Current understanding of angiosarcoma: disease biology and evolving treatment

Angiosarcoma is a very rare soft tissue sarcoma that originates from endothelial cells and typically has a poor prognosis. It is most commonly found in elderly white men and can occur anywhere in the body, particularly in the head, neck, and scalp. Patients who have undergone previous radiation treatment or who have chronic lymphedema also face an elevated risk of this condition. Various genetic changes are suspected to contribute to the development of angiosarcoma, and these changes have been identified as potential targets for treatment. For localized disease, wide surgical resection is often the prudent course of action. A multidisciplinary approach, which may include surgery, radiotherapy, systemic chemotherapy, or immunotherapy, is typically the most effective way to achieve favorable outcomes. In this review, we discuss the general understanding of angiosarcoma and its management, with a particular focus on the current evolving treatments for the disease.