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 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.
Treatment and prognosis
The prognosis is very poor because angiosarcomas are very aggressive tumors that frequently recur locally and often metastasize early.
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