the head and neck regions, particularly on the scalp. However, due to its rarity, scalp angiosarcoma is often overlooked in clinical practice. The lack of distinctive symptoms usually delays the diagnosis and, implicitly, the initiation of appropriate treatment. The treatment of cutaneous angiosarcoma poses great challenges due to its multifocal occurrence and the frequent extensive microscopic spread. A personalized, multimodal therapeutic approach is essential for ensuring a favorable outcome, consisting of a wide surgical excision associated with adjuvant radiotherapy in localized tumors, concurrent adjuvant radiotherapy and chemotherapy, targeted treatments, or immunotherapy in advanced or metastatic diseases. The aim of this manuscript is to review the literature data regarding the individualized management of cutaneous angiosarcoma and to share our own experiences in the field. We wish to underscore the importance of considering cutaneous angiosarcoma in the differential diagnosis of scalp tumors, especially in patients with a history of scalp irradiation as early detection, accurate diagnosis, and a multidisciplinary, personalized management, including surgery with clear margins and adjuvant radiation therapy, are crucial for ensuring a favorable outcome.