Angiosarcomas are uncommon, aggressive, malignant neoplasms of vascular endothelial cell origin. They may arise in any organ or body site but have a predilection for skin and superficial soft tissues.

The most common form of angiosarcoma is cutaneous angiosarcoma (cAS). This is most frequently located in the head and neck regions of older individuals. There is a male preponderance, and risk factors for the development of angiosarcoma include a history of immunosuppression, sun exposure, foreign materials (eg, shrapnel, steel, plastic, Dacron) in the body, and environmental agent exposures such as arsenic and vinyl chloride. Secondary angiosarcomas can develop in sites of chronic lymphedema and radiation exposure, and this subtype may be seen in a younger population.

Angiosarcomas grow rapidly, recur locally, and metastasize widely. Prognosis is poor; 5-year survival rates are less than 20%. This is due to the biologic behavior of the tumor, delays in diagnosis, and poor impact on survival with currently available treatment options.

The appearance of angiosarcoma is highly variable. It may mimic an ecchymosis, cellulitis, rosacea, or facial edema. The tumor or plaque may bleed or ulcerate after minor trauma and grow rapidly. Satellite nodules may be present.

Angiosarcoma is exceedingly rare in children and is more likely to be visceral than cutaneous. Prognosis is abysmal.