It was classically attributed to lymphedemas induced by radical mastectomy to treat breast cancer. Nowadays, we know that it can arise in any chronically lymphedematous region due to any cause2.
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 mastectomy5,6.
It can arise from any cause of chronic lymphedema3,4:
- 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 oncogenesis7.
The prognosis is very poor, because angiosarcomas are really agressive tumors that frequently recur locally and often metastasize early.