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 2.
The incidence is unknown, but studies have shown that it occurred in a rate between 0.03% and 0.45% in patients who survive at least 5 years after radical mastectomy 5,6.
It can arise from any cause of chronic lymphedema 3,4:
- 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 7.
The prognosis is very poor, because angiosarcomas are very aggressive tumors that frequently recur locally and often metastasize early.