It was classically attributed to lymphedemas induced by radical mastectomy to treat breast cancer. Nowadays, we know that it can arise in chronically lymphedematous regions of any cause .
The incidence is unknown, but studies have shown that it occurred at a rate of between 0.03% and 0.45% in patients who survive at least 5 years after radical mastectomy .
It can arise from any cause of chronic lymphedema :
- mastectomy induced lymphedema
- Milroy disease
- congenital lymphedema
- traumatic lymphedema
- filarial lymphedema
- elephantiasis nostras verrucosa
- idiopathic lymphedema
Chronic lymphedema seems to induce a degree of local immunodeficiency that leads to oncogenesis .
Treatment and prognosis
The prognosis is very poor because angiosarcomas are very aggressive tumors that frequently recur locally and often metastasize early.