Angiosarcomas are rare, 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 may be primary, in which case it is most frequently located on the head or neck, particularly the scalp, of older individuals, more frequently men (ratio of 2:1); or secondary. In secondary cases, cAS develops in sites of chronic lymphedema or radiation exposure, and this subtype may be seen in a younger population. This type of cAS has also been termed lymphangiosarcoma.

Angiosarcomas grow rapidly, recur locally, and metastasize widely. Five-year survival rates of primary cAS range from 11%-50%. 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 ecchymosis or hematoma, cellulitis, rosacea, or facial edema, and this may lead to delay in diagnosis. The tumor or plaque may bleed or ulcerate after minor trauma and grow rapidly. Satellite nodules may be present.

Symptomatic thrombocytopenia (Kasabach-Merritt syndrome) has been seen in cases of angiosarcoma.

Pediatric patient considerations: Angiosarcoma is exceedingly rare in children and is more likely to be visceral than cutaneous. The prognosis is poor.